Two cases of lymphomatoid granulomatosis are described. The clinical features included pruritus, acquired ichthyosis, widespread skin nodules and plaques, skin ulceration, patchy alopecia and loss of sweating. Skin biopsies showed lymphomatous and granulomatous changes associated with a vasculitis. In one patient chest radiographs revealed several shadows in the lower zones of the lung fields. His disease improved on treatment with systemic steroids both alone and in combination with cyclophosphamide. The second patient's condition later evolved into Hodgkin's disease, which responded to treatment with cytotoxic drugs. Both patients eventually died of pneumonia possibly related to their therapy.