Clinicopathologic and Trichoscopic Features of Keratosis Follicularis Spinulosa Decalvans: A Case Series Study
December 2023
in “
Journal of dermatology
”
keratosis follicularis spinulosa decalvans KFSD follicular hyperkeratosis alopecia scarring alopecia vellus hair hair follicle disorders inflammation fused infundibulum dilated follicles perifollicular scaling tufted hairs loss of follicular openings terminal hair involvement follicular destruction hair loss scarring hair loss hair follicle issues fused hair follicle opening dilated hair follicles scaling around hair follicles tufted hair loss of hair follicle openings terminal hair hair follicle destruction
Studysummary This study examined the clinicopathologic and trichoscopic characteristics of keratosis follicularis spinulosa decalvans and identified terminal hair involvement and follicular hyperkeratosis as key diagnostic features.
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This case series study investigates the clinicopathologic and trichoscopic features of keratosis follicularis spinulosa decalvans (KFSD), a rare X-linked hereditary disorder characterized by follicular hyperkeratosis, photophobia, and alopecia. The study included 8 patients from 7 families, with an average age of onset of alopecia at 21.25 years. The hallmark feature was progressive scarring alopecia on the midline of the scalp, beginning after puberty, with variable inflammation. All patients exhibited vellus hair–associated follicular hyperkeratosis, though photophobia was not consistently present. Histopathologic examination showed hair follicle disorders with acute-chronic inflammation, including fused infundibulum and dilated follicles at the isthmus level. Trichoscopic features included perifollicular scaling, tufted hairs, and loss of follicular openings. The study concludes that terminal hair involvement and follicular hyperkeratosis are essential for diagnosing KFSD, with follicular changes triggering inflammation and follicular destruction.
