Abstract:

A case of malignant angioendothelioma complicated with herpetiform pemphigus is first reported update. A 71-year-old male presented with multiple dark red infiltrated plaques, nodules and ecchymoses on his forehead with bleeding and painful sensation for 9 months and erythema and vesicles with itching all over the body for 20 days. Biopy of eruption of forehead showed the tumor was located in the dermis. The tumor was composed of blood vessels of different sizes. The hyperplastic endothelial cells of the blood vessels and extravasated red blood cells could be found in the blood vessels. Atypical endothelial cells with hyperchromatic nuclei could be observed and mitoses were present occasionally. Immunohistochemical staining showed that the antibodies to CD31, CD34 were positive. The diagnosis was malignant angioendothelioma. Biopsy of vesicle showed its location within the epidermis, with the infiltration of eosinophilic granulocyte and neutrophils. Direct immunofluorescence demonstrated strong intercellular deposits of IgG and C3 in the mid to lower epidermis. The diagnosis of herpetiform pemphigus was made. Prednisone 30 mg daily was given and vesicles subsideded after one-week’s treatment. The patient was than transferred to orthopedics to receive surgical excision of tumor and perform designed skin flap transferring. No reoccurrence was found 3 months after the above treatment.

Key words: malignant angioendothelioma, herpetiform pemphigus