P8.100 GENERALIZED HYPOTRICHOSIS WITH FEATURES OF LOOSE ANAGEN HAIR SYNDROME- REPORT OF A FAMILY WITH AUTOSOMAL DOMINANT INHERITANCE

Sung-Jan Lin, Shiuo-Hwa Jee, Hsien-Ching Chiu

Department of Dermatology, National Taiwan University Hospital and National

Taiwan University College of Medicine, Taipei, Taiwan

Objectives: To explore the clinical and pathological features of a tentative new genodermatosis featured by generalized hypotrichosis with easily pluckable scalp hair in a Taiwanese family

Methods: Individuals in the family were examined by the authors. Hair pull test, hair pluck test, routine blood biochemistry examination and complete blood counts were performed. The hair was examined by light microscopy and scanning electron microscopy. A questionnaire including hair defects and their chronological changes, nails, teeth, sweating, skin diseases and associated systemic diseases was used. Skin biopsy was performed for pathological examination.

Results: This autosomal dominant hair disorder spans four generations in a Taiwanese family. Involved individuals in this family have sparse scalp hair, eyebrows, and eyelashes since early childhood. Vellus hair can hardly be found on other body surfaces. Scalp hair is easily and painlessly extracted during early childhood and the extracted scalp hair is typical of loose anagen hair. Scalp hair increases in amount and length gradually during childhood or puberty and then can no longer be easily extracted. However, a high percentage of loose anagen hair was still revealed in a hair-pluck trichogram during adulthood. Hair inside nostrils, axillary hair, pubic hair, and facial hair remain sparse in adulthood and become sparser with age. Teeth, nails, and sweating are normal. A cleft upper lip is noted in one affected individual.

Conclusions: The association of loose anagen hair syndrome with absence of body vellus hair and sparse eyebrows, eyelashes, axillary hair, pubic hair and hair inside nostrils has never been reported before. We conclude this syndrome can be a new autosomal dominant genodermatosis or a severe form of familial loose anagen hair syndrome.