Pulmonary lymphangioleiomyomatosis in a male

Pulmonary lymphangioleiomyomatosis has been observed almost exclusively in women, usually in their reproductive years. Exacerbations with pregnancy and after hormonal manipulation have been documented, and it has been suggested that its pathogenesis is due to the influence of hormonal(estrogenic) stimulus. The clinical, roentgenographic, and histopathologic features of this case of pulmonary lymphangioleiomyomatosis in a 22-year-old male are all characteristic of those described in prior reports, except for the patient's sex. With the following case of pulmonary lymphangioleiomyomatosis in a male, we suggest the possibility of the existence of an additional pathogenetic mechanism.