Overlap Between Ulerythema Ophryogenes and Keratosis Follicularis Spinulosa Decalvans: a Case Report

• Main Authors: Stojanović Slobodan, Vučković Nada, Jovanović Marina
• Format: Article
• Language: English
• Published: Sciendo 2015-09-01
• Series: Serbian Journal of Dermatology and Venereology
• Subjects: eyebrows; facial dermatoses; keratosis; hair diseases; erythema; skin diseases, genetic; chromosomes, human, x; darier disease; obrve; dermatoze lica; keratoza; bolesti dlake; eritem; genetske kožne bolesti; humani hromozom x; darierova bolest
• Online Access: https://doi.org/10.1515/sjdv-2015-0012
• ISSN: 1821-0902; 2406-0631

Ulerythema ophryogenes and keratosis follicularis spinulosa decalvans are rare folliculocentric keratotic disorders, from the group of follicular genokeratoses, characterized by keratosis pilaris atrophicans: follicular keratotic papules, sometimes with surrounding erythema, which eventually result in fibrosis, atrophy, progressive scarring and permanent hair loss. Ulerythema ophryogenes begins at birth or soon thereafter; it involves the lateral eyebrows, spreads medially and eventually affects the entire eyebrows, cheeks, and less frequently, forehead and asjecebt scalp. Involvement of the scalp has apparently not been reported in cases in which the eyebrows were predominantly involved. In addition to sporadic cases, ulerythema ophryogenes has been reported among relatives. Keratosis follicularis spinulosa decalvans is also a genetically heterogeneous syndrome which begins in infancy or childhood by involving hair bearing skin, especially the scalp; rarely it is confined to the face involving only eyebrows and eyelashes, but affects predominantly the scalp, leading to severe progressive cicatricial alopecia. Both conditions tend to progress until puberty.