Indian Pediatrics - Images in Clinical Practice

Images in Clinical Practice

Indian Pediatrics 2001; 38: 796

Hereditary Wooly Hair

A one-year-old boy presented with excessive fragility and loss of scalp hair since birth. He was born to non-consanguineous parents after an uneventful pregnancy. A history of similar complaints was obtained from the father. Scalp examination revealed tightly coiled, fine, soft, dry, lusterless and poorly pigmented sparse hair, with irregular and scattered patches of alopecia (Fig. 1). Nails, teeth, genitalia, and sweating were normal. Light microscopy revealed short, thinned, coiled hair, with decreased hair shaft diameter, without nodes or twists. A KOH examination did not reveal fungal elements. Father had similar changes of the scalp hair with normal body hair. Parents were counseled regarding the benign nature of the disorder.

Wooly hair is a hair shaft disorder characterized by tightly coiled scalp hair in persons of non-negroid origin. Four types have been described: hereditary wooly hair, familial wooly hair, symmetrical circum-scribed allotrichia, and wooly hair nevus. Hereditary wooly hair, an autosomal domi-nant form, is characterized by excessively curly and tightly coiled negroid hair affecting only the scalp. Diagnosis is suspected on clinical grounds and confirmed on light and/or scanning electron microscopy. Being a benign disorder that may ameliorate in adult life, only reassurance is required.

Fig. 1. Poorly pigmented coiled, sparse, wooly hair on the scalp.

Gurvinder P. Thami,
Sukhjot Kaur,

Department of Dermatology and Venereology,
Government Medical College and Hospital,
Sector-32, Chandigarh,India.
E-mail: [email protected]