KERATOSIS FOLLICULARIS SPINULOSA DECALVANS
By Gary M. White, MD
Keratosis follicularis spinulosa decalvans (KFSD) is an X-linked disorder characterized by widespread hyperkeratotic follicular papules (including keratosis pilaris-like lesions), facial erythema, hypotrichosis and progressive alopecia of the scalp, eyelashes, and eyebrows.
- KFSD results from mutations in the MBTPS2 gene, which codes for an intramembrane zinc metalloprotease essential for cholesterol homeostasis and endoplasmic reticulum stress response.
- In the past, various terms including ulerythema ophryogenes and atrophoderma vermiculatum have been used to describe the same or similar entities.
Extensive keratosis pilaris associated with scarring alopecia is characteristic. Other features include atopy, PPK, and photophobia.
There is no specific treatment for KFSD. Various drugs have been tried to delay scarring alopecia such as isotretinoin and dapsone. Emollients, topical steroid, and keratolytic agents may be tried. Acitretin benefited one patient.
Int J Trichology 2013;5:29
Homepage | FAQs | Contact Dr. White
It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.