March 1959

Incontinentia Pigmenti (Bloch-Sulzberger Syndrome)A Generalized Disease

Author Affiliations

Department of Pediatrics, Michael Reese Hospital (Dr. Mendelsohn); Department of Dermatology, University of Illinois, formerly of Michael Reese Hospital (Dr. Medansky), and Department of Dermatology, Michael Reese Hospital, and Northwestern University (Dr. Lazar).

AMA Am J Dis Child. 1959;97(3):348-352. doi:10.1001/archpedi.1959.02070010350015

Incontinentia pigmenti is a rare disease. Because it is regarded as a dermatologic problem, few cases have been reported in the pediatric literature. This case is presented to illustrate the multisystem involvement which may be seen in the infant.

Report of Case  A 9-month-old white girl was admitted to Michael Reese Hospital on Feb. 28, 1957. The family history was negative, the mother had not been ill during pregnancy, and the delivery was normal. The history of illness dates from the second day of life, when a generalized vesicular eruption appeared and a diagnosis of epidermolysis bullosa was considered. She was treated with oral hydrocortisone (Cortef) and Mazon (a combination of phenolic substances and organic mercury compound) and Terra-Cortril (an ointment containing 3% oxytetracycline HCl and 1% hydrocortisone, free alcohol) ointments. She was also placed on hypoallergenic soya formula (Sobee) feedings. As the bullae disappeared, a generalized eruption was noted

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