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Genetics Gene mutations inflicting tissue overgrowth have lately been identified [7]. Prognosis Cellulitis complicating upper limb lymphoedema can on occasion be severe and life threatening. In circumstances the place systemic causes, for instance cancer recurrence or heart failure, have result in, or coexist with, the lymphoedema, then therapy of the medical situation have to be undertaken before embarking on specific lymphoedema remedy. The common precept for treating a swollen limb is to limit elevated microvascular filtration and enhance lymph drainage. Lymph drainage responds to train and motion carried out whereas sporting compression [9]. Where cellulitis, specifically recurrent cellulitis, occurs then prophylactic antibiotics could also be indicated [10]. Although surgical decompression and venous angioplasty may be thought-about for thoracic outlet obstruction, the everyday treatment for major subclavian vein thrombosis is oral anticoagulation only [11]. Synonyms and inclusions � Puffy face � Facial lymphoedema � Rosaceous lymphoedema � Morbihan disease � Solid facial oedema � Orofacial granulomatosis � Granulomatous cheilitis � Melkersson�Rosenthal syndrome Part 9: Vascular (b) figure one hundred and five. Oedema could lengthen beyond the face to involve the top and neck, which happens after surgery and/or radiotherapy for head and neck most cancers or with recurrent cancer. In cases of suspected noncancer lymphoedema, lymphoscintigraphy is the investigation of choice to affirm impaired lymph drainage. A venous duplex ultrasound examination is the first investigation of alternative in suspected venous outflow obstruction, Swollen face, head and neck desk one hundred and five. Congenital/genetic Vascular Vascular malformation Lymphatic Syndrome (neck webbing): Turner Noonan Generalized lymphatic dysplasia Mosaic with segmental lymphoedema Lymphangioma/lymphatic malformation overgrowth Macrocephaly, In one examine, the most typical websites of exterior lymphoedema had been the neck and submental area [1]. Conditions that need to be considered embody dermatomyositis, Graves disease and notably rosacea/acne. Eyelid swelling may be fairly simply due to acquired lax pores and skin from photoageing and other processes that have undermined tissue compliance, such as blepharochalasis [2]. Contact allergy or angiooedema, if persistent or recurrent, might slowly compromise lymphatic perform. Equally, one extreme attack of facial cellulitis could harm the lymphatics sufficiently to trigger lymphoedema. Angiosarcoma or Kaposi sarcoma could infiltrate local lymph drainage, and manifest with eyelid oedema. The European literature reviews that 46% of sufferers developed secondary lymphedema as a late effect of head and neck cancer treatment [4]. Any oedema, whatever the trigger, is because of microvascular (capillary) filtration overwhelming the lymph drainage for a sufficient period of time [5]. Head and neck oedema occurring in utero may regress by delivery but can leave signs such as prominent medial epicanthic folds or neck webbing postnatally, as seen in Turner and Noonan syndromes. A lymphatic malformation (lymphangioma) of the top and neck is extra frequent than lymphoedema and gives rise to swelling from lymph fluid current within abnormally formed lymphatics (whereas lymphoedema is lymph fluid within the interstitial space). Facial lymphoedema could also be secondary to different inflammatory pathologies of the skin such as rosacea or pimples vulgaris [6]. The skin or subcutaneous preliminary lymphatics fail rather than the primary regional amassing trunks, however as nicely as telangiectasia and inflammation contribute to oedema by way of elevated fluid filtration. Other inflammatory issues thought-about to trigger facial oedema embrace eczema, psoriasis, an infection, pediculosis [8] and trauma (cauliflower ears). Contact allergy or angiooedema, if persistent or recurrent, might slowly compromise lymphatic function [9]. One severe attack of facial erysipelas or cellulitis may harm the lymphatics sufficiently to cause lymphoedema. Chronic oedema of the eyelids is widespread and may be because of acquired lax pores and skin from photoageing and different processes which have undermined tissue compliance [2]. Medical circumstances to be considered with periocular oedema are dermatomyositis, Cushing syndrome (moon face) and thyroid illness notably Graves illness. Angiosarcoma or Kaposi sarcoma may infiltrate native lymph drainage routes and manifest with facial lymphoedema. Granulomatous inflammation may exist only domestically however a thorough seek for gastrointestinal Crohn illness or systemic sarcoidosis must be made. Granulomatous irritation from administration of lip fillers for beauty functions can even cause chronic swelling [11]. Clinical features the scientific options of facial lymphoedema depend upon the underlying aetiology. Swelling normally affects the central brow, periocular skin and cheeks the place it might be surprisingly asymmetrical. Erythema is all the time current in rosacea but inflammatory pustules and papules could also be conspicuous by their absence. An extension of the oedema inside the mouth is frequent which explains for the rugose adjustments on the buccal mucosal and tongue (scrotal tongue). Lymphoedema may cause facial disfiguration and distress in patients with head and neck most cancers [1]. Lymphoscintigraphy can be performed on the head and neck but is troublesome to interpret. Such imaging may assist distinguish between swelling from fluids and different tissue elements such as fat. Any irritation will want to be handled to scale back the upper lymphatic load arising from increased vascular permeability and blood circulate. Raising the pinnacle of the bed throughout overnight sleep helps to reduce venous strain and subsequently microvascular filtration. Otherwise the usual rules of enhancing lymph circulate through massage techniques and facial workout routines apply [12]. In rosaceous lymphoedema, antibiotic therapy appears disappointing in decreasing swelling; lowdose isotretinoin has been advocated, however may have to be sustained for 1�2 years [13]. The longterm consequence evaluation confirmed complete or partial decision of tissue swelling and oral ulceration in seventy eight. There are stories of therapeutic success with azathioprine, thalidomide, infliximab and mycophenylate mofetil. Modified decongestive lymphoedema therapy could be successful in treating head and neck lymphoedema following cancer remedy [15]. Mons pubis swelling can develop in isolation but more often is related to genital or lower limb lymphoedema. Swollen genitalia and mons pubis Definition and nomenclature Genital lymphoedema might affect the shaft of penis and/or scrotum plus the mons pubis. Synonyms and inclusions � Genital lymphoedema � Penoscotal lymphoedema � Vulval lymphoedema � Elephantiasis � Genital oedema � Massive localized lymphoedema � Acquired genital lymphangioma Genital lymphoedema may be major or secondary (Table 105. For swelling to happen, drainage pathways to each inguinal areas should fail or local genital lymphatics should turn into occluded.

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Subcutaneous fats also acts as an insulating layer in opposition to warmth loss and a protecting cushion towards exterior injury. Subcutaneous fats additionally provides structural support to the overlying pores and skin and has a cosmetic function, for instance within the contours of the face. There are obvious sexual variations in the distribution of fats around the body surface, with an increase in thickness ensuing in the rounded contours of the female trunk, breasts, hips, pubis and thighs. Subcutaneous fat additionally varies in thickness with the race, age and endocrine and dietary standing of the individual. Fat additionally has nice social significance as a major contributor to the sexual attractiveness of ladies on the one hand but, on the opposite hand, as a reason for distress when present in extra: fat kids could additionally be bullied or ostracised at school [2] and fats adults could find it harder to get certain jobs. Brown fats specifically (see later) has a very important thermoregulatory position and acts by rising the basal metabolic fee [3]. Numerous eccrine models are seen along the interface between the deep reticular dermis and subcutis (black arrow). The subcutis is organized into lobules of adipocytes and connective tissue septa (white arrow) surrounding and demarcating every fats lobule, and related vessels and nerves. The subcutis consists of skinny connective tissue septa (black arrow), which delimit lobules of adipocytes (white arrow). This is due to the fact that the lipid content dissolves in routinely processed specimens and the flat spindle nucleus is displaced to the periphery of the cell by a single large intracytoplasmic vacuole, which incorporates fats (white arrow). Cellular composition of subcutaneous tissue [5,6] the first fatcontaining cell, the preadipocyte, seems within the mesenchyme across the 14th week of fetal life. The primitive mesenchymal cell that forms the decided preadipocyte can be capable of maturing to type a fibrocyte, myocyte, chondrocyte or osteoblast. Preadipocytes can terminally differentiate into either brown adipocytes or white adipocytes. Brown fat is a particular sort of granular fats that differs from white fats in its distribution, histology and function. As it has a much larger capillary network surrounding it compared to white fat (which is partly liable for the brown colour), warmth can be rapidly transferred into the circulation. It is most prominent within the neck and higher thorax of the fetus, and it could be homologous to the hibernating gland fat found in some animals [7]. Brown fats is now known to persist into grownup life [8], and it may have a job in preventing weight problems [8]. Warm patches develop in the skin 1 h after taking ephedrine orally, and these warm patches could point out the location of thermogenic brown fats. Evidence for cold induction of brown fats as an adaptive response in people is at current equivocal [9]. White fats adipocytes are the biggest connective tissue cells within the physique, with a diameter of up to a hundred m. The mature adipocyte has a attribute signetring look, as a end result of the flat oval nucleus is displaced to the side by a single, massive, intracellular, fatcontaining vacuole, which is surrounded by perilipin. Each lobule could additionally be subdivided into 1 mm diameter micro- lobules, which symbolize the functional unit of the subcutaneous fats. Each microlobule is composed of a group of adipocytes arrayed round a central arteriole and surrounded by capillaries and postcapillary venules. Each particular person fats lobule is equipped by a small muscular artery (250�500 m diameter) branching from the septa to form arterioles (up to a hundred m diameter) that offer every individual microlobule. Each arteriole branches to form a community of capillaries that surrounds every particular person adipocyte. In addition to an ample blood provide, subcutaneous fats also contains a rich lymphatic plexus, which receives vessels from the dermis. These lymph vessels traverse the subcutaneous layer parallel to the skin surface for far, earlier than ultimately penetrating the deep fascia and draining into the regional lymph nodes. The nature of the adipocyte and its relationship to blood vessels and lymphatics has been reviewed in detail by Ryan and Curri [10]. Both white fats and brown fat are innervated by noradrenergic fibres of the sympathetic nervous system and parasympathetic fibres. The adipocytes might comprise solely 25% of the whole cell inhabitants of a lobule; the rest, the stromavascular fraction, being macrophages, fibroblasts, mast cells, pericytes, endothelial cells and preadipocytes, enabling appreciable crosstalk between cells by the use of domestically secreted cytokines together with leptin and adiponectin (see later). All fat tissue is composed of lobules of fats cells with their supporting connective and stromavascular tissue. In addition to the subcutaneous fats, approximately 20% of fat tissue happens internally, in the mediastinal and retroperitoneal tissues, the mesentery and the bone marrow and in and round individual organs, including blood vessels. In addition, perivascular adipose tissue reveals increased angiogenesis in comparability with subcutaneous fats [13]. The fact that some genetic lipodystrophy sufferers lose peripheral fats but fat padding for absorption of mechanical stress is maintained, is further proof for depotspecific differences. Subcutaneous fat is split by the superficial fascia into two compartments, superficial and deep. The fats mass in the superficial (areolar) layer is compartmentalized into lobules by vertical and indirect fibrous septal planes and bands, whilst that of the deeper (lamellar) layer has its septae extra horizontally positioned. The superficial layer is fairly constant, but the deeper is extra variable, with an increase in fats mass accumulating between cut up horizontal septae. In females, subcutaneous fat is most abundant in the gluteofemoral region and breasts, resulting within the socalled gynaecoid distribution, whereas in males the android distribution of shoulders and upper arms, neck and lumbosacral area predominates. The synthesis (anabolism) and catabolism of fat in the subcutaneous depot is determined by many components, together with nourishment and endocrine and neural exercise. The position of the autonomic nervous system in regulating fat metabolism is now properly established [19], being particularly necessary for rapid vitality need in comparability with the slower control exerted by neuroendocrine factors [20]. A lower in parasympathetic exercise leads to increased lipolysis, as does an increase in sympathetic activity, with the opposites stimulating lipogenesis [21]. The fat contained inside adipocytes are predominantly triglycerides (triacylglycerols), particularly these of palmitic and stearic acids and the unsaturated oleic acid. All the fatty acids have a fair variety of carbon atoms, predominantly C16 and C18, with a couple of C14 and C12. Adipose tissue in vitro has a metabolic price much like that of kidney tissue, and approximately half that of liver. Approximately half the triglyceride within the adipose tissue of rats and mice is catabolized and reconstituted in the midst of every week or so. At the same time, insulin inhibits hydrolysis and breakdown of triglyceride, conserving the power store. In both tissues, the important a part of the method consists of the oxidation in the mitochondria of the longchain fatty acids. This in flip might either be transformed to glycogen by reversal of glycolysis, or it might be transformed to pyruvate. Skeletal muscle readily oxidizes fatty acids but glucose, if out there, is preferentially used. During adipogenesis the local extracellular matrix additionally modifications, the consequences of which might play their very own function in differentiation. This is supported by the truth that fat tissue repair is improved if components of this matrix are included with the donor adipocytes. Insulin sensitivity Insulin secretion, stimulated by raised blood glucose ranges after meals to reverse hyperglycaemia, has two major effects.

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Unlike squamous cell carcinomas, basal cell carcinomas solely rarely originate on the vermilion however generally occur periorally [11�14]. In distinction to squamous cell carcinomas, basal cell carcinomas more commonly arise on the higher than the lower lip. The lesions can even arise de novo on the vermilion [15] or occasionally the mucosa of the lip, although unfold of a tumour from an adjacent website may hardly ever happen. Basal cell carcinoma has a number of varieties that may be divided as follows: � Nodular: the most frequent type around the lips presents as a waxy translucent nodule with fine telangiectasias, typically ulcerated. Multiple lesions are generally encountered and the varied forms have overlapping medical features. Basal cell carcinoma could be incessantly pigmented, resembling melanomas and different melanocytic lesions. Basal cell carcinoma could, of course, be a feature of naevoid basal cell carcinoma syndrome (Gorlin�Goltz syndrome, see Chapter 141). Furthermore, in addition to having a considerably increased threat for brand spanking new pores and skin cancers, patients with basal cell carcinoma have been shown to have an increased risk of growing noncutaneous cancers, including respiratory cancers, testicular most cancers, breast cancer and nonHodgkin lymphoma [16�18]. Basal cell carcinoma of the lips should be differentiated from different nodules, including squamous cell carcinoma, keratoacanthoma, trichoepithelioma and sebaceous adenoma. Since lesions that come up periorally are often aggressive, early detection and affirmation by biopsy will forestall infiltration and destruction of the underlying structures. Various treatment modalities for basal cell carcinoma embrace scalpel, electrosurgery, cryosurgery and laser surgical procedure, radiation, curettage and intralesional chemotherapy or interferon [19�21]. Selection of the treatment modality depends on the scale, site and histological sample of the tumour as properly as the age of the patient. Since lip lesions are sometimes of the nodular or morphoeic varieties, Mohs micrographic surgery [22], using microscopically managed excision, probably presents the highest cure price with the best preservation of tissue. Basal cell carcinoma of the lip Actinic radiation is a major aetiological factor in the growth of basal cell carcinoma, higher than 85% occurring on the solar exposed areas of the top and neck [1,2]. Fairskinned individuals who burn and people whose occupations require extreme exposure to sunshine are at greatest risk; the tumour is uncommon in dark skinned individuals, and 95% happen after the age of 40 years [3]. Keratoacanthomas are frequent selflimiting proliferative tumours that come up most regularly in males after the sixth decade of life [1,2]. Although some consider keratoacanthomas symbolize welldifferentiated squamous cell carcinomas, significant differences between the 2 entities have been demonstrated [3]. A number of welldocumented variants, many with typically distributed eruptive keratoacanthomas, have been described. The role of actinic damage is strongly supported by the truth that the majority of lesions happen on sunexposed skin (90%), with as a lot as 10% occurring periorally or on the vermilion border of the lips, usually on the decrease lip. Keratoacanthomas usually manifest at the vermilion border, as indurated domeshaped nodules displaying a attribute central, keratinfilled, crusted and frequently darkened crater. While cutaneous lesions are asymptomatic, labial and oral lesions are frequently painful [5�12]. They usually seem as an ulcer with a rolled margin, often on the anterior or maxillary gingiva, clinically indistinguishable from squamous cell carcinoma. It is unclear whether or not intraoral keratoacanthomas regress spontaneously, as all have been excised for analysis. Keratoacanthomas develop rapidly, attaining a dimension typically larger than 1 cm, could additionally be domestically invasive and lead to significant tissue harm but, if left untreated, many undergo spontaneous involution after 1�2 months [13]. When lesions develop intraorally or on the lips, they want to instantly be subjected to biopsy for confirmation, since squamous cell carcinomas at these sites frequently metastasize. Intralesional remedy with methotrexate or 5fluorouracil can be employed with glorious results [14,15]. Other suggested medical therapies embody intralesional interferon 2a and systemic isotretinoin [16�18]. Treatment is by application of petroleum jelly and avoidance of the opposed environmental situations. Cheilitis Synonyms and inclusions � Inflammation of the lips Cheilitis could come up as a main disorder of the vermilion zone or the inflammation may prolong from close by skin or, much less typically, from the oral mucosa (Box one hundred ten. Contact cheilitis Contact cheilitis is an inflammatory reaction provoked by the irritant or sensitizing action of chemical compounds. Most instances are caused by the deliberate utility of lipsticks or lipsalves but many substances have been incriminated, typically from unintentional contact with an offending substance (Box one hundred ten. Lipsticks are composed of mineral oils and wax (which type the stick), castor oil as a solvent for the dyes, lanolin as an emollient, preservatives, perfumes and colors [1�7]. An eosin impurity used to be an essential sensitizer [8] but is now hardly ever if ever used. Other components sometimes incriminated embody azo dyes, carmine, oleyl alcohol [9], lanolin, perfumes, azulene, propyl gallate [10], sesame oil [11], stearates [12], shellac and colophony [13,14]. Petrolatum chapsticks might cause an unusual type of acne with a single row of large open comedones alongside the cutaneous margin of the upper lip [18]. The keratin of the vermilion loses its plasticity, in order that the lips turn into sore, cracked and scaly. The affected individual tends to lick the lips, or to choose at the scales, which can irritate the condition. Lipstick cheilitis is typically confined to the vermilion but extra usually extends beyond. There may be persistent irritation and scaling or a extra acute response with oedema and vesiculation. If a small sucked object is accountable, the response could also be confined to one part of the lips. Azo dyes Carmine Colophony Eusolex Oxybenzone ptertiarybutylphenol Propyl gallate Sesame oil Wax Azulene Castor oil Eosin Lanolin Phenyl salicylate Ricinoleic acid Shellac Benzoic acid Cinnamon Ester gum Oleyl alcohol Propolis Salol Vanilla Diagnosis. If acute eczematous changes are clearly current, the prognosis of contact cheilitis presents no issue. If the modifications are confined to irritation and scaling, the assorted types of exfoliative cheilitis should be excluded. Topical corticosteroids or pimecrolimus will often give symptomatic relief but the offending substance should be identified and averted. Metal hair clips, metal pencils, the cobalt paint on blue pencils, nail varnish, and the metal, wooden, nickel and reed mouthpieces of musical wind devices [15] could additionally be implicated. Apple Banana Cherry Kiwi fruit Mango Parsley Pear Artichoke Carrot Fennel Lemon Onion Parsnip Pineapple Asparagus Celery Garlic Lime Orange Peach Plum a hundred and ten. Aromatic retinoids corresponding to etretinate and isotretinoin trigger cheilitis, dryness and cracking of the lips in lots of sufferers [1,2]. Eczematous cheilitis the lips are often concerned secondarily to atopic eczema (see Chapter 41). In a large Russian series, virtually half the cases had related thyroid disease [1], however this statement has not been confirmed.

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The persistent excessive strain exerted on the skin of the soles of the ft could end in plantar hyperkeratosis and postmenopausal plantar keratoderma (keratoderma climactericum) [2,6]. Lower limb venous hypertension from no matter trigger may be exacerbated within the overweight by excessive intraabdominal pressures and by immobility. The risks of venous eczema and venous ulceration are increased they usually may be tougher to manage. The prevalence of superficial skin infections similar to candidosis, dermatophytosis and erythrasma is raised within the obese [2]. Pyogenic infections corresponding to folliculitis and furunculosis are additionally extra regularly seen and could additionally be recurrent. Obesity additionally will increase the risk of surgical wound infection and of necrotizing fasciitis [8]. It is more and more acknowledged that adipose tissue has a quantity of, complicated regulatory and hormonal capabilities in addition to its position as an energy retailer (see Chapter 99); these features could also be disturbed within the obese. Altered adipocytokine secretion may affect irritation in the overweight [9,10]: for example, delayedtype hypersensitivity responses are increased in weight problems and decline with weight reduction [11]. Adipocytokines may play a role within the wellrecognized hyperlink between extreme psoriasis and obesity, with evidence that weight reduction, together with that achieved by bariatric surgical procedure, can produce important enchancment in psoriasis [2,12,13]. There can additionally be some evidence of a link between obesity and atopy together with atopic eczema [2,13]. Case control examine of semicircular lipoatrophy, a model new occupational disease in office workers. Skinrelated complications of insulin therapy: epidemiology and rising administration strategies. Lipodystrophy reactions to insulin: results of continuous insulin infusion and new insulin analogs. Treatment of local, persistent cutaneous atrophy following corticosteroid injection with regular saline infiltration. Lipodystrophia centrifugalis abdominalis infantilis: statistical evaluation of 168 circumstances. Lipodystrophia centrifugalis abdominalis infantilis in a 4yearold Caucasian woman: association with partial IgA deficiency and autoantibodies. Centrifugal lipodystrophy of the scalp presenting with an archform alopecia: a 10year followup statement. Prevalence of lipohypertrophy in insulin treated diabetic sufferers and predisposing components. Resolution of lipohypertrophy following change of short appearing insulin to insulin lispro (Humalog). Subcutaneous lipomatosis Benign symmetrical lipomatosis 1 BreaGarcia B, CameselleTeijeiro J, CoutoGonzalez I, TaboadaSuarez A, GonzalezAlvarez E. Multiple symmetric lipomatosis: medical features and consequence in a longterm longitudinal study. Surgical treatment of multiple symmetric lipomatosis with ultrasoundassisted liposuction. This manifests as elevated dangers of zits, hirsutism, androgenetic alopecia and polycystic ovary syndrome within the overweight [2,15]. Type 2 diabetes is a standard complication of weight problems and is related to a wide selection of specific skin issues (see Chapter 64). Obesity is a element of a selection of genetic issues of which Prader�Willi syndrome is doubtless considered one of the finest identified. The presence of weight problems may worsen a variety of different skin disorders together with hidradenitis suppurativa and pilonidal sinus. Surgical wound therapeutic is impaired within the overweight with an elevated danger of incisional hernia [16]. Clinical options and metabolic derangements in acquired generalized lipodystrophy: case stories and evaluate of the literature. Clinical options and metabolic and autoimmune derangements in acquired partial lipodystrophy: report of 35 instances and evaluate of the literature. Clinical review 153: lipodystrophy in human immunodeficiency virusinfected sufferers. Changes in body composition with ritonavirboosted and unboosted atazanavir treatment in combination with lamivudine and stavudine: a 96week randomized, controlled research. Impact of switching antiretroviral remedy on lipodystrophy and other metabolic complications: a evaluation. Congenital infiltrating lipomatosis of the face: clinicopathologic evaluation and treatment. Congenital infiltrating lipomatosis of the face: report of circumstances and review of the literature. Encephalocraniocutaneous lipomatosis accompanied by the formation of bone cysts: Harboring clues to pathogenesis Clinical and pathological features of 31 circumstances of lipedematous scalp and lipedematous alopecia. Hyperplasia of the subcutaneous adipose tissue is the first histopathologic abnormality in lipedematous scalp. Anatomy and physiology of subcutaneous adipose tissue by in vivo magnetic resonance imaging and spectroscopy: relationships with sex and presence of cellulite. Purpura is the hallmark of vasculitis affecting smaller vessels of the pores and skin, and will be the dominant function or a minor a half of a systemic vasculitis; in any other case purpura is usually seen by haematologists because of platelet and coagulation problems. Vasculitis additionally causes other cutaneous lesions with a purpuric component, together with urticarial lesions, nodules, ulcers, livedo and skin necrosis. The management of purpura may involve a multidisciplinary strategy including haematology, general drugs, nephrology, rheumatology and dermatology. Classification of purpura is troublesome, as related scientific patterns might come up from different causes, together with vasculitic and nonvasculitic. Nonspecific capillaritis could additionally be idiopathic, druginduced or a manifestation of cutaneous Tcell lymphoma. Purpura due to idiopathic thrombocytopenic purpura could also be a combination of illnesses [2]. Microvascular occlusion may give rise to palpable lesions and irritation as a secondary component much like vasculitis, clinically and histopathologically. The differential prognosis of purpura is predicated on morphology [1], as few sufferers current with a identified aetiology. Haematological causes (thrombocytopenia and clotting factor abnormalities) can be quickly and easily evaluated by full blood count and laboratory measures of clotting occasions. It is important to differentiate between problems causing primarily both inflammatory or noninflammatory lesions [5]. Purpura is discoloration of the skin or mucous membranes as a end result of extravasation of the purple blood cells. Thrombocytopenia normally causes petechiae however more extensive bleeding might occur at decrease ranges of platelet count.

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As trauma and terminal phalanx fractures can mimic acute paronychia, radiography is suggested when the latter occurs after trauma. Herpetic paronychia could trigger complete destruction of the nail, bacterial superinfection and systemic spread that will cause meningitis [4]. It can be observed in patients with paronychial an infection caused by orf or herpes viruses [5]. Paronychia of the nice toe of infants Undersized infant jumpsuits can be answerable for paronychia of the good toe. The undersized clothes likely produce primary trauma with subsequent an infection or potential focal ischaemia, growing the dangers of an infection after minor trauma [6]. It may be associated with an infection secondary to an underlying continual dermatosis, for instance an irritant contact dermatitis from moist work or exposure to caustic supplies. Alternatively, it may be secondary to atopic eczema or psoriasis, the place minor provocation may end up in energetic illness [7]. Handling of moist foods represents a specific hazard, as these usually combine a quantity of predisposing components including wet working conditions, a cold surroundings and irritation from the meals itself. Chronic paronychia is predominantly a illness of domestic and Nail fold infections ninety five. The situation begins as a slight erythematous swelling of the paronychial tissues. It may be painless however, if tender, is much much less so than in acute paronychia, besides when pressed. Inflammation adjoining to the nail matrix disturbs nail progress, resulting in irregular transverse ridges and different surface irregularities, which can be mixed with discoloration. There is a few proof that the darkening of the lateral edges of the nail plate could additionally be because of the pigment of Candida spp. The lateral discoloured edges of the nail plate become crossridged when the disease mainly impacts the lateral nail fold. Repeated acute exacerbations produce numerous irregular transverse ridges or waves on the nail surface, which frequently turns into tough. Neoscytalidium dimidiatum can also produce darkening of the lateral edges of the nail plate; against this, paronychia as a outcome of moulds similar to Fusarium spp. In longstanding instances, the scale of the nail could additionally be lowered, and this reduction is exaggerated by the bolstering of the fold throughout the nail. Most of the nail deformity is as a end result of of inflammation, which interferes with the formation of the nail, however a real Candida infection of the nail plate is occasionally seen, especially in patients with immunodeficiency. Much of the persistent irritation seen on this dysfunction probably arises from an irritant reaction to materials sequestered beneath the proximal nail fold. The loss of the cuticle implies that detergent and different solvents may gain access to this tight area and act like a chronic irritant patch take a look at. Acute exacerbations occur from time to time and are as a end result of secondary bacterial infection. Besides this most typical type of continual paronychia, an extended listing of causes of paronychia is supplied (see Table 95. Topical imidazoles are normally enough to treat Candida and should present modest activity against some bacteria. Twice a day application of Dakin resolution (sodium hypochlorite) could be very efficient against Pseudomonas infection. When vital nail dystrophy ensues and medical remedy has been unsuccessful, chronic paronychia can be treated surgically with good outcomes and determination of the dystrophic nail. In patients who expertise repeated acute flares related to continual paronychia, additional removing of the bottom of the nail plate is beneficial. Primary syphilis on the finger the finger accounts for 5�14% of extragenital main syphilitic chancres. It may present as a deep painful horseshoeshaped whitlow with diffuse induration of paronychial tissues and related regional lymphadenopathy. Pain and tenderness of the fingertips with swelling and serous discharge can also be observed [9]. Periungual toe infections in neutropenic sufferers In neutropenic sufferers with haematological or solidorgan malignancy, the most common causes of paronychia with cellulitis of the toe are Fusarium and, much less frequently, Aspergillus. The key to profitable management is early removing of the infected nail for analysis and institution of acceptable therapy [10]. There is a yellow discoloration of the nail and onycholysis could affect the distal third of the nail. The severe throbbing ache is similar to that related to a subungual haematoma and is caused by pressure. Perhaps an important part of the remedy, however the one most difficult to obtain, is preserving the hands dry. Patients concerned in wet work ought to be advised to wear cotton gloves underneath rubber or plastic gloves and avoid manicure of the proximal nail fold. General hand care with emollients and protection from trauma and irritants is helpful. Partial avulsion of the abnormal nail area permits the nail mattress to be treated with chlorhexidine, mupirocin or fusidic acid [11]. Other disorders of the perionychium Druginduced paronychia Paronychia could additionally be caused by certain medicine such as isotretinoin and may involve a number of digits [1]. Periungual tissues topic to trauma � onychotillomania Hangnails are small parts of the sexy epidermis that have cut up away from the lateral nail fold. Hangnails are frequent in people who deal with irritants or who work primarily with their hands. Inflammation is normally current causing pain, particularly if the hangnails are interfered with (onychotillomania). In addition to classical hangnails, scaling of the nail folds with scattered small haemorrhages and focal erosions or necrosis may be noticed, typically involving the toes. The discomfort skilled by the subjects might render many delicate tasks difficult and even inconceivable. There seems to be no anatomical basis for the location of fissure, although it may replicate some structural weak spot distal to the lateral nail grooves. Hypertrophy of the lateral nail fold this condition is usually the results of longstanding ingrown nails in adults. It is also seen as a congenital condition appearing as overgrowths of the lateral nail folds of each halluces shortly after birth. Hypertrophy of the lateral nail fold resolves spontaneously through the first yr of life [13]. In children with psoriasis, the reported prevalence of nail involvement ranges from 7% [4] to 39% [5]; pitting has been observed within the first week of lifetime of a neonate whose mom had extreme psoriasis [6]. Psoriatic nail modifications are prominent in childhood nail disease, parakeratosis pustulosa: roughly one third of affected youngsters will develop manifest psoriasis over time, a smaller fraction will have variants of eczema and half of the entire will get higher [7]. Pathophysiology Nail unit psoriasis is a localized form of the method energetic elsewhere in the physique and the features symbolize a mix of local skin changes and secondary results on nail plate growth.

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There may be uncovered bone in the floor of the canal and swelling of the delicate tissues of the pinna and beyond. The tympanic membrane is incessantly necrotic in youngsters, however is characteristically spared in adults [2]. Age Necrotizing exterior otitis is commoner within the middleaged and older affected person, although it has been reported in kids [1,2]. Other differentials embrace carcinoma and granulomatous ailments, which can be excluded by taking samples for histology. Recently, there have been cases of bisphosphonateinduced osteonecrosis of the auditory canal mimicking a number of the options of necrotizing external otitis [10]. Classification of severity Involvement of the cranial nerves, associated meningitis and/or cerebral abscess represent the extreme finish of a spectrum. Complications and comorbidities Cranial nerve involvement might not get well, significantly in facial palsy. Other problems embody temporomandibular joint osteomyelitis, dural sinus thrombosis, meningitis and cerebral abscess. Many other organisms have been demonstrated, together with Staphylococcus aureus, Klebsiella spp. Disease course and prognosis Mortality was around 50%, but now could be normally 20% or lower. Adverse prognostic elements are cranium base osteomyelitis, intracranial extension and involvement of a number of cranial nerves [11]. Otomycosis Definition and nomenclature Otomycosis is an inflammatory situation of the ear canal in which yeast or fungal organisms play a vital part. Swabs, and if necessary, tissue should be despatched for aerobic, anaerobic and fungal tradition. When available, gallium scintigraphy could be the most helpful for assessing response to treatment [16,17]. Introduction and basic description Otomycosis is acknowledged clinically by seen growth of filamentous fungi on the skin of the ear canal. Management Whenever potential, remedy must be guided by tradition and sensitivities, and the antimicrobial remedy given for no less than 6�8 weeks. As properly as monitoring for medical improvement, repeat imaging may be needed to assess whether the disease is responding. If the Pseudomonas is resistant to ciprofloxacin, an aminoglycoside plus ceftazidime is more probably to be the best choice. For other micro organism and fungi, the selection of remedy might be dictated by the microbial tradition. Third line Hyperbaric oxygen is usually used as an adjunct, however its role has not but been established [19,20]. Predisposing components Heat, humidity, trauma, frequent publicity to water, and prolonged use of antiobiotics and/or steroid drops within the ear canal could all be important predisposing components. Particularly within the immunosuppressed, the fungi can penetrate into the dermis and beyond. Occasionally, different yeasts and fungi are found, including phycomycetes, Rhizopus, Actinomyces and Penicillium, and, hardly ever, dermatophytes. The fact that these organisms could be pathogenic in addition to saprophytic has been confirmed in a variety of studies [2,4,5]. Presentation On examination, the dominant function is the presence of wispy filamentous plenty, which can be isolated or diffusely present in the canal. Usually, only one ear is concerned, but otomycosis is quite commonly bilateral within the immunosuppressed [6]. In the exterior auditory canal, benign tumours are probably to present with listening to loss and may predispose to infection. Exostoses are usually bilateral, symmetrical, multiple, diffuse, broadly primarily based growths of bone. Frequent publicity to chilly water, such as from surfing, is an aetiological factor in practically all cases. Somewhat comparable are osteomas, although these can usually be differentiated by their solitary and unilateral distribution. Other benign lesions that may current within the canal are fibrous dysplasia � each monostotic and polyostotic (Albright syndrome), eosinophilic granuloma, cholesteatoma and keratosis obturans, benign ceruminous gland tumours, cartilaginous choristomas and temporomandibular joint herniation. Part 10: SiteS, Sex, age Classification of severity the presence of cellulitis of the surrounding delicate tissues without bacterial infection represents more severe infection, and is more likely within the immunocompromised. Otomycosis can become an invasive illness with penetration into the temporal bone in the immunosuppressed. Disease course and prognosis Otomycosis is often a continual dysfunction, however can be cured if adequately treated and any predisposing factors corrected. It is commonly difficult to distinguish between adenoma and carcinoma on histological grounds [1,2]. The tumours comprise benign and pleomorphic adenomas, adenocarcinomas, adenoid cystic carcinomas and maybe others including mucoepidermoid carcinomas. Tumours of the cerumen glands have been reported in association with other sweat gland tumours elsewhere [4]. Investigations Fungal microscopy and culture are needed to set up the prognosis. Management the ear canal must be carefully cleaned of debris and discharge and delicate suction used if available. They include aluminium acetate, acetic acid, tumours of the auricle and external auditory canal 108. Extramammary Paget illness of the external ear and/or canal resembles Bowen illness or an inflammatory dermatosis [7]. Other symptoms embrace bleeding, otorrhoea and, with unfold of the neoplasm, nerve palsies. Because of the potential for malignant behaviour, all ceruminous gland tumours should be totally excised with an enough margin of regular tissue [8]. Other predisposing factors embody prior ionizing radiation, a persistent dermatosis similar to lupus vulgaris, and genetic elements similar to xeroderma pigmentosum and Gorlin syndrome. The commonest premalignant lesion is the photo voltaic keratosis, which may happen on all sunexposed aspects of the auricle, but is very frequent on the upper floor of the helix. The medical displays include an erythematous telangiectatic patch, a focal area of scaling or hyperkeratosis, or a cutaneous horn. On the auricle, development to squamous carcinoma from photo voltaic keratosis may happen more readily than at other sites. Other premalignant lesions embody Bowen disease, radiation and tar keratoses and, rarely, keratoacanthoma. They include excision, curettage, electrosurgery, cryotherapy, 5fluorouracil and photodynamic remedy. The choice will depend upon numerous elements, together with the necessity for a tissue prognosis, dimension and location of the lesion, likely cosmetic outcome and the out there amenities. Followup is essential for detection of recurrences and the looks of new lesions. Lesions closely resembling squamous carcinoma, such as keratoacanthoma, ought to probably be totally excised to guarantee accurate prognosis.

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It is unlikely that a single pathophysiological pathway is answerable for the diverse scientific features seen in sufferers with the different subtypes of rosacea. The function of ultraviolet light in the causation of rosacea has been repeatedly advised and is supported by the facial distribution (mainly on the convexities) and its prevalence on the bald (exposed) scalp of male sufferers. Actinic elastosis is a distinguished discovering in facial skin biopsies from patients with rosacea, but whether this reflects the expected degree of change in center aged sufferers with skin sorts 1 or 2 or if this relates on to the pathogenesis of rosacea is unclear. It may be that ultraviolet light publicity is a subsetspecific aetiological factor. However, alteration in the follicles in androgenetic alopecia from terminal to vellus may also be vital and has to be thought of instead explanation. These mites may also play a task in the modulation of the host innate immune system described above, as has been proven by other microorganisms within the skin microbiome [15]. Alterations in the cutaneous microenvironment in patients with rosacea similar to modifications in lipid profile, cutaneous pH or skin barrier function [18] might facilitate an overgrowth of commensal organisms, which may then trigger a host immune response as quickly as a important level is reached. A consistent finding is a lowered tear breakup time in patients with rosacea on account of insufficient lipid elements of the tear movie. Meibomian cysts (usually painless), representing persistent irritation of the meibomian glands, may appear in crops. It has been suggested that Demodex mite infestation might play a role in the initiation of inflammatory ocular adjustments that happen in these modified sebaceous glands of the eyelid. This speculation is supported by the presence of mites within the collarettes and sleeves of keratin at the bases of the lashes, indicating a attainable frequent aetiological link between cutaneous and ocular rosacea [20]. These sufferers could develop rosacea at an earlier age than those with no household historical past of the dysfunction. Studies have persistently proven that folks with skin varieties 1 or 2 are more susceptible to developing rosacea than these with darker pores and skin. It has been advised that sufferers with the carcinoid syndrome who flush frequently are predisposed to growing rosacea, but convincing evidence for susceptibility to rosacea on this unusual illness is missing. A common discovering is the histological evidence of chronic actinic damage, which is present in most biopsies, as may be expected in a predominantly sunsensitive middleaged population. A gentle perivascular and interstitial lymphocytic infiltrate with frequent plasma cells is often seen in this subtype. Increased numbers of mast cells have been reported in the lesional skin of rosacea patients and these may take part within the induction of inflammation and the recruitment of neutrophils. A correlation between the degree of follicular infestation with Demodex mites and the upregulation of proinflammatory genes has been shown. Sometimes Demodex mite remnants are seen within these granulomas or abscesses [22,23]. Blepharitis in rosacea mainly includes the posterior meibomian glands (posterior blepharitis). There is an inflammatory infiltrate (lymphocytic/ histiocytic/neutrophilic) that varies based on the severity of the condition. Some degree of granulomatous inflammation could also be seen in biopsies from patients with all cutaneous forms of rosacea [24]. The term granulomatous rosacea (lupus miliaris disseminatus faciei, acnitis, pimples agminata) is, nonetheless, best reserved for an unusual variant which is discussed in further element later within the chapter. In this situation sarcoidal or tuberculoid granulomas with or without abscess formation, were noticed in biopsies from 25 patients with perifollicular fibrosis evident in some late lesions [25]. Genetics Although as much as 25% of patients with rosacea have a optimistic household history, no rosaceaspecific genes have yet been recognized. Transcriptome profiling evaluation reveals distinct gene profiles for every rosacea subtype (with certain genes overlapping between subtypes) [34]. A current evaluation of the microorganisms which may doubtlessly induce rosacea has recognized several potential candidates together with Staphylococcus epidermidis, Chlamydophila pneumoniae and the Demodexassociated bacterium Bacillus oleronius [26]. Several research have documented an increased inhabitants of Demodex mites within the facial pores and skin of patients with rosacea [27,28]. A systematic review of case�control research (with 28 527 participants) has proven a major affiliation between the degree of Demodex infestation and the presence of rosacea [29]. An increased interest within the potential role of this organism in skin disorders is indicated by a lately proposed classification of Demodexrelated human dermatoses [33]. Some patients complain of an disagreeable burning or stinging sensation in the pores and skin; that is often exacerbated by sunlight and wind exposure. There is frequently other proof of actinic injury (actinic keratoses, actinic lentigines, and so forth. There can also be mild facial oedema, most noticeable if there are widespread inflammatory lesions. Papules typically appear to be in numerous phases of evolution and untreated lesions wax and wane spontaneously over a course of weeks. Lesions that resolve sometimes heal with out scarring but could leave persistent postinflammatory erythema. Clinical features Rosacea is a dysfunction that usually presents in middle age (peak onset between 35 and 50 years). Patients with rosacea current a spectrum of medical options that differ in accordance with each subtype. Rhinophyma has additionally been reported to comply with actinic injury and to happen in sufferers with pimples vulgaris. As the dysfunction progresses, sufferers remark that their nose is rising in dimension and that the distal end is changing into bulbous. Sometimes these modifications are greatest visualized (as on this case) by viewing the nostril from below. Skin floor biopsy to consider the contents of the dilated follicles in rhinophyma usually reveals the presence of trichostasis in addition to Demodex mites. Conjunctival fibrosis and keratitis are rare and indicative of severe ocular rosacea. These lesions respond to the topical and systemic medications as prescribed for the facial lesions. Metophyma, gnathophyma and otophyma discuss with phymatous adjustments of the forehead, jaw and ears, respectively. Investigations revealed her to have pulmonary and ocular involvement making a analysis of sarcoidosis somewhat than granulomatous rosacea. There is a profuse eruption of small, firm, monomorphic, plumred, domeshaped papules on the chin and cheeks and around the eyes. Its characteristic orangered appearance with adherent massive scales and its distribution (alae nasi, eyebrows, scalp, and so forth. Lupus erythematosus (systemic and subacute) and dermatomyositis could current with photodistributed facial erythema and must be considered in a affected person with systemic signs. Involvement of the neck and other exposed areas level towards the proper diagnosis and acceptable serological screening should be carried out.

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With the corneal surface failure, these corneal issues account for the management challenges posed by the disease. Extending this to clinical settings, the ensuing uncertainty in regards to the prognosis has meant that immunosuppressive remedy has been delayed or deferred in some patients. Current aggressive therapy regimens with systemic immunosuppression, have been shown to scale back the rate of development. Because sufferers can occasionally progress to blindness inside months from the onset, each early diagnosis and effective therapy are important in bettering the prognosis. At presentation, between 25 and 38% of sufferers with ocular illness have important visible loss and about 30% turn into legally blind. Squamous metaplasia of the conjunctival epithelium and a reduction in goblet cells are nonspecific findings, as is an increased inflammatory cell infiltrate. When ocular circumstances are reported in research, the authors believe that the detailed immunopathology findings must be recorded for each case so that the analysis could be interpreted within the mild of future modifications to diagnostic standards. Taking conjunctival biopsies for direct immunofluorescence this has been described recently [21]. Fornix biopsies ought to be avoided as taking biopsies from this site might result in fornix contracture. The biopsy could be taken utilizing topical anaesthesia with tetracaine, the conjunctiva is tented up and a chunk about 2 mm in diameter minimize off with spring scissors. This could be done in the outpatient division utilizing magnification from the slit lamp microscope. Routine histopathology of conjunctival biopsies is of little value within the prognosis as a end result of the conjunctiva is fragile and detection of basement membrane zone cleavage is unreliable. The aim of treatment is the successful management of every of 5 principal components of these illnesses. These parts of disease are present to variable degrees in numerous ailments. The only demonstrated technique of slowing the development of scarring is nice management of inflammation, normally with systemic therapy. Manage any ocular surface illness Ocular floor disease is secondary to earlier or current lid and conjunctival scarring and irritation. This floor disease causes a lot of the harm to the cornea and is responsible for further irritation. Trichiasis and entropion, blepharitis, dry eye and filamentary keratitis, keratinization, persistent epithelial defect, microbial keratitis and corneal perforation might all outcome from a mixture of a poor tear movie, poor lid closure and corneal harm secondary to trichiasis. Treat an infection if present Remove preservatives Treat blepharitis No Treat dry eye Treat filamentary keratopathy Treat trichiasis and entropion Treat keratin In conjunctival irritation nonetheless present Part 10: SiteS, Sex, age � Blepharitis: use oral tetracyclines and institute a lid hygiene routine (see section Blepharitis, meibomian gland dysfunction, rosacea and seborrhoeic dermatitis). Exclude and treat any secondary an infection the compromised setting of the cicatrized conjunctiva harbours extra probably pathogenic bacteria [33] and fungi corresponding to yeasts. The index of suspicion for an infection have to be high, for instance in the presence of any corneal epithelial defect, provided that the everyday clinical indicators of a white cell mediated corneal infiltrate could additionally be suppressed in an immunosuppressed host, and topical steroids can also contribute to lack of an infiltrate. Eliminate or minimize treatment toxicity Treatment toxicity results principally from the preservative benzalkonium chloride, a element of most reusable bottles of eye drop preparations in addition to of topical glaucoma medications and aminoglycoside eye drops. Unnecessary topical treatment ought to therefore be avoided and unpreserved drops or saline used as far Cicatrizing conjunctivitis related to immunobullous issues 109. The effects of topical treatment toxicity are onerous to distinguish from those of the ocular floor disease. After withdrawal of toxic topical remedy, the mean restoration period is 2 weeks but could extend to 3 months. Suppress inflammation/commence immunosuppressive therapy Any inflammation on the upper bulbar conjunctiva which persists once any ocular surface disease has been handled and any infection and therapy toxicity excluded, is prone to be because of underlying autoimmune exercise. In some instances, conjunctival autoimmune exercise can, nonetheless, manifest as a patchy distribution of inflammation, and this may not necessarily involve the upper bulbar conjunctiva. In these instances, persistence of inflammation despite maximal treatment of ocular surface illness is a sign that systemic immunosuppression is required. Second line: systemic immunosuppressive therapy For reasonable disease (hyperaemia, intense infiltration), immunosuppressants used initially include a sulpha agent corresponding to sulphapyridine or dapsone [7], or the antimetabolite methotrexate. The dose of sulphapyridine is 500 mg daily for 2 weeks then 1 g daily (sulfasalazine 1�2 g daily is an alternative); 55% of patients reply in 1�2 months, but 15% develop an allergic response. Another agent which may be thought-about is azathioprine (1�2 mg/ kg/day; efficient in about 60%). For extreme disease (hyperaemia, limbitis, conjunctival ulceration), begin oral prednisolone 1 mg/kg/day, reduce the dose after 2 weeks and tail off after 2�4 months. For severe and rapidly progressive illness, cyclophosphamide is the drug of alternative. Patients are started on this with a 2month course of systemic steroids, which accelerates the management of inflammation, whereas the cyclophosphamide takes impact (2�3 months). Steroids, the sulphones (dapsone and sulphapyridine) and the bone marrow suppressive immunosuppressants (methotrexate, mycophenolate, azathioprine and mycophenolate) may be combined though the neutrophil depend, in particular, must be monitored. For these patients, intravenous immunoglobulin or a biological agent (antitumour necrosis factor or rituximab) may be efficient. Systemic ciclosporin has been little utilized in cicatrizing conjunctivitis due to lack of evidence of efficacy, concern about potential fibrogenic unwanted facet effects as nicely as probably renal toxicity in the elderly subset of sufferers. Cyclophosphamide remedy is normally discontinued after 1 year and substituted by sulphonamides or immunosuppressive agents as described above. Results of these therapies have been reported in nonrandomized series or case stories. Most of this remedy is empirical, and a sulphonamide (dapsone or sulphapyridine) is commonly used together with an alkylating agent (cyclophosphamide) or an antimetabolite (azathioprine, methotrexate or mycophenolate mofetil) to management inflammation. Corneal epithelial defects are widespread and should progress to corneal ulceration with or without bacterial superinfection. In mildly affected sufferers, this causes chronic delicate discomfort, photophobia and barely lowered vision. The conjunctival shortening leads each to entropion, leading to ocular floor abrasion by regular as well as by metaplastic lashes, and may also trigger lid shortening, resulting in lowered eye closure (lagophthalmos), which is easily ignored. Lash abrasion and trichiasis result in the development of corneal epithelial defects which, as a end result of the poor tear movie, could persist. Persistent epithelial defect predisposes to corneal stromal melts and perforation, which are often precipitated by infection. A grading system for the persistent ocular manifestations of Stevens�Johnson syndrome has been proposed [40]. The severe inflammation can also result in blinding opacity of the corneal epithelium, not only on account of squamous metaplasia, but also by lack of corneal epithelial progenitor cells (stem cells).


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