[Lymphomatoid granulomatosis: cyclic nodular lymphomatoid panniculitis with immunologic deficiency]

The case of a 66 years old woman having presented an unusual cyclic dermatosis of 11 months duration is reported. The cutaneous elements were successively located in a subcutaneous, dermal and epidermal situation, and presented a spontaneous healing with sometimes deep retractive residual scars after an ulceration or not. Pathologically, the lesions consisted in dense mononuclear infiltrates and vascular lesions. This disease was associated with an immuno-deficiency state characterized by extremely low levels of circulating IgM. This deficit was found to be persistant as it was still present two years later. However during this lapse of observation no cutaneous lesions recurred. The possible connexions of such a case with those of lymphomatous granulomatosis are discussed. In the absence of pulmonary lesion in the case reported here, no identification to the syndrome isolated by Liebow seems permitted. The authors offer a new denomination for this unusual entity.