Keratosis pilaris atrophicans

Keratosis pilaris atrophicans faciei manifests as follicular, horny papules encircled by an erythematous halo encompassing the chin, cheeks, forehead, and eyebrows. Gradual hair loss occurs on the lateral edges of the eyebrows after this.{{cite journal | last=Fekete | first=Gyula | last2=Fekete | first2=László | last3=Neagu | first3=Nicoleta | last4=Bacârea | first4=Vladimir | last5=Drăgănescu | first5=Miruna | last6=Brihan | first6=Ilarie | title=Keratosis pilaris atrophicans faciei: An observational, descriptive, retrospective clinical study | journal=Experimental and Therapeutic Medicine | publisher=Spandidos Publications | volume=22 | issue=5 | date=2021-09-20 | issn=1792-0981 | doi=10.3892/etm.2021.10766 | page=| pmc=8495552 }}

Atrophoderma vermiculatum is characterized by the formation of keratotic, inflammatory papules on the face that atrophicate, leave behind pitted scars that resemble honeycombs or reticula.{{cite journal | last=Lee | first=Young Chae | last2=Son | first2=Sook-Ja | last3=Han | first3=Tae Young | last4=Lee | first4=June Hyunkyung | title=A Case of Atrophoderma Vermiculatum Showing a Good Response to Topical Tretinoin | journal=Annals of Dermatology | publisher=Korean Dermatological Association and The Korean Society for Investigative Dermatology | volume=30 | issue=1 | year=2018 | issn=1013-9087 | doi=10.5021/ad.2018.30.1.116 | page=116}}

Follic hyperkeratosis and scarring alopecia are keratosis follicularis spinulosa decalvans clinical hallmarks. Early childhood or infancy is when follicular hyperkeratosis first appears on the face, affecting the nose, forehead, cheeks, and eyebrows. Alopecia areata that leaves scars on the scalp and eyebrows starts in early childhood and gets worse. Additional related features include excessive periungal cuticles, corneal dystrophy with photophobia, and palmoplantar keratoderma.{{cite journal | last=Maheswari | first=UmaG | last2=Chaitra | first2=V | last3=Mohan | first3=SubbiahS | title=Keratosis follicularis spinulosa decalvans: A rare cause of scarring alopecia in two young Indian girls | journal=International Journal of Trichology | publisher=Medknow | volume=5 | issue=1 | year=2013 | issn=0974-7753 | doi=10.4103/0974-7753.114713 | doi-access=free | page=29| pmc=3746224 }}

The aberrant keratinization of the follicular infundibulum causes keratosis pilaris atrophicans, which is characterized by irritation and blockage of the growing hair shaft. Alopecia, fibrosis, atrophy, and shrinking of the hair bulb are caused by persistent inflammation. The possibility that the genes controlling follicular keratinization are located on chromosome 18p is suggested by the association with a number of congenital disorders caused by partial monosomy or deletion in chromosomal arm 18p.{{cite web | title=UpToDate | website=UpToDate |url=https://www.uptodate.com/contents/keratosis-pilaris-atrophicans | access-date=2024-03-17}}

It has been demonstrated that a mutation in the desmoglein 4 gene causes autosomal recessive keratosis pilaris atrophicans.{{cite journal | last=Cohen-Barak | first=E | last2=Danial-Farran | first2=N | last3=Hammad | first3=H | last4=Aleme | first4=O | last5=Krauz | first5=J | last6=Gavish | first6=E | last7=Khayat | first7=M | last8=Ziv | first8=M | last9=Shalev | first9=S | title=Desmoglein 4 Mutation Underlies Autosomal Recessive Keratosis Pilaris Atrophicans | journal=Acta Dermato Venereologica | publisher=Medical Journals Sweden AB | volume=98 | issue=8 | year=2018 | issn=0001-5555 | doi=10.2340/00015555-2976 | pages=809–810}}

Keratosis pilaris atrophicans can be diagnosed clinically. Usually, a skin biopsy is not required to make the diagnosis. When carried out, it exhibits general characteristics such as modest perifollicular inflammatory infiltration and keratotic plugs in the pilosebaceous units.

For keratosis pilaris atrophicans, there are no proven treatment options. With age, the condition frequently becomes better. It is advised to use ultraviolet (UV) protection because the condition is frequently made worse by light.

• Cicatricial alopecia
• List of cutaneous conditions

{{reflist}}

• {{cite journal | last=Malvankar | first=DipaliD | last2=Sacchidanand | first2=S | title=Keratosis follicularis spinulosa decalvans: A report of three cases | journal=International Journal of Trichology | publisher=Medknow | volume=7 | issue=3 | year=2015 | issn=0974-7753 | doi=10.4103/0974-7753.167461 | doi-access=free | page=125 | ref=none| pmc=4639957 }}
• {{cite journal | last=Fekete | first=Gyula | last2=Fekete | first2=László | last3=Neagu | first3=Nicoleta | last4=Bacârea | first4=Vladimir | last5=Drăgănescu | first5=Miruna | last6=Brihan | first6=Ilarie | title=Keratosis pilaris atrophicans faciei: An observational, descriptive, retrospective clinical study | journal=Experimental and Therapeutic Medicine | publisher=Spandidos Publications | volume=22 | issue=5 | date=2021-09-20 | issn=1792-0981 | doi=10.3892/etm.2021.10766 | ref=none| pmc=8495552 }}

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Category:Conditions of the skin appendages