Lethal systemic degos disease with prominent cardio-pulmonary involvement.

A 48-year-old man presented with acute abdominal pain underwent laparotomy that revealed two perforated ulcers in jejunum. He had skin lesions with porcelain white atrophic scar which were ignored for 3 years, whereas the disease revealed own malignant nature and progressed to nervous, gastrointestinal, and cardiopulmonary systems. The diagnosis of Degos disease was established on the basis of clinical and histopathological features. He expired due to cardio-pulmonary insufficiency after 5 months from the onset of systemic involvement. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs. Distribution of skin lesions that involved palmoplantar surfaces, genitalia and scalp and, furthermore, course of disease as rapid progressive cardio-polmunary involvement are remarkable point in this patient. On the other hand, this case highlights importance of clinicopathologic correlation, specially in the dermatologic field.

Bullous mycosis fungoides: a case report.

Mycosis fungoides (MF) on extremely rare occasions is associated with vesiculobullous eruptions. We describe a 74-year-old man with previous documented histopathologic diagnosis of poikilodermic-type MF who recently developed flaccid acral bullae on erythematous MF plaques and on normal appearing skin. Histopathology and direct immunofluorescent studies revealed extensive lichenoid changes with intraepidermal bullae. Atypical lymphocyte infiltration was present at the dermoepidermal junction, in bulla fluid, and on the peripheral blood smear, but in lymph node and bone marrow tissue specimens atypical lymphocytes were absent. The plausible cause of these blister formations in cutaneous lymphoma bullosa may be from excessive epidermotropism or toxicity of the tumoral infiltrate.