Epidemiology of folliculitis decalvans and keratosis follicularis spinulosa decalvans in British Columbia
Folliculitis decalvans (FD) is a rare inflammatory scalp disorder, classified as primary neutrophilic cicatricial alopecia that predominantly occurs in middle-aged adults. The hallmark of FD is the development of scarred areas and follicular pustules on the scalp. Keratosis follicularis spinulosa decalvans (KFSD) or Siemens-1 syndrome is a rare X-linked disease of unknown etiology affecting the skin and the eye. KSFD is characterized by diffuse keratosis pilaris with a scarring alopecia of the scalp and associated photophobia, facial erythema, and palmoplantar keratoderma. Although initially described as a sex-linked disorder, several different inheritance patterns have been observed. The cicatrical alopecia in KSFD shows clinical and histological similarities to FD. The incidence of these two forms of cicatricial alopecia is unknown. The goal of this research project is to determine the prevalence and incidence of folliculitis decalvans and keratosis follicularis spinulosa decalvans in British Columbia. A province wide survey amongst dermatologists and pathologists will be arranged. The physicians will be asked to report all FD and KSFD cases to the Department of Dermatology and Skin Science, UBC. The study will help to get a better understanding of the epidemiology of these rare disfiguring scalp diseases.