Scleredema

Since 1900 when scleredema was described by Buschke a total of 107 cases have been reported with but few exceptions in the foreign literature. The disease is characterized by the appearance of brawny, nonpitting, painless edema usually starting on the neck and rapidly spreading by continuity to the head, chest and back. Although the abdomen and extremities may be involved, the hands and feet are spared. The disease is preceded by a febrile illness, usually a streptococcal infection, and commonly undergoes spontaneous involution without sequelae within three to eighteen months. Systemic involvement, including pleural, pericardial and joint effusions, has recently been stressed. Twenty-seven per cent of the cases occurred within the first decade and females were involved more frequently than males. The present case showed the unusual findings of parotid involvement, possible pericardial involvement and, microscopically, early neutrophilic perivascular infiltration. Encouraging results with epinephrine therapy were noted, and the possibility of more dramatic therapeutic response with the 11,17-oxysteroids proven to be of value in other diffuse collagen diseases has been discussed.