Hilar - Lymphadenopathy predictive of cutaneous sarcoid

Introduction

Sarcoidosis, a multisystemic disease, of undetermined etiology, is found world -wide. It affects both the males and females of younger age-groups.[1],[2],[3],[4] It is frequently diagnosed in developed countries and its occurrence is inversely proportional to systemic tuberculosis and/or leprosy.[5] Cutaneous manifestations of sarcoid are cardinal and may be of considerable significance since their presence may suggest previously undiagnosed systemic disease,[6],[7] through the presence of primarily epithelioid,noncaseating naked granuloma.[8] The sequence of events in the patients are being recounted currently to illustrate and emphasize the importance of bilateral hilar lymphadenopthy as a diagnostic hallmark of impending cutaneous sarcoid.

Case Report

A 34-year-old man presented with progressively increasing asymptomatic swellings of varying sizes over the face for the past 4 months. He was apparently well about a year ago, when he had an episode of pain and swelling of the legs, followed 15 days after a similar episode affecting the hands. He had a consultation from an internist who thoroughly investigated, and diagnosed the condition as tuberculous bilateral hilar lymphadenophthy. Accordingly, he was administered anti- tuberculosis treatment (ATT), comprising rifampicin 450 mg, isonicotinic hydrazide 300mg, ethambutol 800mg, and pyrazinamide 1,500mg daily. These drugs were accepted well for 11 days, but on 12th day he had an extensive erythematosquamous eruption (erythema multiforme). Pyrazinamide was withdrawn and the rash subsided in the course of a week. Rest of the drugs were continued. In the course of the treatment (5 months after), he noticed progressively increasing asymptomatic pinhead to pea-sized eruption over the forehead, eyelids, top of the shoulder, and front and back of the upper chest. Consitutional symptoms were conspicuously absent. Despite the appearance of cutaneous lesions the treatment was continued for 9months′ period.

Skin surface examination revealed flesh colored papules and/or nodules of the sizes varying from 0.3 to 2 cm. The nodules were fixed to the underlying tissue. The overlying skin was movable and ulceration was conspicuously absent. One of the lesions had telangiectasia. The lesions were multiple and distri- buted over the forehead [Figure - 1], medial 1/3 of the right upper eyelids, shoulder and front and back of the upper chest. They were bilateral and asymmetrical. Blood examination comprising TLC, DLC, Hb, ESR was within normal limits so also was urinanalysis. Ziel-Neelson stained sputum for AFB was repeatedly negative. Hilar lymph- adenopathy was prominent in both anterioposterior [Figure - 2] and lateral skiagram of chest. Injection of 0.1 ml Spans tuberculin 5Tu(Mantoux) was negative. Colored doppler study of lower limb venous system too was normal. Thorax CT- scan indicated bilateral hilar and mediastinal lymphadenopathy. SCOT, SGPT and serum alkaline phosphatase were normal. Hematoxylineosin stained sections prepared from a representative nodule was marked by the presence of several lobules extending from upper dermis into subcutaneous fat. The lobules were surrounded by condensed collagen. Each lobule was characterized by the presence of well circumscribed epithelioid granulomas. Occasional, giant cells were identified, a few of them had inclusion bodies. Only sparse infiltration of lymphocytes were located at the periphery of the granulomas, the naked granulomas [Figure - 3]. At this juncture, after forming the diagnosis of cutaneous / extra-cutaneous sarcoid, prednisolone in the dosage of 40mg a day in two divided doses was prescribed and is being continued with excellent results. The earlier skin lesions have regressed and there is no fresh crop of lesions.

Discussion

Cutaneous and/or extra-cutaneous manifestations of sarcoidosis are seldom diagnosed, in developing countries where its presence is inversely proportional to systemic tuberculosis and/ or leprosy.[9],[10],[11] The current case continued to occupy the physician′s attentions, and erroneously administered ATT There was no favourable response during the course of the treatment. It is indeed intriguing that an exclusive bilateral hilar lymphadenopathy failed to form a focus of a dialogue to short out the diagnosis that too in the absence of relative and absolute criteria apart from supportive immunologic evidence for diagnosis of tuberculosis.[10] The clinical features in the form of flesh colored papuls and or nodules were quite characteristic, and were in line with those described in the ritual text.[1],[2],[3],[4] Nonetheless, the conditions such as trichoepitheliomas, adenoma sebaceum, lymphoma, leukemia cults and cutaneous lymphoid hyperplasia were considered in its differential diagnosis. One time excision biopsy of the representative lesions was considered imperative to define the cardinal histologic characteristic as are documented in the case under review.