Antonyan AS, Kerr H. Darier-Roussy disease.. J Am Acad Dermatol 2018; 79(3):AB98.
J Am Acad Dermatol
A 59-year-old African-American woman presented with a 3-week history of an asymptomatic plaque on her left upper eyelid and painless subcutaneous nodules on her forearms. She denied any joint pain, ocular, respiratory, neurologic, or other systemic symptoms. Examination revealed a red-brown indurated plaque on the medial margin of left upper eyelid extending to the medial canthus, as well as nontender cord-like subcutaneous nodules on the extensor surfaces of bilateral forearms, one of which had overlying hyperpigmentation. Chest x-ray revealed interval development of bilateral hilar prominence. ESR, CRP, and ACE levels were elevated. CT chest revealed multilevel mediastinal and hilar adenopathy, subpleural interstitial opacities, pleural and intraparenchymal nodules and pleural thickening, as well as thyromegaly with nodules. A punch biopsy of a left forearm nodule revealed noncaseating epithelioid granulomas in the deep dermis and subcutis, with minimal surrounding lymphocytic inflammation. Several eosinophilic stellate asteroid body inclusions and Langhans-type giant cells were noted. Foreign bodies were not detected on polarization microscopy. Grocott’s methenamine silver and acid-fast bacilli staining was negative. The diagnosis of Darier-Roussy subcutaneous sarcoidosis was made. A four-month trial of hydroxychloroquine 200 mg bid was ineffective, with progression of her skin lesions. She was then started on low dose prednisone therapy (20 mg daily, tapered off over 3 months) along with methotrexate tapered up to 15 mg weekly, with complete resolution of her skin lesions after 3 months of treatment. Methotrexate was discontinued after 5 months of therapy, but restarted 6 months later due to recurrence of her subcutaneous nodules. Darier-Roussy disease is the subcutaneous variant of cutaneous sarcoidosis, and is strongly associated with systemic disease. It typically appears at the outset of systemic disease, making it useful in the diagnosis of systemic sarcoidosis, and is generally thought to portend a favorable prognosis. The most common systemic finding is nonchronic, nonsevere pulmonary sarcoidosis with bilateral hilar adenopathy. Treatment for this subcutaneous variant of sarcoidosis is based on the severity of systemic disease and degree of disfigurement, with the mainstay being systemic corticosteroids. Other treatment options include antimalarials, methotrexate, NSAIDS, TNF-alpha inhibitors, and intralesional corticosteroids.