Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology, Seventh Edition

SECTION 18

Skin Signs of Renal Insufficiency

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Classification of Skin Changes

image Acute renal failure

image Edema

image Uremic frost (deposition of urea crystals on skin surface in severe uremia)

image Chronic renal failure

image Edema

image Uremic frost

image Calciphylaxis

image Bullous disease of hemodialysis (pseudoporphyria, see Section 23)

image Nephrogenic fibrosing dermopathy

image Acquired perforating dermatosis

Calciphylaxis ICD-9: 275.49 image ICD-10: E83.59 image

image Calciphylaxis is characterized by progressive cutaneous necrosis associated with small- and medium-sized vessel calcification and thrombosis.

image It occurs in the setting of end-stage renal disease, diabetes mellitus, and secondary hyperparathyroidism. Most often follows initiation of hemo- or peritoneal dialysis.

image Precipitating factors: glucocorticosteroids, albumin infusions, IM tobramycin, iron dextran complex, calcium heparinate, vitamin D.

image Preinfarctive lesions show mottling or livedo reticularis pattern, dusky red (Fig. 18-1A).

image Turn into black, leathery eschar (Fig. 18-1B) and ulcer with tightly adherent black or leathery slough. Ulcers enlarge over weeks to months; when debrided reach down to fascia and beyond; areas of plate-like induration can be palpated surrounding infarcted or ulcerated lesions (Fig. 18-2).

image Extremely painful.

image Lower extremities, abdomen, buttocks, penis.

image Azotemia. Calcium X phosphate ion product usually elevated. Parathormone levels usually but not always elevated. Dermatopathology: calcification of media of small- and medium-sized blood vessels in dermis and subcutaneous tissues.

image Slowly progressive, despite therapy. Ulcus become secondarily infected.

image Management: treatment of renal failure, partial parathyroidectomy when indicated, debridement of necrotic tissue.

image

Figure 18-1. Calciphylaxis (A) Early stage. An area of mottled erythema, starburst-like, and reminiscent of livedo reticularis with two small ulcerations. Patient has chronic renal failure and is on hemodialysis. Even at this early stage, lesions are extremely painful. (B) Calciphylaxis, more advanced lesion. An area of jagged necrosis on the lower leg in a patient with diabetes and chronic renal failure who is on hemodialysis. The surrounding skin is indurated and represents a plate-like subcutaneous mass that is appreciated only upon palpation.

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Figure 18-2. Calciphylaxis, extensive Lesions are ulcerated, the surrounding skin is indurated, best seen on left thigh where skin is hairless. Similar lesions are also found on the abdomen.

Nephrogenic Fibrosing Dermopathy (NFD) ICD-9: 701.8 image ICD-10: L90.8 image

image NFD is a fibrosing disorder in patients with acute or chronic renal failure.

image Most patients receiving hemodialysis, peritoneal dialysis; in acute renal failure, NFD occurs without dialysis.

image It is part of a wider spectrum of nephrogenic systemic fibrosis involving the heart, lungs, diaphragm, skeletal muscle, liver, genitourinary tract, and central nervous system.

image Etiology unknown but exposure to gadodiamide containing contrast media for MR angiography is a strong association. Gadodiamide is found only in lesions and not in normal tissue. Myofibroblasts and fibrogenic cytokines (e.g., transforming growth factor β) may be involved in the pathogenesis.

image NFD is characterized by acute onset, brawny indurations, plaque-like or nodular, bound down upon palpation (Fig. 18-3); up to 20 cm and more in diameter, with an uneven rippled surface.

image Mostly on lower extremities, less often on upper extremities and torso but not the face.

image Tingling, tender, often painful.

image Differential diagnosis: morphea, pretibial myxedema, lipodermatosclerosis, panniculitis.

image Course is chronic, unremitting; prognosis guarded.

image Therapy unknown. Imatinib may be beneficial

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Figure 18-3. Nephrogenic fibrosing dermopathy A brawny plate-like induration bound down upon palpation, with an uneven surface on the legs. This patient had end-stage chronic renal failure and was on hemodialysis.

Acquired Perforating Dermatosis* ICD-9: 709.8 image ICD-10: L87.0 image

image Occurs in chronic renal failure and diabetes mellitus; in up to 10% of patients undergoing hemodialysis.

image Chronic pruritic condition triggered by trauma.

image Umbilicated papules with central hyperkeratotic crust (Fig. 18-4).

image Transepidermal elimination of collagen.

image Relationship with other perforating disorders not clear.

*For more detailed information, see Minocha JS and Schlosser BJ: Chapter 69 in Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, and Wolff K (eds.). Fitzpatrick’s Dermatology in General Medicine, 8th ed. New York, McGraw-Hill, 2008:727–731.

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Figure 18-4. Acquired perforating dermatosis in a patient undergoing hemodialysis There are purpuric umbilicated papules with a central hyperkeratotic crust.