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Impressive skin manifestation of systemic sarcoidosis

Identifieur interne : 002253 ( Main/Exploration ); précédent : 002252; suivant : 002254

Impressive skin manifestation of systemic sarcoidosis

Auteurs : A. M. H. A. Janitha P. Chandani [Allemagne] ; Yael D. Adler [Allemagne] ; Edgar Dippel [Allemagne] ; Christos C. Zouboulis [Allemagne]

Source :

RBID : Pascal:02-0358927

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English descriptors

Abstract

A 30-year-old man presented with livid-red to brownish, strongly infiltrated, and markedly elevated plaques of 1-5 cm in diameter with fine scaling on the buttocks, lower trunk, and lower extremities of 2 years' duration (Figs 1 and 2). Complete blood cell count and routine serum chemistry were within normal limits, with the exception of erythrocyte sedimentation rates of 18 and 36 mm in the first and second hour, respectively, and a C-reactive protein level of 8.1 mg/dL. Serum angiotensin converting enzyme (ACE) levels were elevated at 90 U/L (normal values, 18-60 U/L), while serum lysozyme levels, vitamin D, and calcium were normal. The patient presented an anergic reaction to the seven antigens of Multitest Merieux. Histopathology of the skin lesions revealed typical, but very large, noncaseating, naked, epithelioid cell granulomas throughout the dermis (Fig. 3). Chest X-ray revealed bilateral hilar and mediastinal lymph node enlargement with reticular-nodular shadowing in both lung fields (Fig. 4). Lung function test diagnosed a middle-grade restrictive ventilatory defect. Myocardial scintigraphy with thallium-201 showed evidence of defective uptake in the anterior, posterior, and apical regions of the left ventricle which was compatible with cardiac involvement (Fig. 5). Abdominal ultrasound showed mild hepatosplenomegaly (liver, 16 cm in medioclavicular line; spleen, 12.3 cm longitudinal diameter). X-Ray of the hands detected a 1 -cm cyst in the proximal phalanx of the left index finger (Fig. 6). Eye and neurologic examination were normal. Despite the clinical picture of the skin lesions, which was compatible with cutaneous B-cell lymphoma, our findings were compatible with stage II intrathoracic and systemic sarcoidosis with atypical cutaneous plaque-type lesions, lung, mesothoracic lymph node, heart, spleen, liver, and bone involvement. Thus, the patient was treated initially with prednisolone, 50 mg orally, tapered to 10 mg over a period of 8 weeks, and chloroquine, 250 mg orally daily. Three weeks after steroid therapy, there was marked clinical improvement, as well as improvement of inflammatory markers and normalization of serum ACE levels (19.4 U/L). The patient was followed up for 6 months and showed flattening of the skin lesions to macules with color brightening, as well as stabilization of the systemic disease (Fig. 7).


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<div type="abstract" xml:lang="en">A 30-year-old man presented with livid-red to brownish, strongly infiltrated, and markedly elevated plaques of 1-5 cm in diameter with fine scaling on the buttocks, lower trunk, and lower extremities of 2 years' duration (Figs 1 and 2). Complete blood cell count and routine serum chemistry were within normal limits, with the exception of erythrocyte sedimentation rates of 18 and 36 mm in the first and second hour, respectively, and a C-reactive protein level of 8.1 mg/dL. Serum angiotensin converting enzyme (ACE) levels were elevated at 90 U/L (normal values, 18-60 U/L), while serum lysozyme levels, vitamin D, and calcium were normal. The patient presented an anergic reaction to the seven antigens of Multitest Merieux. Histopathology of the skin lesions revealed typical, but very large, noncaseating, naked, epithelioid cell granulomas throughout the dermis (Fig. 3). Chest X-ray revealed bilateral hilar and mediastinal lymph node enlargement with reticular-nodular shadowing in both lung fields (Fig. 4). Lung function test diagnosed a middle-grade restrictive ventilatory defect. Myocardial scintigraphy with thallium-201 showed evidence of defective uptake in the anterior, posterior, and apical regions of the left ventricle which was compatible with cardiac involvement (Fig. 5). Abdominal ultrasound showed mild hepatosplenomegaly (liver, 16 cm in medioclavicular line; spleen, 12.3 cm longitudinal diameter). X-Ray of the hands detected a 1 -cm cyst in the proximal phalanx of the left index finger (Fig. 6). Eye and neurologic examination were normal. Despite the clinical picture of the skin lesions, which was compatible with cutaneous B-cell lymphoma, our findings were compatible with stage II intrathoracic and systemic sarcoidosis with atypical cutaneous plaque-type lesions, lung, mesothoracic lymph node, heart, spleen, liver, and bone involvement. Thus, the patient was treated initially with prednisolone, 50 mg orally, tapered to 10 mg over a period of 8 weeks, and chloroquine, 250 mg orally daily. Three weeks after steroid therapy, there was marked clinical improvement, as well as improvement of inflammatory markers and normalization of serum ACE levels (19.4 U/L). The patient was followed up for 6 months and showed flattening of the skin lesions to macules with color brightening, as well as stabilization of the systemic disease (Fig. 7).</div>
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