[Lymphomatoid granulomatosis with "epithelial islands" (author's transl)]

The lymphomatoid granulomatosis was first definded by Liebow, Carrington and Friedman as a vascular angiodestructive lymphoreticular proliferation process. The clinical symptoms and signs are pruritus, acquired ichthyosis and the following cutaneous alterations: widespread skin nodules and plaques, patchy alopecia and anhidrosis. Our patient revealed the cutaneous pattern mentioned above and a pathological pneumonography, showing diffuse infiltrates in the lower lobes of the lung. Epithelial islands are a characteristic feature of the lymphadenoid infiltrate in our patient. To our knowledge, only the two patients with lymphomatoid granulomatosis described by MacDonald and Sarkany (10, especially case 1) revealed this special pattern.