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Alopecia areata 1(AA1)

MedGen UID:
400208
Concept ID:
C1863094
Disease or Syndrome
Synonym: AA1
 
Monarch Initiative: MONDO:0007082
OMIM®: 104000

Definition

Alopecia areata is a genetically determined, immune-mediated disorder of the hair follicle with an estimated lifetime risk of approximately 2%, making it one of the most common human autoimmune diseases. It shows a spectrum of severity that ranges from patchy localized hair loss on the scalp to the complete absence of hair everywhere on the body (Gilhar and Kalish, 2006). [from OMIM]

Clinical features

From HPO
Autoimmunity
MedGen UID:
2136
Concept ID:
C0004368
Pathologic Function
The occurrence of an immune reaction against the organism's own cells or tissues.
Nail pits
MedGen UID:
57463
Concept ID:
C0150993
Finding
Small (typically about 1 mm or less in size) depressions on the dorsal nail surface.
Alopecia totalis
MedGen UID:
75525
Concept ID:
C0263504
Disease or Syndrome
Loss of all scalp hair.
Alopecia universalis
MedGen UID:
120481
Concept ID:
C0263505
Disease or Syndrome
Alopecia areata is a common disorder that causes hair loss. "Alopecia" is a Latin term that means baldness, and "areata" refers to the patchy nature of the hair loss that is typically seen with this condition.\n\nIn most people with alopecia areata, hair falls out in small, round patches, leaving coin-sized areas of bare skin. This patchy hair loss occurs most often on the scalp but can affect other parts of the body as well. Uncommonly, the hair loss involves the entire scalp (in which case the condition is known as alopecia totalis) or the whole body (alopecia universalis). Other rare forms of alopecia areata, which have different patterns of hair loss, have also been reported.\n\nAlopecia areata affects people of all ages, although it most commonly appears in adolescence or early adulthood. Hair loss occurs over a period of weeks. The hair usually grows back after several months, although it may fall out again. In some cases, unpredictable cycles of hair loss followed by regrowth can last for years. In addition to hair loss, some affected individuals have fingernail and toenail abnormalities, such as pits on the surface of the nails.\n\nThe hair loss associated with alopecia areata is not painful or disabling. However, it causes changes in a person's appearance that can profoundly affect quality of life and self-esteem. In some people, the condition can lead to depression, anxiety, and other emotional or psychological issues.
Trachyonychia
MedGen UID:
107484
Concept ID:
C0546956
Disease or Syndrome
Excessive longitudinal ridging that gives the surface of the nail plate a rough appearance. It results from multiple foci of defective keratinization of the proximal nail matrix.
Patchy alopecia
MedGen UID:
350774
Concept ID:
C1862862
Finding
Transient, non-scarring hair loss and preservation of the hair follicle located in in well-defined patches.

Professional guidelines

PubMed

Ramírez-Marín HA, Tosti A
Expert Opin Emerg Drugs 2022 Dec;27(4):379-387. Epub 2022 Nov 21 doi: 10.1080/14728214.2022.2149735. PMID: 36408593
Fukuyama M, Ito T, Ohyama M
J Dermatol 2022 Jan;49(1):19-36. Epub 2021 Oct 28 doi: 10.1111/1346-8138.16207. PMID: 34709679
Starace M, Orlando G, Alessandrini A, Piraccini BM
Am J Clin Dermatol 2020 Feb;21(1):69-84. doi: 10.1007/s40257-019-00479-x. PMID: 31677111

Recent clinical studies

Etiology

King B, Zhang X, Harcha WG, Szepietowski JC, Shapiro J, Lynde C, Mesinkovska NA, Zwillich SH, Napatalung L, Wajsbrot D, Fayyad R, Freyman A, Mitra D, Purohit V, Sinclair R, Wolk R
Lancet 2023 May 6;401(10387):1518-1529. Epub 2023 Apr 14 doi: 10.1016/S0140-6736(23)00222-2. PMID: 37062298
Fukuyama M, Ito T, Ohyama M
J Dermatol 2022 Jan;49(1):19-36. Epub 2021 Oct 28 doi: 10.1111/1346-8138.16207. PMID: 34709679
Afford R, Leung AKC, Lam JM
Curr Pediatr Rev 2021;17(1):45-54. doi: 10.2174/1573396316666200430084825. PMID: 32351186
Simakou T, Butcher JP, Reid S, Henriquez FL
J Autoimmun 2019 Mar;98:74-85. Epub 2018 Dec 15 doi: 10.1016/j.jaut.2018.12.001. PMID: 30558963
Strazzulla LC, Wang EHC, Avila L, Lo Sicco K, Brinster N, Christiano AM, Shapiro J
J Am Acad Dermatol 2018 Jan;78(1):1-12. doi: 10.1016/j.jaad.2017.04.1141. PMID: 29241771

Diagnosis

Gómez-Quispe H, Muñoz Moreno-Arrones O, Hermosa-Gelbard Á, Vañó-Galván S, Saceda-Corralo D
Actas Dermosifiliogr 2023 Jan;114(1):25-32. Epub 2022 Sep 5 doi: 10.1016/j.ad.2022.08.018. PMID: 36067826
Ramírez-Marín HA, Tosti A
Expert Opin Emerg Drugs 2022 Dec;27(4):379-387. Epub 2022 Nov 21 doi: 10.1080/14728214.2022.2149735. PMID: 36408593
Fukuyama M, Ito T, Ohyama M
J Dermatol 2022 Jan;49(1):19-36. Epub 2021 Oct 28 doi: 10.1111/1346-8138.16207. PMID: 34709679
Afford R, Leung AKC, Lam JM
Curr Pediatr Rev 2021;17(1):45-54. doi: 10.2174/1573396316666200430084825. PMID: 32351186
Starace M, Orlando G, Alessandrini A, Piraccini BM
Am J Clin Dermatol 2020 Feb;21(1):69-84. doi: 10.1007/s40257-019-00479-x. PMID: 31677111

Therapy

King B, Zhang X, Harcha WG, Szepietowski JC, Shapiro J, Lynde C, Mesinkovska NA, Zwillich SH, Napatalung L, Wajsbrot D, Fayyad R, Freyman A, Mitra D, Purohit V, Sinclair R, Wolk R
Lancet 2023 May 6;401(10387):1518-1529. Epub 2023 Apr 14 doi: 10.1016/S0140-6736(23)00222-2. PMID: 37062298
Ramírez-Marín HA, Tosti A
Expert Opin Emerg Drugs 2022 Dec;27(4):379-387. Epub 2022 Nov 21 doi: 10.1080/14728214.2022.2149735. PMID: 36408593
King B, Ohyama M, Kwon O, Zlotogorski A, Ko J, Mesinkovska NA, Hordinsky M, Dutronc Y, Wu WS, McCollam J, Chiasserini C, Yu G, Stanley S, Holzwarth K, DeLozier AM, Sinclair R; BRAVE-AA Investigators
N Engl J Med 2022 May 5;386(18):1687-1699. Epub 2022 Mar 26 doi: 10.1056/NEJMoa2110343. PMID: 35334197
Gupta AK, Talukder M, Venkataraman M, Bamimore MA
J Dermatolog Treat 2022 Jun;33(4):1896-1906. Epub 2021 Jul 20 doi: 10.1080/09546634.2021.1945527. PMID: 34159872
Starace M, Orlando G, Alessandrini A, Piraccini BM
Am J Clin Dermatol 2020 Feb;21(1):69-84. doi: 10.1007/s40257-019-00479-x. PMID: 31677111

Prognosis

Fukuyama M, Ito T, Ohyama M
J Dermatol 2022 Jan;49(1):19-36. Epub 2021 Oct 28 doi: 10.1111/1346-8138.16207. PMID: 34709679
Afford R, Leung AKC, Lam JM
Curr Pediatr Rev 2021;17(1):45-54. doi: 10.2174/1573396316666200430084825. PMID: 32351186
Sibaud V
Am J Clin Dermatol 2018 Jun;19(3):345-361. doi: 10.1007/s40257-017-0336-3. PMID: 29256113
Strazzulla LC, Wang EHC, Avila L, Lo Sicco K, Brinster N, Christiano AM, Shapiro J
J Am Acad Dermatol 2018 Jan;78(1):15-24. doi: 10.1016/j.jaad.2017.04.1142. PMID: 29241773
Strazzulla LC, Wang EHC, Avila L, Lo Sicco K, Brinster N, Christiano AM, Shapiro J
J Am Acad Dermatol 2018 Jan;78(1):1-12. doi: 10.1016/j.jaad.2017.04.1141. PMID: 29241771

Clinical prediction guides

King B, Zhang X, Harcha WG, Szepietowski JC, Shapiro J, Lynde C, Mesinkovska NA, Zwillich SH, Napatalung L, Wajsbrot D, Fayyad R, Freyman A, Mitra D, Purohit V, Sinclair R, Wolk R
Lancet 2023 May 6;401(10387):1518-1529. Epub 2023 Apr 14 doi: 10.1016/S0140-6736(23)00222-2. PMID: 37062298
Mahmud MR, Akter S, Tamanna SK, Mazumder L, Esti IZ, Banerjee S, Akter S, Hasan MR, Acharjee M, Hossain MS, Pirttilä AM
Gut Microbes 2022 Jan-Dec;14(1):2096995. doi: 10.1080/19490976.2022.2096995. PMID: 35866234Free PMC Article
King B, Ohyama M, Kwon O, Zlotogorski A, Ko J, Mesinkovska NA, Hordinsky M, Dutronc Y, Wu WS, McCollam J, Chiasserini C, Yu G, Stanley S, Holzwarth K, DeLozier AM, Sinclair R; BRAVE-AA Investigators
N Engl J Med 2022 May 5;386(18):1687-1699. Epub 2022 Mar 26 doi: 10.1056/NEJMoa2110343. PMID: 35334197
Fukuyama M, Ito T, Ohyama M
J Dermatol 2022 Jan;49(1):19-36. Epub 2021 Oct 28 doi: 10.1111/1346-8138.16207. PMID: 34709679
Strazzulla LC, Wang EHC, Avila L, Lo Sicco K, Brinster N, Christiano AM, Shapiro J
J Am Acad Dermatol 2018 Jan;78(1):1-12. doi: 10.1016/j.jaad.2017.04.1141. PMID: 29241771

Recent systematic reviews

Liu M, Gao Y, Yuan Y, Yang K, Shen C, Wang J, Tian J
JAMA Netw Open 2023 Jun 1;6(6):e2320351. doi: 10.1001/jamanetworkopen.2023.20351. PMID: 37368402Free PMC Article
Cruciani M, Masiello F, Pati I, Marano G, Pupella S, De Angelis V
Blood Transfus 2023 Jan;21(1):24-36. Epub 2021 Nov 15 doi: 10.2450/2021.0216-21. PMID: 34967722Free PMC Article
Kinoshita-Ise M, Sachdeva M
J Dermatol 2022 Jan;49(1):4-18. Epub 2021 Nov 22 doi: 10.1111/1346-8138.16233. PMID: 34806223
Toussi A, Barton VR, Le ST, Agbai ON, Kiuru M
J Am Acad Dermatol 2021 Jul;85(1):162-175. Epub 2020 Jun 17 doi: 10.1016/j.jaad.2020.06.047. PMID: 32561373Free PMC Article
Lee HH, Gwillim E, Patel KR, Hua T, Rastogi S, Ibler E, Silverberg JI
J Am Acad Dermatol 2020 Mar;82(3):675-682. Epub 2019 Aug 19 doi: 10.1016/j.jaad.2019.08.032. PMID: 31437543

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