Ichthyosiform Sarcoidosis with Renal Involvement

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Introduction: The ichthyoses, also called disorders of keratinization, are a heterogeneous group of disorders characterized by generalized skin scaling. The great majority of ichthyoses are inherited, but acquired forms can develop in the setting of malignancy, autoimmune or infectious diseases. Case Description: A 51-year-old man presented to the hospital for evaluation of acute kidney injury. The skin exam showed xerosis with hyperpigmented scales lesions on his arms, legs, abdomen, and back (Figure 1-A). Initial workup revealed the following: creatinine 6.27 mg/dL (baseline 0.8 mg/dL), calcium 15.1 mg/dL, PTH 9.1 pg/mL (10-55 pg/mL), vitamin D 1,25 dihydroxy 97.3 pg/mL (20-76 pg/mL), and angiotensin-converting enzyme 120 u/L (14-82 u/L). Urinalysis was positive for proteinuria with a urine protein/creatinine ratio of 900 mg/g Cr. Renal biopsy demonstrated diffuse granulomatous interstitial nephritis (Figure 2-A). Immunofluorescence showed granular capillary wall IgG. Electron microscopy showed podocyte foot process effacement and subepithelial electron-dense deposits (Figure 2-B). Skin biopsy revealed hyperkeratosis with nonnecrotizing granulomas. The patient was diagnosed with ichthyosiform sarcoidosis, granulomatous interstitial nephritis, and secondary membranous nephropathy. He was started on prednisone 60 mg daily. His serum creatinine and serum calcium returned to normal with significant improvement in his ichthyosis (Figure 1-B). Discussion: Ichthyosiform lesions are one of the extremely rare cutaneous manifestations of sarcoidosis that may precede or coincide with the diagnosis of systemic sarcoidosis. Renal manifestations include abnormal calcium metabolism, nephrolithiasis and nephrocalcinosis, acute interstitial nephritis, and membranous glomerulonephritis.

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