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W2009180675 abstract "End-stage renal disease (ESRD) is defined as progressive and irreversible kidney dysfunction that lasts longer than 3 months. Nitrogenous by-products of protein catabolism, represented as urea and otherwise known as blood urea nitrogen or uremia, commonly accumulate within the serum of these patients due to inadequate renal excretion. Cutaneous disorders are a common manifestation of patients with ESRD. Several studies have examined the prevalence of dermatologic disease in this setting. Nunley1 reported that 50–100% of patients have at least one dermatological disorder. Pico et al.2 assessed the prevalence of dermatologic problems among one-hundred and two patients with chronic renal failure undergoing dialysis. All patients examined had at least one cutaneous lesion with the most prevalent disorder being hyperpigmentation. It has been proposed that many of the cutaneous disorders experienced by patients undergoing dialysis have little to do with the uremic syndrome and are related to the underlying pathologic process that induced the renal disease.1 On the contrary, others proposed that changes in skin histology were more related to the severity and duration of the renal failure and less with its underlying etiology.3, 4 Recent advances in the treatment have improved the quality of life and life expectancy of these patients, resulting in changes in the frequency and types of disorders observed in conjunction with ESRD. Dermatologic conditions such as uremic frost, erythema papulatam uremicum, uremic roseola, and uremic erysipeloid now seldom occur. Various specific and non-specific skin abnormalities are observed in patients with ESRD. Non-specific disorders include pigmentary disorders, pruritus, xerosis, acquired ichthyosis, and half-and-half nail. Specific disorders include acquired perforating dermatosis, calciphylaxis, bullous dermatoses, and fibrosing dermopathy of uremia. Dermatological manifestations of renal disease may further be divided into three general categories including (1) dermatological manifestations of disease associated with the development of ESRD, (2) dermatological manifestations of uremia, and (3) dermatological disorders associated with renal transplantation. This literature review discusses the clinical and pathologic manifestations of uremia and end-stage renal failure. Pruritus, defined as itching, represents a prevalent and disturbing skin manifestation of uremia and ESRD.5 Pruritus is not a specific symptom of ESRD as it is seen in a variety of unrelated clinical entities such as atopy, hepatobiliary disease or even Hodgkin's disease. Pruritus, though uncommon with acute renal failure, is associated with protracted uremia and in particular is seen in association with ESRD. This temporal relationship is underscored by the relationship between the alleviation of symptoms and frequency of dialysis.6 Clinically, significant pruritus has been shown to affect 15–49% of patients with chronic renal failure and 50–90% of the dialysis population.5 Stahle-Backdahl6 described severe pruritus in 8%, moderate pruritus in 24%, and mild pruritus in 66% of patients. Gilchrest7 has reported a prevalence of 19–90% among hemodialysis (HD) patients. In a study conducted by Pico et al.2 in 102 patients of which 74 were receiving HD and 28 peritoneal dialysis (PD), pruritus was found in 63% of patients, and it was more prevalent in patients receiving HD than PD (42% vs. 32%). Uremic pruritus can be generalized or localized and is not associated with a rash. The clinical manifestations of pruritus include excoriations, lesions of lichen simplex chronicus, and prurigo nodularis. Pruritus also contributes to koebnerization of acquired perforating dermatoses. It is generally accepted that itching is conveyed by very thin type C unmyelinated afferent nerve fibers that enter the spinal cord via the dorsal roots where sensory impulses are coordinated and integrated with those of other afferents.8 It has been proposed that the sensation of itch is also mediated by various chemical neurotransmitters.9 These chemical mediators include pentapeptide enkephalins, proteases, serotonin, and histamine.9 However, histamine is considered the major endogenous pruritogenic substance. There are a significant number of proposed etiologies for pruritus in ESRD including: integumentary changes related to xerosis, urochrome deposition, uremic toxemia, calcium and phosphate dysregulation, hyperthyroidism, mast-cell proliferation with a concomitant increase in histamine levels, dialysis component allergic reactions, and hypovitaminosis D.10Based on the fact that pruritus in renal patients is present in chronic but not acute renal failure, Tapia11 has suggested that pruritus may be due to a slowly accumulative metabolic process or hormonal derangement. Many have documented the role of divalent ions (calcium, phosphorus, and magnesium) in the pathogenesis of uremic pruritus by demonstrating higher contents of all three ions in the skin of pruritic uremic patients than in either normal volunteers or non-pruritic uremic patients.12-14 Mast cell changes and histamine might be involved in the pathogenesis of uremic pruritus. Mast cell hyperplasia in the bone marrow and skin has been reported to occur in patient's with chronic renal failure and dialysis patients.15, 16 It has also been reported that there is increased plasma histamine levels in patients with ESRD.17 They concluded that high histamine levels might result from mast-cell elaboration and thus mediate uremic pruritus. Histamine is the most extensively studied pruritogenic substance and the only one that has been definitely linked to pruritus. Stahle-Backdahl18 studied the role of histamine in 26 dialysis patients (18 with and eight without pruritus) and nine healthy controls. All dialysis patients were administered intradermal histamine, while control subjects were injected with saline. The patient with pruritus had a significantly stronger dose-dependent itch response to escalating histamine concentrations than did patients without pruritus or healthy controls. The perception of itching persisted and, in patients suffering from pruritus, was augmented by histamine administration, indicating an increased sensitivity to histamine in these patients. The histologic findings vary considerably with the duration and severity of the symptoms. Mild cases show superficial erosion of the keratin epithelial layer. Vigorous excoriation is capable of inducing epithelial ulceration with an accumulation of neutrophils and fibrin at the base of the ulcer. Protracted cases show varying degrees of irregular psoriasiform epidermal hyperplasia with vertical streaking of the papillary dermal collagen. Xerosis refers to the dry or roughened skin texture frequently seen in dialysis patients. It is a common cutaneous malady also associated with colder climates, excessive exposure to sun, excessive use of detergents, or pathologic entities such as ichthyosis and atopic dermatitis. Significant xerosis has been reported in 50–75% of the dialysis population.19 The characteristic features of xerosis include complaints of pruritus, and the appearance of dry, fissured skin with scaling. The appearance of xerotic skin has been likened to ‘cracked porcelain’. It is usually most pronounced over the extensor surfaces of the extremities. Xerosis may be complicated by fissures, ulcers, lichen simplex chronicus, cellulitis, and irritant or contact dermatitis. The pathophysiological mechanisms underlying the development of xerosis are essentially unknown. There are several studies that have proposed a direct relation between xerosis and stratum corneum water content. Park20 investigated the etiology of dry skin and the sweat glands in response to intradermal injection of pilocarpine nitrite, in 18 uremic patients with xerosis. They postulated that a thicker though fractured stratum corneum led to increased transepidermal water loss. They also reported a functional abnormality of the eccrine sweat glands, suggesting compromised eccrine secretion leading to epithelial dehydration. Their findings, however, have been refuted by Stahle-Backdahl18 and Ponticelli,5 who reported no correlation between xerotic skin and the stratum corneum water content. Histologic sections may show normal epithelium or mild-to-moderate epidermal hyperplasia with hyperkeratosis and acanthosis. Superimposed features of eczema or lichenification may be observed. Literally referring to the clinical appearance of fish scales, ichthyosis consists of the presence of generalized scale. Ichthyosiform dermatoses can be classified into two major types: the genetic ichthyosis and acquired ichthyosis. Acquired ichthyosis is associated with a variety of systemic conditions including lymphoma, sarcoidosis, lupus erythematosus, and uremia. The incidence and pathophysiology of ichthyosis in uremia is unknown. The pathology consists of epidermal hyperplasia and acanthosis with variable loss of the granular layer. A spectrum of pigmentary alterations have been described in the dialysis population including pallor, brown-to-slate-gray discoloration, yellowish (sallow) hue, and brownish hyperpigmentation in sun-exposed areas. A large number of uremia-related changes are responsible for cutaneous pigmentary changes. Pigmentary alteration occurs in 25–70% of dialysis population and increases over the duration of renal disease.1 Pico et al.2 reported that 70% of 102 dialysis patients manifested cutaneous pigmentary alteration. A yellow tinge was seen in 40% of the patients, diffuse hyperpigmentation of the sun-exposed areas was seen in 22%, and skin pallor was seen in 8% of the cases. These cutaneous alterations were seen equally in the HD and the PD groups, with the exception of the yellowish skin color, which was significantly more prevalent in the HD patients. Diffuse hyperpigmentation on sun-exposed areas has been attributed to an increase in melanin in the basal layer of the epidermis.21 This hyperpigmentation results from an increase in melanin production due to an increase in poorly dialyzable beta-melanocyte-stimulating hormone.22 The intensity of melanin pigmentation increases with respect to the duration of ESRD. The yellowish skin color has been attributed to retained liposoluble pigments such as lipochromes and carotenoids which are deposited in the dermis and the subcutaneous tissue.23 The brown-to-slate-gray discoloration noted in conjunction with ESRD has been attributed to hemosiderin deposition. The nails may show evidence of latent skin disease in psoriasis, lichen planus, alopecia areata and serious systemic disease in renal and hepatic insufficiency. The most consistent nail change ascribed to renal disease is the half-and-half nail syndrome.24Another less commonly described nail change associated with renal disease is Mees' lines characterized by partial leukonychia of the nail plate. Although, half-and-half nails are not always seen in renal failure, they occur in as many as 40% of the patients on dialysis.1 Several other studies have reported an incidence ranging from 16 to 50.6%.24-26 Pico et al.2 reported that the nail changes increased in prevalence with respect to time of dialysis and was significantly more pronounced in the HD group. It has also been reported by Nunley1 that half-and-half nails disappear several months after successful renal transplantation. The nails exhibit a whitish or normal proximal portion and an abnormally brown distal portion. The latter portion of the nail comprises more than one-third of the nail plate. The pathogenesis of half-and-half nail has been attributed to increased levels of melanocyte stimulating hormone (MSH).24Histologic sections show increased melanin pigment within the nail plate. Studies in skin biology continue to indicate the importance and the complexity of dermal–epidermal interface in healthy and diseased skin. The perforating dermatoses represent a disturbance in this relation, manifested by the histological hallmark of transepidermal elimination of dermal substance. It is a process in which the altered connective tissue component within the dermis (collagen or elastic fibers) is eliminated through the epidermis with little damage to surrounding structures.27 This may occur as an epi-phenomenon in several skin diseases including keratoacanthoma, healing wounds, granuloma annulare,28 or constitute the principle pathological alteration in five conditions regarded as the primary perforating dermatoses: perforating disorder of uremia (PDU), Kyrle's disease (KD), perforating folliculitis (PF), reactive perforating collagenosis (RPC), and elastosis perforans serpiginosa (EPS). The histopathologic features of each of the perforating disorders show great overlap and, indeed, many of the disorders are similarly associated with renal disease, diabetes mellitus, or both conditions.29, 30 Given the clinical and histopathological similarities among the primary perforating disorders, they will be discussed as a group. Noteworthy, historical, clinical, or histologic differences among these entities will be highlighted. In North America, the reported incidence of acquired perforating disorders varies from 4.531 to 10%32 of patients receiving maintenance HD. A similar incidence of 11% has been recently reported in Britain.32 There is predilection for this condition among African–American patients.30 The clinical features of PDU resemble the classic features of primary perforating disorders of KD, PF, and RPC. Generally, the skin lesions consist of grouped dome-shaped papules or nodules, 1–10 mm in diameter. The papules are often umbilicated with a central crust-filled crater. The papules may assume an annular or serpiginous orientation in EPS. Occasionally, verrucous plaques are seen distributed on hair-bearing areas subject to friction. Verrucous follicular based papules and plaques dominate the clinical picture of KD. In dark skin, brown or hyperpigmented papules are typically observed. The trunk and the extremities are the most commonly involved followed by the face and the scalp. The lesions of EPS are more commonly seen on the face or neck. Spontaneous resolution of well-developed individual lesions with the continued development of new lesions characterizes the clinical course of PDU. Most of these disorders are associated with diabetes mellitus and/or renal disease. Notable exceptions include EPS, which is associated with inborn errors of metabolism including Ehlers–Danlos disease, Marfan's disease, osteogenesis imperfecta as well as d-penicillamine administration and Down's syndrome. The pathogenesis of these disorders remains unknown. Numerous possible mechanisms have been proposed. Ultrastructural studies have contributed to the understanding of the pathophysiology of acquired perforating disorders. These studies have shown that different materials are consistently eliminated at different stages of the disease process.29, 33-37 The abundance of polymorphonuclear neutrophil remnants in the early stages of these disorders has led to the speculation that cellular dissolution of neutrophils with proteolytic enzyme release, including collagenase and elastase elaboration, may initiate the pathologic process.38 Factors responsible for creating or maintaining the focus of epidermal perforation could not be explained. In another study involving nine men and six women subjects with various perforating disorders of the skin, investigators reported elevated serum and lesional fibronectin levels.39 As fibronectin is chemotactic for neutrophils and is also capable of inciting epithelial migration and proliferation, it was postulated that dermal matrix deposition of fibronectin could be responsible for the disease process. Interestingly, both diabetes mellitus and ESRD have been shown to be associated with elevated serum fibronectin. Other proposed mechanisms include defects in vitamin A or D metabolism associated with renal disease capable of inducing faulty epithelial proliferation and differentiation.33, 34, 40, 41 The histologic features of each of the perforating disorders are variable and overlapping.30 The common histologic denominator of these five conditions is the transepidermal elimination of amorphous dermal material. As the elimination canal or extruded material is often focal, serial sections may be required to confirm the histologic diagnosis. The transepidermal elimination canal(s) may be single or multiple (Fig. 1) and be juxtaposed directly over the degenerated dermal material or assume an angled orientation eccentric to the dermal nidus of amorphous material. The eliminatory canal may assume a straight, angled, or corkscrew orientation either through the follicular actrotrichia or epithelium proper. Follicular based eliminatory canals with surrounding giant cells are more commonly seen in the clinical context of PF, EPS, or KD (Fig. 2). The basilar portion of the eliminatory canal often assumes a cystic (Fig. 3) or bulbous flask-like orientation (Fig. 4). The top of the canal may be represented as a crater or cup-shaped depression. The degenerated material consists of collections of acute and chronic inflammatory cells as well as degenerated collagen and elastin bundles. Eosinophilic stained elastin fibers generally predominate in EPS and basophilic stained collagen bundles in RPC.27 Low-power photomicrograph depicting two transepidermal elimination canals with surmounted fibrinopurulent debris. High-power photomicrograph of perforating folliculitis showing histologic sequelae consisting of perifollicular giant cells. Low-power photomicrograph depicting the cystic base of a perforating lesion. High-power photomicrograph of perforating disorder depicting bulbous follicular structure filled with fibrinopurulent debris. Calciphylaxis is a devastating and life-threatening condition of progressive cutaneous necrosis due to small- and medium-sized vessel calcification. It is usually complicated by secondary infection and sepsis.42 It is a well-recognized complication of secondary hyperparathyroidism in chronic renal failure.43, 44 The term ‘calciphylaxis’ was introduced in 1962 by Selye45 after work on experimental non-uremic rats. A few years later, a syndrome characterized by peripheral ischemic tissue necrosis and cutaneous ulceration was reported in uremic patients, and because of the resemblance to the animal model of Seyle46 it was termed calciphylaxis. It is noteworthy that the syndrome of calciphylaxis described in uremic patients only remotely resembles the experimental model of Seyle.47 Furthermore, the hallmark of calciphylaxis in humans is calcium deposition in the tunica media of arteries, which is absent from Selye's observation. Therefore, the term ‘calcific uremic arteriolopathy’ has been suggested as an alternative to calciphylaxis.48 In humans, there are two distinct clinical syndromes with similar histologic findings. The first is symmetrical ascending acral gangrene, often with painful plaques of the skin on shins. The second is a destructive necrotic calcifying panniculitis, affecting the subcutaneous fat typically in poorly nourished obese white diabetic women.49 Diagnosis is made based on a history of renal failure, clinical findings, elevated parathyroid hormone (PTH) level, elevated calcium phosphate product, and histologic features. The diagnosis is best made clinically because biopsy and radiographic findings maybe non-specific.50 Other terms used interchangeably with calciphylaxis include systemic calcification, uremic gangrene syndrome, calcinosis cutis, necrotizing panniculitis, and cutaneous necrosis syndrome. Calciphylaxis occurs in both peritoneal and HD patient and can occasionally occur prior to dialysis.51, 52 The incidence is reported to be 1% in patients with chronic renal failure53 and 4% of patients receiving HD.51, 54 Women are affected more commonly than men (3 : 1).55 Firm, bilaterally symmetric non-ulcerating painful preinfarctive ischemic plaques51 that appear as mottling or violaceous discoloration on the extremities are seen. A reticulated pattern resembling livedo reticularis (retiform purpura) is often seen. Flaccid or hemorrhagic bullae may form over ischemic tissue.56 Lesions gradually enlarge over weeks to months and when debrided deep ulcers reaching down to the fascia are seen. Ischemic skin frequently becomes secondarily infected; infection can remain localized or become invasive, causing cellulitis and bacteremia.49, 50, 56 Peripheral pulses are preserved distal to the area of necrosis. Myopathy, hypotension, fever, dementia, and infarction of the central nervous system, bowel, or myocardium have been described in association with cutaneous necrosis.57 This condition is termed systemic calciphylaxis.58 The course tends to be progressive, despite all therapeutic interventions. Pain is a constant feature associated with ischemia and secondary infection. In advanced disease, gangrene of fingers, toes,59 and penis60 may result in autoamputation. Local infection and sepsis are common complications. Overall, the prognosis is poor with 5-year mortality rates reported to be greater than 50%.56, 61 Proximal locations of necrosis (thighs, buttocks, and trunk) are associated with an unfavorable prognosis with 63% mortality compared to acral locations (calves, forearms, fingers, toes, and penis) with 23% mortality.53 The pathogenesis of calciphylaxis is multifactorial and remains speculative.42, 47 In animal models, it is described as a condition of induced systemic hypersensitivity in which tissues respond to appropriate inciting agents with calcium deposition. Most patients have secondary hyperparathyroidism and an elevated calcium phosphate product, which is thought to be a major inciting factor of calciphylaxis.47, 53, 62 The tissues are sensitized by parathyroid hormone, with subsequent deposition of calcium in the media of small- and medium-sized arteries in the subcutaneous tissue leading to ischemic tissue necrosis, ulceration, and peripheral gangrene.47, 53, 62 Functional protein C deficiency has also been hypothesized to produce a hypercoagulable state, which contributes to thrombosis, resulting in skin ischemia, necrosis, and digital gangrene in systemic calciphylaxis.56, 63 Other cited risk factors are low serum albumin, elevated serum phosphate, and serum alkaline phosphatase levels.64 Biopsies are discouraged due to reported development of ulceration in the region of the incision.51 Also, single biopsy may be negative in the presence of the disease necessitating multiple biopsies.51 The histologic features of calciphylaxis are suggestive but not pathognomonic.42, 43 Incisional biopsies of early lesions show subtle and almost unapparent histologic changes. Late lesions characteristically show epidermal ulceration, dermal necrosis, and mural calcification with intimal hyperplasia of small- and medium-sized blood vessels in the dermis and subcutaneous tissue.43, 65 The size of affected vessels ranges from 0.02 to 0.60 mm. The most common finding is acute and chronic calcifying septal panniculitis (Fig. 5). Delicate calcium deposition surrounding lipocytes or global calcification of septal capillaries occurs in subcutaneous tissue.42 Endovascular fibroblastic proliferation is more common in advanced lesions.65 Fibrin thrombi can be noted in subcutaneous and superficial dermal vessels or calcified vessels.42, 43, 65 Intermediate-power photomicrograph depicting calcifying panniculitis of calciphylaxis. The following conditions should be considered in the differential diagnosis: autoimmune vasculitis, peripheral vascular disease, diabetes mellitus, pyoderma gangrenosum, cholesterol embolization syndrome, systemic vasculitis, cytofibrinogenemia, and systemic oxalosis.43, 49 Calcium deposition in the tunica media of the arteries (Mönckeberg's sclerosis) poses the greatest diagnostic differential challenge. The lesions of Mönckeberg's preferentially involve the medium-sized muscular arteries and tend to eccentrically affect the vascular wall without thrombosis (Fig. 6). In contrast, calciphylaxis tends to involve the walls of medium- and small-sized arteries to fill and/or obstruct the lumen and are often associated with superimposed thrombi. Additional changes frequently noted with respect to calciphylaxis include varying degrees of epidermal ulceration with ischemic necrosis of the dermis. The crystalline deposits of oxalosis consist of calcium oxalate. While found in blood vessels, these crystals are typically yellow and show bireferengent properties on polaroscopy (Fig. 7). High-power photomicrograph of intramural calcification seen in Mönckeberg's calcific sclerosis. High-power photomicrograph depicting perivascular crystalline deposits of oxalosis. The development of blistering disorders among patients on HD is well documented. Patients with chronic renal failure can develop two types of bullous dermatosis: a ‘true’ porphyria such as porphyria cutanea tarda (PCT), and ‘dialysis-porphyria’ which is also known as ‘pseudoporphyria’.21, 66-71 PCT is a vesiculobullous skin disorder characterized by an acquired or inherited autosomal dominant defect in heme biosynthesis, involving a deficiency in the enzyme uroporphyrinogen decarboxylase.72 It has been reported in patients with chronic renal failure being treated with HD with a prevalence ranging from 1.2 to 18%.70 Pseudoporphyria, in the setting of chronic renal disease, was first described in 1975 by Korting69 who reported a bullous disease resembling PCT in patients with chronic renal failure. Later, Gilchrest, Rowe, and Mihm66 described ‘bullous dermatosis of hemodialysis’. In addition, cases of chronic renal failure-associated pseudoporphyria without accompanying dialysis have been reported.68 Pseudoporphyria has been reported to also be precipitated by certain drugs, including tetracyclines, furosemide, nalidixic acid, dapsone, pyridoxine, and naproxen.73 The two groups are clinically and histologically similar and are characterized by a blistering photosensitive skin rash.74 Therefore, dialysis patients presenting with bullous skin lesions require careful evaluation. PCT diagnosis is based on a predominance of plasma uroporphyrin, urine uroporphyrins I and III, heptacarboxyl porphyrin, and elevated fecal heptacarboxyl porphyrin and isocoproporphyrin.75 In pseudoporphyria, porphyrin levels in the serum, urine, or stool remain normal.42, 76 Therefore, the most important diagnostic test in the evaluation of the cutaneous porphyrias is the measurement of porphyrins in urine, stool, and blood.77 The clinical presentation of PCT and pseudoporphyria is very similar.76 Crops of delicate 0.25- to 1-cm vesicles occur most commonly on the extensor forearms and dorsal hands. Other areas such as the fingers, extensor legs, upper chest, or face may be involved as well. Milia, atrophic scarring, and hyperpigmentation may develop.74 However, in pseudoporphyria, most patients do not manifest hypertrichosis or sclerodermoid plaques.78 The exact pathogenesis of PCT in hemodialyzed patients is unknown. Azotemia has been suggested as a possible mechanism by decreasing the activity of uroporphyrinogen decarboxylase which catalyses the first decarboxylation pathway of the porphyrin pathway.67, 74 Another proposed mechanism is an impaired ability to excrete porphyrins in patients with renal failure. Porphyrins are poorly dialyzed by conventional ‘low-flux’ dialysis membranes because of binding with high-molecular weight proteins.49, 79 Porphyria has also been associated with an increase in plasma levels of 5-aminolevulinic acid and porphobilinogen in the serum of patients undergoing HD.80 The origin of pseudoporphyria complicating HD remains unclear.66 Photoactive drugs such as furosemide and tetracycline have been commonly implicated.49, 81 Dermal microangiopathic changes and decreased subcutaneous oxygenation during HD might facilitate frictional blistering.73 Other proposed factors are aluminum hydroxide78, 82 erythropoietin83 and susceptibility to oxygen-free radicals.84 One study suggests that altered zinc and associated antioxidant status following dialysis leads to a partial loss of activity of 5-aminolevulinate dehydratase and deaminase activities causing pseuodporphyria.80 Histopathologic features of PCT and pseudoporphyria are identical.73, 85 In both cases, subepidermal separation with or without festooning of dermal papillae is seen with scant-to-mild lymphocytic perivascular infiltrate76 (Fig. 8). Periodic acid–Schiff-positive thickening of blood vessel walls and sclerosis of collagen may be noted.77 However, blood vessel wall thickening and sclerosis of collagen has been reported to occur less frequently in pseudoporphyria when compared to PCT.77 Intermediate-power photomicrograph depicting cell-poor subepi- dermal blister with festooned dermal papillae in psuedoporphyria. Positive direct immunofluorescence is suggestive, but not requisite, for the diagnosis of pseudoporphyria. Direct immunofluorescence reveals little difference between PCT and pseudoporphyria.73 Granular deposits of immunoglobulin G (IgG) and C3 are present at the dermoepidermal junction and in the upper dermal vasculature presenting as thickened doughnut-like blood vessels.66, 77 IgM, IgA, and fibrinogen may also be deposited. Cystoid bodies, if present, are more readily apparent in PCT than in pseudoporphyria.77 Indirect immunofluorescence is uniformly negative. This skin condition is characterized by diffuse thickening of the skin with hyperpigmentation, occasional discrete papules, and nodules most prominent on the extremities. The condition was recently described by Cowper,86, 87 who identified renal transplant and renal dialysis patients who developed a scleromyxedema-like cutaneous fibrosing disorder. This occurs in patients on renal dialysis or post renal transplantation. Ages have been reported between 31 and 74 years at time of disease onset.86 There is extensive thickening and hardening of the skin associated with brawny hyperpigmentation. In some cases, distinct papules and subcutaneous nodules were also seen. The extremities are involved in all cases. The torso is less commonly involved. Cutaneous changes consisted largely of indurated plaques and papules on the extremities and trunk. Systemic findings seen in scleromyxedema, which the condition resembles in some respects, were absent.86, 87 Although unknown, various mechanisms have been proposed including diasylate and/or dialysis membranes. Histopathologic features resemble those of scleromyxedema. There is an increase in dermal fibroblast-like spindled cells associated with collagen remodeling and mucin deposition. Haphazardly arranged dermal collagen bundles are seen with surrounding clefts and a strikingly increased number of similarly arranged spindled and plump fibroblast-like cells. CD-34-positive dermal dendrocytes are abundant, with dendritic processes aligned with elastic fibers and around collagen bundles in a dense network. Factor XIIIa and CD-68-positive mono- and multinucleated cells are also present in increased numbers. Electron microscopy shows increased elastic fibers closely apposed to dendritic cell processes. The entire dermis is commonly involved, with increased spindle cells, collagen, mucin, and elastic fibers extending through the subcutis along the septa of fatty lobules.86" @default.
W2009180675 created "2016-06-24" @default.
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W2009180675 title "A current review of the cutaneous manifestations of renal disease" @default.
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W2009180675 doi "https://doi.org/10.1034/j.1600-0560.2003.00109.x" @default.
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W2009180675 countsByYear W20091806752015 @default.
W2009180675 countsByYear W20091806752016 @default.
W2009180675 countsByYear W20091806752017 @default.
W2009180675 countsByYear W20091806752018 @default.
W2009180675 countsByYear W20091806752019 @default.
W2009180675 countsByYear W20091806752020 @default.
W2009180675 countsByYear W20091806752021 @default.