Renal Disease and the Skin




Abstract


Chronic kidney disease (CKD) has numerous deleterious systemic effects including impaired function of the heart, brain, and nervous system, altered hormonal balance and bone metabolism, and a decreased ability to resist infections. Cutaneous disorders are common in patients with CKD and can be due to various genetic or acquired conditions or metabolic abnormalities. These dermatologic manifestations can be summarized in three categories: (1) dermatologic signs of diseases causing renal failure; (2) dermatologic conditions relatively unique to uremia; and (3) cutaneous diseases in renal transplant recipients related to immunosuppression and/or drugs used to immunosuppress. With the mortality of CKD decreasing and prevalence increasing, dermatologists will continue to encounter cutaneous manifestations of CKD and may be the first to identify and treat many of these conditions.




Keywords

Acquired perforating dermatoses, Alopecia, Calcinosis cutis, Calciphylaxis, Chronic kidney disease, Dialysis, End-stage renal disease, Immunosuppression, Kyrles disease, Lindsay’s nails, Nephrogenic systemic fibrosis, Pigmentary alteration, Pruritus, Pseudoporphyria, Renal transplant recipients, Uremic frost, Xerosis

 





Key Points





  • Skin manifestations of chronic kidney disease (CKD) are commonly encountered and can be due to (1) the cause of the underlying renal disease, either acquired or heritable; (2) conditions unique to uremia; or (3) immunosuppressive therapies and/or immunosuppression itself in renal transplant recipients.



  • Diabetes and hypertension account for most cases of end-stage renal disease (ESRD) in the United States; however, many diseases, such as amyloidosis, connective tissue diseases, hepatitis C and B viral infections, and numerous genetic diseases may also cause ESRD. These conditions often possess characteristic cutaneous findings, which may be the first clue to an underlying kidney disease.



  • Skin findings such as alopecia, nail changes, pigmentary alteration, pruritus, and xerosis are not specific to uremia per se, but are frequently observed in patients with impaired renal function and impact quality of life.



  • Calciphylaxis is a rare but severe syndrome with high morbidity and mortality that involves calcium deposition in small vessels within the dermis and subcutaneous tissue, leading to exquisitely tender, retiform purpuric plaques that frequently ulcerate.



  • Acquired perforating dermatoses represent a spectrum of disorders with transepidermal elimination of material from the dermis with little damage to surrounding tissue, clinically presenting as keratotic lesions most commonly on the trunk and extremities.



  • Nephrogenic systemic fibrosis is characterized by thickened collagen in the skin and other organs, hyperpigmented, brawny plaques and papules most frequently starting on the extremities, and an association with exposure to gadolinium-based contrast agents in patients with renal compromise.



  • Bullous diseases in CKD include porphyria cutanea tarda (PCT), pseudoporphyria, and bullous disease of dialysis.



  • Renal transplant recipients are at risk for medication-related cutaneous changes, infections, and cutaneous malignancies secondary to immunosuppression.





Introduction


Chronic kidney disease (CKD) has numerous deleterious systemic effects including impaired function of the heart, brain, and nervous system, altered hormonal balance and bone metabolism, and increased susceptibility to infections. Cutaneous disorders are common in patients with CKD and can be due to various genetic or acquired conditions or metabolic abnormalities. These dermatologic manifestations can be summarized in three categories: (1) dermatologic signs of diseases causing renal failure ( Table 38-1 ); (2) dermatologic conditions relatively unique to uremia ( Table 38-2 ); and (3) cutaneous disorders in renal transplant recipients (RTR) related to immunosuppression and/or drugs used to immunosuppress ( Table 38-3 ). This chapter will focus on dermatologic conditions unique to uremia, while disorders in categories 1 and 3 are summarized in table format only. Given that mortality from CKD is decreasing, the prevalence of this disease is increasing. Therefore, dermatologists will continue to encounter cutaneous manifestations of CKD and may be the first to identify and treat many of these conditions.



TABLE 38-1

Dermatologic Conditions in Diseases Causing Chronic Kidney Disease




























































































































































































Disease Dermatologic Manifestations Renal Features
Metabolic Disorders
Diabetes mellitus Acanthosis nigricans Diabetic nephropathy
Eruptive xanthomas Nephrotic syndrome
Necrobiosis lipoidica
Diabetic dermopathy
Bullous diabeticorum
Amyloidosis Macroglossia Nephrotic syndrome
Purpura (most classically in the periorbital region)
Atherosclerosis/cholesterol emboli Blue toes Renal emboli with hematuria and eosinophiluria
Cutaneous necrosis
Retiform purpura
Splinter hemorrhage
Connective Tissue Diseases
Systemic sclerosis Calcinosis cutis Malignant hypertension
Cutaneous sclerosis Renal crisis
Distal digital infarcts
Nailfold capillary changes
Sclerodactyly
Mat telangiectases
Salt-and-pepper depigmentation
Polyarteritis nodosa Palpable purpura Glomerulonephritis
Nodules Vasculitis
Ulcers
Systemic lupus erythematosus Acute cutaneous lupus erythematosus (butterfly rash) Glomerulonephritis
Chronic cutaneous lupus (discoid lupus) Nephrotic syndrome
Livedo reticularis
Subacute cutaneous lupus erythematosus
Granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis) Palpable purpura Glomerulonephritis
Petechiae Vasculitis
Saddle nose deformity
Strawberry gums
Hepatitis Viruses
Hepatitis C Lichen planus Glomerulonephritis
Porphyria cutanea tarda:
Photodistributed
Milia
Sclerodermatous changes
Vesicles/bullae
Hypertrichosis (on temples)
Necrolytic acral erythema
Mixed cryoglobulinemia:
Digital infarcts
Livedo reticularis
Palpable purpura
Hepatitis B Polyarteritis nodosa Glomerulonephritis
Genetic Disorders
Fabry’s disease Angiokeratomas of lower abdomen, hip, and inguinal region Varying degrees of proteinuria
Urinary globotriaosylceramide
Inheritance: X-linked recessive Cortical and parapelvic cysts
Defect: α-galactosidase-A deficiency Renal failure is more common in men
Birt–Hogg–Dubé Trichodiscomas Renal cancers of variable histology
Inheritance: autosomal dominant Fibrofolliculomas
Defect: folliculin gene (FLCN) mutation Acrochordons (see Chapter 17 )
Tuberous sclerosis Facial angiofibromas Angiomyolipomas
Inheritance: genetically heterogeneous; autosomal dominant transmission with high spontaneous mutation rate Connective tissue nevi Renal cysts
Hypopigmented macules (“ash-leaf macules”) Polycystic kidneys
Shagreen patch Renal cell carcinoma
Defect: genes TSC1/TSC2 with protein products hamartin and tuberin Periungual fibromas (see Chapter 17 )
Nail–patella syndrome Nail dysplasia: Varying degrees of proteinuria
Inheritance: autosomal dominant Triangular lunulae Renal tubular defects
Defect: LIM-homeodomain protein LMX1B Hypoplastic nails
Lack of creases over distal interphalangeal joints
Hereditary multiple leiomyomas of skin Multiple cutaneous leiomyomas, typically regionally grouped (see Chapter 17 ) Renal cell carcinoma
Inheritance: autosomal dominant
Defect: mutation in gene encoding fumarate hydratase


TABLE 38-2

Dermatologic Diseases Associated with Uremia





Alopecia
Calcinosis cutis
Calciphylaxis
Kyrle disease (acquired perforating dermatosis)
Nail changes:


  • Beau’s lines



  • Half-and-half nails (Lindsay’s nails)



  • Nailfold capillary abnormalities

Nephrogenic systemic fibrosis
Pruritus-related skin changes
Pigmentary alteration
Porphyria cutanea tarda
Pseudoporphyria
Xerosis
Uremic frost

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Sep 15, 2019 | Posted by in Dermatology | Comments Off on Renal Disease and the Skin

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