Hilar - Lymphadenopathy predictive of cutaneous sarcoid
2 Skin Care Centre, 108 Darya Gani, New Delhi, India
3 Department of Pathology, Lady Hardinge Medical college, New Delhi, India
Virenda N Sehgal
Dermato - Venereology (Skin/VD) Centre, Sehgal Nursing Home, A/6 Panchwati, Delhi -110 033
|How to cite this article:
Sehgal VN, Sehgal N, Jain S, Balal P. Hilar - Lymphadenopathy predictive of cutaneous sarcoid. Indian J Dermatol Venereol Leprol 2002;68:161-163
AbstractA case of hilar lymphadenopathy, a cardinal sign of extra- cutaneous sarcoidosis, is being described in detail. It continued to be a diagnostic dilemma until the appearance of several papules and/or nodules overthe face, front and back of the upper chest. The diagnosis was clinched by demonstration of non-caseating, epithelioid naked granuloma and affirmative response to oral administration of prednisolone in dosage of 40 mg a day.
Sarcoidosis, a multisystemic disease, of undetermined etiology, is found world -wide. It affects both the males and females of younger age-groups.,,, It is frequently diagnosed in developed countries and its occurrence is inversely proportional to systemic tuberculosis and/or leprosy. Cutaneous manifestations of sarcoid are cardinal and may be of considerable significance since their presence may suggest previously undiagnosed systemic disease,, through the presence of primarily epithelioid,noncaseating naked granuloma. 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.
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 Pyrazinamide was withdrawn and the rash subsided in the course of a week.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.
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.,, 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. 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.,,, 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.
Sharma OP. Cutaneous sarcoidosis: clinical features and management. Chest 1972;61:320-325.[Google Scholar]
Kerdel FA, Moschella SL. Sarcoidosis: An updated review. J Am Acad Dermatol 1984;11:1-9.[Google Scholar]
Elgart ML. Cutaneous sarcoidosis: Definition and types of lesions. Clin Dermatol 1986;4:35-45.[Google Scholar]
Elgart ML. Cutaneous lesions of sarcoidosis. Primar Care 1978;5:249-262.[Google Scholar]
Sartwell PE. Racial differences in sarcoidosis. Ann NY Acad Sci 1976;278:368-370.[Google Scholar]
Callen JP Relationship of cutaneous sarcoidosis to systemic disease. Clin Dermatol 1985;4:46-53.[Google Scholar]
Mayock RL, Bertrand P Morrison CE, et al. Manifestations of sarcoidosis: Analysis of 145 patients, with a review of nine series selected from the literature. Am J Med 1963;35:67-89.[Google Scholar]
Lever WF, Schaumburg-Lever G. sarcoidosis. In: Histopathology of the Skin 7th ed. Philadelphia: JB Lippincot 1990:252-256.[Google Scholar]
Oliver KE, Kataria YP Cutaneous manifestations of sarcoidosis. Arch Int Med 1985;145:1811-1814.[Google Scholar]
Sehgal VN. Cutaneous tuberculosis. Dermatology Clinics 1994;12:645-653.[Google Scholar]
Bhattacharaya SN, Sehgal VN. Leprosy in India. Clinics in Dermatology 1999;17:159-170.[Google Scholar]