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Copy of Dermatologic Manifestations of Renal Diseases

Dermatologic Manifestations of Renal Disease
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heba h

on 3 November 2013

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Transcript of Copy of Dermatologic Manifestations of Renal Diseases

Dermatologic Manifestations of Renal Diseases
Skin changes in
Renal failure
and dialysis
Skin changes
in renal
transplant
Renal failure and dialysis
Pigmentation
Anaemia presenting as pallor is an early and
common sign in renal failure.
A muddy brown hyperpigmentation develops in many cases,
attributed to retention of chromogens and deposition of melanin .
Increased nail pigmentation, usually confined to
the distal aspect, occurs in a proportion of patients .
This distal brown or more normal red colour, combined with a proximal white appearance gives rise to the ‘half-and-half’ nails, a distinctive pattern seen in about 10% of patients with renal failure
Purpura due to a mild thrombocytopenia or more marked platelet dysfunction is common and may be partly corrected by dialysis.

Wound healing is prolonged and patients may be more susceptible to pressure sores
Pruritus
Uremia is the most common metabolic cause of pruritus.
Generalized, severe pruritus occurs in about one-third
of renal failure cases .
Up to 85% of patients on haemodialysis
suffered from itching in one study , one-third before dialysis,
the others after;
12% had reduced pruritus after 6 months’ dialysis.
In dialysis, lowering the magnesium concentration of the dialysate has been reported as helpful .
In intractable itching, UVB radiation is an effective therapy , benefit being associated with a reduction of skin phosphorus to normal values.
Oral cholestyramine and activated charcoal , Erythropoietin therapy , Naltrexone and Gabapentin has recently been demonstrated to be effective in uraemic pruritus
Xerosis
Significant xerosis occurs for unknown reasons in 50-92% of the dialysis population. Some patients may develop acquired ichthyosis.
Some authors have suggested that ESRD-associated xerosis may be a result of a decrease in water content in the epidermis.
Clinical and histologic evaluations have shown an overall decrease in sweat volume in patients with uremia, as well as atrophy of sebaceous glands.
These changes can allow dehydration of the stratum corneum

Many patients respond to routine use of emollients.
Acquired Perforating Dermatosis
A perforating disorder variously described
as acquired reactive perforating collagenosis, Kyrle’s disease or
perforating folliculitis occurs in renal failure, often there is diabetes,
diabetic nephropathy or retinopathy.
It is a chronic pruritic conditions triggered by trauma
Many patients exhibit the Koebner phenomenon and up to 10% of dialysis patients may be affected.
The cutaneous lesions consist of umbilicated hyperpigmented
papules up to 1 cm in diameter with a central keratinous plug . The extensor surfaces of the limbs are more commonly
affected but the trunk and face may be involved.
Treatment by renal transplantation, if it is ineffective topical retenoids or broadbanb UVB can be used .
NEPHROGENIC FIBROSING DERMOPATHY
NFD or more recently termed Nephrogenic Systemic Fibrosis has been found only in patients with renal disease.
Most affected individuals have been on hemodialysis, and many have restarted dialysis after failure of a renal transplant.
NFD also has been described in a few patients with ARF.
Clinically, patients progressively develop erythematous sclerotic dermal indurated plaques, sometimes with finger-like projection on the arms and legs.
Nodules and contractures occur in more advanced disease with sparing of the head and neck.
Pruritus is a common feature.
The skin can have a peau d'orange appearance, and plaques may have an amoeboid advancing edge. The skin is often shiny and hard to the touch. A woody consistency is typical.
Plasmapheresis, IVIG, thalidomide, UVA with or without psoralen, extracorporeal photochemotherapy, cyclosporine and cyclophosphamide have been used in individual cases or small case series, with varying results
Porphyria Cutanea Tarda
True PCT has been reported to occur in patients on dialysis. An increased incidence of PCT should be expected to occur in ESRD.
Healing of crusted erosions and blisters leaves milia, hyperpigmented patches, and hypopigmented atrophic scars.
In patients with CRF, porphyrins may not be effectively cleared by hemodialysis or excreted in the urine.
Deferoxamine may lower serum porphyrin levels in some patients.
Others may require renal transplantation to obtain complete resolution of symptoms.
Bullous dermatosis of dialysis or pseudoporphyria may occur in up to one-fifth of patients with chronic renal failure undergoing hemodialysis who are typically develop these lesions only after months to years of maintenance dialysis regimens.
Bullous Disease of Dialysis
This condition is often clinically indistinguishable from PCT with marked skin fragility , Vesicles and bullae filled with clear or hemorrhagic fluid and exudative erosions formation on sun-exposed skin occur chiefly on the dorsal hands, although the scalp, face, and neck also may be affected.
However, Milia, dyspigmentation, and hypertrichosis is less common, and plasma porphyrin levels are typically normal.
We can treat the patient by Erythropoietin or N-acetylcysteine along with Sunlight avoidance .
Calciphylaxis
Metastatic calcification of the skin in CRF results from secondary or tertiary hyperparathyroidism. Abnormally elevated levels of PTH may trigger deposition of crystalline calcium pyrophosphate in the dermis, subcutaneous fat, or arterial walls.
Vascular calcification is common in patients with long-term CRF and seldom symptomatic. Occasionally, calcified vessels may thrombose acutely, resulting in calciphylaxis.
This acute thrombosis produces livedoid areas that are excruciatingly painful due to ischemia, and quickly become hemorrhagic and ulcerate . Calciphylaxis is associated with a high mortality, particularly when the skin of the trunk is involved.
Treatment of calciphylaxis includes analgesia, debridement of gangrenous tissue, and parathyroidectomy.
Renal transplant recipients
Immunosuppression-related disorders
Some infections, such as candidal infection, herpes
simplex infection, and impetigo are prominent during the first
post-transplant year.
Dermatomycoses, herpes zoster and folliculitis
are more common after the first post-transplant year
The incidence of warts increased steadily after transplant, such that of patients transplanted for more than 5 years, 92% were found to have warts and 65% had more than five each . Warts may become unusually florid , and recalcitrant to therapy.
Fungal organisms are the most common cause of infection in the RTR, occurring in 7-75%.
Pityriasis versicolor has been shown to be the most common fungal infection and occurs in 18-48% of RTRs, which is a higher rate 2-3 times more often than the general population.
Therapeutic options for these infections include antimicrobials, surgical debridement, and/or a reduction in immunosuppression.
Skin Tumors
Transplant recipients are at greatly increased risk of developing skin Malignancies, and most are primary cutaneous malignancies.
Medication-related disorders
Patients are at heightened risk for developing opportunistic infections during the first 6 months after transplantation because of the use of higher doses of immunosuppressive agents.
Nonmelanoma skin cancer
Squamous cell carcinoma (SCC) is the most common cutaneous malignancy in RTRs and occurs 50-250 times more frequently in the RTR than in the general population. In contrast, basal cell carcinoma occurs 6-10 times more frequently in the RTR.
Nonmelanoma malignancies occur at a younger age in RTRs and are characterized by a more rapid and aggressive course, a higher recurrence rate, and a greater metastatic potential.
Management of nonmelanoma skin cancers includes sun avoidance, use of broad-spectrum sunscreens, early detection of malignant and precancerous lesions, and aggressive therapy. Complete surgical excision with margin control is necessary.
Kaposi sarcoma
Kaposi’s sarcoma affecting the skin as well as other organs is well recognized after transplantation; it was reported in 3% of
kidney recipients in one series , and has been documented as being between 84- and 500-fold commoner in subjects with a transplant compared to the normal population
The highest incidence is in the first year after transplantation. Kaposi sarcoma usually appears 2-24 months after transplantation. The development of Kaposi sarcoma in RTRs is associated with human herpes virus 8
Therapeutic options for the RTR with Kaposi sarcoma include cessation of immunosuppressive medications, radiotherapy, chemotherapy, excision, and cryotherapy.
Melanoma occurs 2-9 times more frequently in the transplant population than in the general population .
OTHER
Lymphomas of both B (associated with Epstein–Barr virus) and T–cell lineage (often CD30+) are reported
Merkel cell tumours also seem to be more frequent and carry a poor prognosis;








increased risk up to 80-fold greater than the normal population has been suggested.
Appendageal tumours are
common, as is sebaceous gland hyperplasia.

Sebaceous carcinomas are overrepresented in organ recipients
Uremic frost
Uremic frost is commonly found in the beard or on other parts of the face, neck, and trunk as fine white-to-yellow crystals that dissolve readily when challenged by a drop of water.
Treatment by Dialysis .
The most common presenting sign of porphyria cutanea tarda is fragility of sun-exposed skin after mechanical trauma, leading to erosions and bullae, typically on hands and forearms and occasionally on face or feet
Cushingoid changes
Gingival hyperplasia
Disorders of the pilosebaceous unit, including acne, folliculitis, hypertrichosis, keratosis pilaris, sebaceous gland hyperplasia, epidermal cysts
The tumors usually start on the skin of the distal portions of the lower extremities as unilateral or bilateral bluish-red (hematoma-like) macules.
These lesions tend to progress slowly both horizontally and vertically and develop into firm plaques and, eventually, into nodules.
During tumor progression the color changes to brownish and the skin overlying the tumor becomes hyperkeratotic and, in particular on the lower extremities, may ulcerate. Surrounding the tumor there is frequently a pitting edema which may evolve into fibrosis

Fitzpatrick's Dermatology
Rook's Textbook of Dermatology
Bolognia Dermatology
http://emedicine.medscape.com/article/1094846-overview
References:
Thank you for your listening !
Actinic keratoses also occur at a younger age and develop at a faster rate in RTRs. Actinic keratoses frequently have more severe cytologic atypia and may have more rapid progression to SCC.
Cutaneous Manifestations of Renal Diseases
Dr. Heba Bosbait
MSc in Clinical Dermatology, Cardiff, UK
National Guard Hospital
Department of Dermatology
Full transcript