Pityriasis rubra pilaris with mesangial glomerulonephritis:
Pityriasis rubra pilaris (PRP) is a group of uncommon, chronic dermatosis of juvenile or adult onset. It presents with scaling (pityriasis), reddish-orange patches (rubra), hyperkeratosis around hair follicle (pilus), and palmoplantar keratoderma. PRP may co-exist with various autoimmune disorders including dermatomyositis, vitiligo, and hypoparathyroidism. However, its association with the renal disorder is rare.

The present case has been reported in the Indian Journal of Nephrology. A 10-year-old boy presented with hematuria, oliguria, and anasarca for 4 weeks and few episodes of generalized tonic-clonic seizure for 2–3 days. There was no history of sore throat or skin infections prior to this illness.

A few scaly, bilateral symmetrical, and nonitchy skin lesions were noted on elbows, knees, hands, and feet. The investigation revealed 4+ urinary albumin and 18–20 red blood cells/hpf. The 24 h urine albumin was 2114 mg.

His serum albumin was 1.8 g/dl and cholesterol was 302 mg/dl. Serum urea and creatinine were 39 mg/dl and 0.6 mg/dl, respectively. Antistreptolysin O titer on repeated occasion was low and C3 was 55.3 mg/dl (normal: 90–180 mg/dl), antinuclear factor was negative, partial thromboplastin, activated partial thromboplastin time was normal.

Renal biopsy revealed an increase in mesangial cellularity and matrix along with fibrous crescents, patent capillary loops and no spikes or duplication in the basement membrane. Biopsy from the skin lesion established the diagnosis of circumscribed juvenile type of PRP (type 4). His renal disease was treated with oral prednisolone and remission achieved after 2 weeks. The patient is on regular follow-up for last 2 years with no relapse of proteinuria until date. He is now under treatment of dermatologist for PRP.

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