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Keratosis pilaris atrophicans

Keratosis pilaris atropicans is a group of idiopathic genodermatoses that consists of three unique clinical entities: atrophoderma vermiculatum, keratosis follicularis spinulosa decalvans, and keratosis pilaris atrophicans faciei.[1]

Signs and symptoms edit

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.[2]

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.[3]

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.[4]

Causes edit

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.[5]

It has been demonstrated that a mutation in the desmoglein 4 gene causes autosomal recessive keratosis pilaris atrophicans.[6]

Diagnosis edit

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.[5]

Treatment edit

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.[5]

See also edit

References edit

  1. ^ Callaway, Sanders R.; Lesher, Jack L. (2004). "Keratosis Pilaris Atrophicans: Case Series and Review". Pediatric Dermatology. 21 (1): 14–17. doi:10.1111/j.0736-8046.2004.21103.x. ISSN 0736-8046.
  2. ^ Fekete, Gyula; Fekete, László; Neagu, Nicoleta; Bacârea, Vladimir; Drăgănescu, Miruna; Brihan, Ilarie (2021-09-20). "Keratosis pilaris atrophicans faciei: An observational, descriptive, retrospective clinical study". Experimental and Therapeutic Medicine. 22 (5). Spandidos Publications. doi:10.3892/etm.2021.10766. ISSN 1792-0981. PMC 8495552.
  3. ^ Lee, Young Chae; Son, Sook-Ja; Han, Tae Young; Lee, June Hyunkyung (2018). "A Case of Atrophoderma Vermiculatum Showing a Good Response to Topical Tretinoin". Annals of Dermatology. 30 (1). Korean Dermatological Association and The Korean Society for Investigative Dermatology: 116. doi:10.5021/ad.2018.30.1.116. ISSN 1013-9087.
  4. ^ Maheswari, UmaG; Chaitra, V; Mohan, SubbiahS (2013). "Keratosis follicularis spinulosa decalvans: A rare cause of scarring alopecia in two young Indian girls". International Journal of Trichology. 5 (1). Medknow: 29. doi:10.4103/0974-7753.114713. ISSN 0974-7753. PMC 3746224.
  5. ^ a b c "UpToDate". UpToDate. Retrieved 2024-03-17.
  6. ^ Cohen-Barak, E; Danial-Farran, N; Hammad, H; Aleme, O; Krauz, J; Gavish, E; Khayat, M; Ziv, M; Shalev, S (2018). "Desmoglein 4 Mutation Underlies Autosomal Recessive Keratosis Pilaris Atrophicans". Acta Dermato Venereologica. 98 (8). Medical Journals Sweden AB: 809–810. doi:10.2340/00015555-2976. ISSN 0001-5555.

Further reading edit

  • Malvankar, DipaliD; Sacchidanand, S (2015). "Keratosis follicularis spinulosa decalvans: A report of three cases". International Journal of Trichology. 7 (3). Medknow: 125. doi:10.4103/0974-7753.167461. ISSN 0974-7753. PMC 4639957.
  • Fekete, Gyula; Fekete, László; Neagu, Nicoleta; Bacârea, Vladimir; Drăgănescu, Miruna; Brihan, Ilarie (2021-09-20). "Keratosis pilaris atrophicans faciei: An observational, descriptive, retrospective clinical study". Experimental and Therapeutic Medicine. 22 (5). Spandidos Publications. doi:10.3892/etm.2021.10766. ISSN 1792-0981. PMC 8495552.

External links edit

keratosis, pilaris, atrophicans, keratosis, pilaris, atropicans, group, idiopathic, genodermatoses, that, consists, three, unique, clinical, entities, atrophoderma, vermiculatum, keratosis, follicularis, spinulosa, decalvans, keratosis, pilaris, atrophicans, f. Keratosis pilaris atropicans is a group of idiopathic genodermatoses that consists of three unique clinical entities atrophoderma vermiculatum keratosis follicularis spinulosa decalvans and keratosis pilaris atrophicans faciei 1 Keratosis pilaris atrophicansSpecialtyDermatologyTypesKeratosis pilaris atrophicans faciei atrophoderma vermiculatum and keratosis follicularis spinulosa decalvans Contents 1 Signs and symptoms 2 Causes 3 Diagnosis 4 Treatment 5 See also 6 References 7 Further reading 8 External linksSigns and symptoms editKeratosis 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 2 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 3 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 4 Causes editThe 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 5 It has been demonstrated that a mutation in the desmoglein 4 gene causes autosomal recessive keratosis pilaris atrophicans 6 Diagnosis editKeratosis 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 5 Treatment editFor 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 5 See also editCicatricial alopecia List of cutaneous conditionsReferences edit Callaway Sanders R Lesher Jack L 2004 Keratosis Pilaris Atrophicans Case Series and Review Pediatric Dermatology 21 1 14 17 doi 10 1111 j 0736 8046 2004 21103 x ISSN 0736 8046 Fekete Gyula Fekete Laszlo Neagu Nicoleta Bacarea Vladimir Drăgănescu Miruna Brihan Ilarie 2021 09 20 Keratosis pilaris atrophicans faciei An observational descriptive retrospective clinical study Experimental and Therapeutic Medicine 22 5 Spandidos Publications doi 10 3892 etm 2021 10766 ISSN 1792 0981 PMC 8495552 Lee Young Chae Son Sook Ja Han Tae Young Lee June Hyunkyung 2018 A Case of Atrophoderma Vermiculatum Showing a Good Response to Topical Tretinoin Annals of Dermatology 30 1 Korean Dermatological Association and The Korean Society for Investigative Dermatology 116 doi 10 5021 ad 2018 30 1 116 ISSN 1013 9087 Maheswari UmaG Chaitra V Mohan SubbiahS 2013 Keratosis follicularis spinulosa decalvans A rare cause of scarring alopecia in two young Indian girls International Journal of Trichology 5 1 Medknow 29 doi 10 4103 0974 7753 114713 ISSN 0974 7753 PMC 3746224 a b c UpToDate UpToDate Retrieved 2024 03 17 Cohen Barak E Danial Farran N Hammad H Aleme O Krauz J Gavish E Khayat M Ziv M Shalev S 2018 Desmoglein 4 Mutation Underlies Autosomal Recessive Keratosis Pilaris Atrophicans Acta Dermato Venereologica 98 8 Medical Journals Sweden AB 809 810 doi 10 2340 00015555 2976 ISSN 0001 5555 Further reading editMalvankar DipaliD Sacchidanand S 2015 Keratosis follicularis spinulosa decalvans A report of three cases International Journal of Trichology 7 3 Medknow 125 doi 10 4103 0974 7753 167461 ISSN 0974 7753 PMC 4639957 Fekete Gyula Fekete Laszlo Neagu Nicoleta Bacarea Vladimir Drăgănescu Miruna Brihan Ilarie 2021 09 20 Keratosis pilaris atrophicans faciei An observational descriptive retrospective clinical study Experimental and Therapeutic Medicine 22 5 Spandidos Publications doi 10 3892 etm 2021 10766 ISSN 1792 0981 PMC 8495552 External links edit Retrieved from https en wikipedia org w index php title Keratosis pilaris atrophicans amp oldid 1214695426, wikipedia, wiki, book, books, library,

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