Keratosis follicularis spinulosa decalvans. Report of two cases and literature review

Abstract

We report herein two cases of keratosis follicularis spinulosa decalvans (KFSD) and review the literature on this condition. The entity is one of a group of related disorders that shows keratosis pilaris with inflammation followed by atrophy. The clinical features and course of KFSD are characteristic. During infancy, keratosis pilaris begins on the face and, by childhood, progresses to involve the trunk and extremities. Sometime during childhood or up to the early teenage years, a cicatricial alopecia of the scalp and eyebrows develops and is the hallmark of this disorder. Hyperkeratosis of the palms and soles is a frequently associated finding and is usually manifested during adolescence. Other features occurring with this syndrome include atopy, photophobia, and corneal abnormalities. Sex-linked inheritance has been proposed by several authors.

Keratosis Follicularis Spinulosa Decalvans Associated with Acne Keloidalis Nuchae and Tufted Hair Folliculitis

“…Three syndromes included in this group are KFSD, keratosis pilaris atrophicans faciei, and atrophoderma vermiculatum. [1][2][3] KFSD was first described by Siemens in 1926. [4] It is an uncommon X-linked disorder characterized by widespread follicular hyperkeratosis with variable degrees of inflammation, cicatricial alopecia of the scalp, and photophobia.…”

“…As the nuchal skin responds to keratin. [1,2] There may be a superficial perifollicular and intrafolinflammation by forming keloidal scars, it is hypothesized that licular neutrophilic infiltrate when lesions are acutely inflamed.…”

“…[1,7] KFSD typically begins in infancy with the appearance of keratosis pilaris on the face, which progresses to a more widespread distribution on the trunk and limbs. [2] Follicular hyperkeratosis of the scalp with a variable degree of inflammation results in scarring alopecia of the scalp and eyebrows in early childhood or adolescence. The alopecia may be limited to only a few patches on the vertex, resembling folliculitis decalvans, or it may be more widespread.…”

Keratosis Follicularis Spinulosa Decalvans: A New Observation

“…These disorders have been grouped under the category of keratosis pilaris atrophicans, which comprises [1][2][3]. KFSD (involvement of scalp, eyebrows, and eyelashes in addition to other associated features such OPEN ACCESS http://scidoc.org/IJCDR.php as palmoplantar keratoderma, photophobia, widespread KP, and atopic); Keratosis pilaris atrophicans facial (KPAF) or ulerythema ophryogenes (involvement of eyebrows, forehead, and cheeks) ; Atrophoderma vermiculatum (AV) (involvement of the cheeks and periauricular regions, which is followed by reticulate atrophy) the variant of KFSD that starts at puberty is called folliculitis spinulosa decalvans (FSD).…”

Molecular genetic analysis of two families with keratosis follicularis spinulosa decalvans: refinement of gene localization and evidence for genetic heterogeneity

“…KFSD is both clinically and genetically heterogeneous (Guillet et al 1987;Oranje et al 1994;Rand and Baden 1983). The expression of the disease may vary within families, and several (familial) cases have been described where KFSD occurs together with metabolic abnormalities (Grosshans et al 1978), with deafness and recurrent infections (Britton et al 1978), and with mental retardation and aminoaciduria (Adler and Nyhan 1969).…”