Cutaneous manifestations of lymphomatoid granulomatosis. Report of 44 cases and a review of the literature.

A review of records of 44 patients who had lymphomatoid granulomatosis (LYG) with skin lesions showed that cutaneous manifestations are protean and generally transient. Subcutaneous or dermal nodules were present in more than 60% of cases. A maculopapular rash or macular erythema was less common, and ulceration was rare. Skin lesions were widely distributed. Frequently, in patients who had early cutaneous involvement with LYG, associated involvement of the nervous system and joints occurred. Findings from laboratory studies were generally inconclusive. Lymphomatoid granulomatosis progressed to lymphoma in 18% of the cases, and death from respiratory failure was common. Theories on the possible cause and the pathogenesis of LYG are presented.