SARCOIDOSIS PRESENTING AS CUTANEOUS HYPOPIGMENTATION WITH REPEATEDLY NEGATIVE SKIN BIOPSIES

A 38-year-old Venezuelan woman presented with depigmentation of the lower right side of the face and contiguous right side of the neck. A clinical diagnosis of vitiligo was made, but a skin biopsy showed only changes of postinflammatory pigmentary alteration (Fig. 1). A second biopsy approximately 2 months later showed similar changes. Soon after this, the patient developed subcutaneous nodules on her feet and legs. A biopsy showed a septal panniculitis consistent with erythema nodosum (Figs. 2 and 3), and a third skin biopsy from a hypopigmented area, done at the same time, yet again showed postinflammatory pigmentary changes and no sarcoidal granulomas. Soon thereafter, she developed a variety of systemic symptoms including arthralgia, myalgia, dysphagia, fever, night sweats, proximal muscle weakness, epigastric discomfort, progressive shortness of breath on exertion, and blurred vision. An optic examination showed keratitis sicca. A chest x-ray showed bilateral hilar lymphadenopathy. A CT scan showed extensive mediastinal lymphadenopathy without evidence of parenchymal involvement, and a gallium scan showed increased uptake in hilar and right paratracheal areas. A mediastinal lymph node biopsy was performed and showed noncaseating granulomas consistent with sarcoidosis (acid-fast and silver stains were negative for mycobacteria and fungi, respectively) (Fig. 4). Biopsy material from both mediastinum and lung was cultured for acid-fast bacilli but failed to grow any. Angiotensin-converting-enzyme serum level was elevated at 89 U/L (reference range: < 55 U/L). The patient was treated with prednisone. Rifampin (for approximately 3 months) and isoniazid were also given because of a positive tuberculin skin test. Her erythema nodosum has resolved and other systemic systems have improved.