Sarcoidosis with subcutaneous nodules

Abstract

S arcoidosis is a multisystem granulomatous disease of unknown etiology most commonly affecting young adults. It frequently presents with intrathoracic, dermatologic, or ophthalmologic involvement. The course and prognosis correlate with the mode of onset [1]. Acute sarcoidosis with fever and hilar lymphadenopathy often resolves spontaneously. The insidious onset of chronic sarcoidosis is usually followed by relentless progression of disease. The diagnosis is based on the appropriate clinico-radiographic findings supported by histologic evidence of non-caseating granulomas in the affected organ system after other infectious or granulomatous diseases are excluded. Since the skin and soft tissue are readily accessible for physical examination and histologic evaluation, it is important to recognize the cutaneous manifestations of sarcoidosis. These are divlded into specific and non-specific on the basis of whether there is histopathologic evidence of sarcoidal granulomas in the skin lesions. The most common non-specific skin eruption is erythema nodosum, which occurs in 17 percent of patients [2]. Table I lists the reported types of specific cutaneous sarcoidosis. In 1904, Darier and Roussy [3] reported on a patient with sarcoidosis in whom the sole cutaneous manifestation was subcutaneous nodules. Since then, patients with clinical and pathologic evidence of sarcoidosis in the subcutaneous tissue have been diagnosed with the eponym Darier-Roussy sarcoidosis [4--12]. However, this term has also been used for other forms of granulomatous panniculitis. Subsequent reports of DarierRoussy sarcoidosis have included a mixed group of diseases including erythema nodosum, erythema induratum, foreign body granulomas, syphilis, and tuberculosis [5]. The purpose of our report is to describe six new cases of sarcoidosis presenting with subcutaneous nodules. We propose a definition of subcutaneous sarcoidosis that includes subcutaneous nodules with normal overlying epidermis and histologic evidence of non-caseating granulomas in the lesions. We support Vainsencher and Winkelmann's [5] concept that the eponym Darier-Roussy sarcoid has caused confusion and that the term subcutaneous sarcoidosis is a more appropriate one. The clinical course of our patients with this rare type of sarcoid was followed and the prognosis is discussed. It is important to recognize