Ichthyosiform sarcoidosis

Asok Kumar Gangopadhyay

 Department of Dermatology , R.K.M. Seva Pratishthan, Kolkata -700026, India

Correspondence Address:
Asok Kumar Gangopadhyay
7,B.M Road, Calcutta--700026
India

Introduction

Sarcoidosis is an idiopathic multisystem granu-lomatous disease that commonly involves the lungs, eyes, lymph nodes and the skin. Cutaneous manifestiations of sarcoidosis occur in 20% to 30% of patients with systemic involvement.[1] Sarcoidosis limited to the skin occurs in approximately 25% of patients.[2] Acquired ichthyosis is a rare cutaneous manifestation of sarcoidosis. Only 20 cases of IS are reported in the world literature so far.[3] Here we report such a rare case.

Case Report

A Hindu housewife of 25, presented with dry scaly lesions. on upper extremities, chest, ab-domen and face for the last 3 years. The disease started insidiously when she first noticed dry scaly lesions appearing over the arms, chest wall, back and abdomen very slowly in a course of 2 years. The face was involved later. There were a few scattered lesions on her thighs, otherwise the lower extremities were spared. There was no sig-nificant problem as such except mild discomfort due to the dryness-particularly during the winter months. The lesions did not clear up in summer. She was keeping good health and there was no systemic complaint during all these years. There was no history of any drug intake, of family history of ichthyosiform disorder. Cutaneous examination revealed silver-coloured, rhomboidal scaly plaques over the upper extremities, chest, back and abdo-men and few scattered on both the thighs. [Figure - 1] The anterior aspect of the elbows were also involved. There was no palmo-plantar keratoderma. There was no change in nails, hair or oral mucosa. There was no anaesthesia any where, nor any thickening of peripheral nerves. Systemic examination revealed no abnormality. There was no lymphadenopathy, nor any abnormality detected in the eyes. Routine examination of blood was normal. Serum calcium was 6mEq/L. Serum angiotensin converting enzyme (SACE) was 64 u/1 (normal 5-52 u/1 at 37°C). Mantoux test was negative. Antinuclear factor was negative. Chest x-ray and ultrasound study of abdomen showed no abnormality. Biopsy from the lesion on forearm showed compact orthokeratosis with diminished granular layer. Multiple noncaseating epithelioid cell granulomas were found deep in the dermis. Periodic acid - Schiff and Fite Faraco′s stains were negative for fungus and mycobacteria respec-tively.

Discussion

Acquired ichthyosis from sarcoidosis was first described by Braverman in 1981.[3] Subsequently the term IS was coined and the concurrent presence of sarcoidal granuloma and ichthyosis in histology was considered to be the diagnostic hallmark of the condition. All the 20 patients described earlier were non-white. There was no special predillection for any gender. Systemic involvement of sarcoidosis was initially present or subsequently developed in 19 of the 20 patients with IS.[3] The case reported herein has got epithelioid cell granuloma, negative Mantoux test, high serum calcium and SACE level but no systemic involvement. She needs a long term follow up to look for development of systemic features of sarcoidosis in later life!

1.	Samtson AV. Cataneous sarcoidosis. Int J Dermatol 1992 ; 31 385-391. [Google Scholar]
2.	Hannor, Needelman A, Eiferman RA, et al, Cutaneous sarcoidal granulomas and the development of systemic sarcoidosis. Arch Dermatol 1981;117:203-207. [Google Scholar]
3.	Cather JC, Cohen PR. Ichthyosiform sarcoidosis. J Am Acad Dermatol 1999;40:862-865. [Google Scholar]