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September 1960


Arch Dermatol. 1960;82(3):443-451. doi:10.1001/archderm.1960.01580030137032

Incontinentia Pigmenti. Presented by Dr. Charles S. Keuper.  A girl aged 4 is presented from the Children's Hospital with a "birth mark" on the left leg and thigh which has been present since early infancy. It has not changed in character.A Grade III systolic murmur was heard at the age of 3 months, and a ventricular septal defect has been diagnosed in the Cardiac Clinic.There is reticulated, yellow-brown pigmentation on the left leg and ankle, extending to the midthigh posteriorly, and most prominent on the anterior aspect of the knee and leg.

Biopsy.—  The basal layer was hyperpigmented, but no pigment-laden macrophages were seen in the corium. The corium showed a rather striking number of mast cells.

Discussion  Dr. Aaron B. Lerner: This little girl has pigmented areas on her arms and legs. The rest of her skin is rather light in color. The first thing that comes

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