Systemic chlamydial infection associated with generalized lymphedema and lymphangiosarcoma

A Chlamydial agent was isolated repeatedly from a variety of body fluids in a Negro adolescent boy with a fatal progressive lymphedema and widespread lymphangiosarcoma. The hybrid Chlamydial organism cultured resembled closely Chlamydia trachomatis. Autopsy revealed thickened 'elephantine' skin of the lower trunk, scrotum and penis. Microscopic examination disclosed widespread lymphatic dilatation and disseminated lymphangiosarcoma, particularly prominent in the lungs, peribronchial lymph nodes, scrotal tissue, thymus gland, sternothyroid muscle, diaphragm, bone marrow, thoracic duct, pleura, and rectum. Angiosarcomatous change of the skin was extensive and a skin nodule obtained from the left thigh showed a fibrosarcoma like proliferation with invasion of dermal papillae and extensive acanthosis of the underlying epidermis with early bulla formation (designated as 'Kaposi's sarcoma').