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Acne inversa

MedGen UID:
57993
Concept ID:
C0162836
Disease or Syndrome
Synonym: Hidradenitis suppurativa
SNOMED CT: Suppurative hidradenitis (59393003); Hidradenitis suppurativa (59393003); Hidradenitis axillaris (59393003); Apocrine acne (59393003); Verneuil's disease (59393003)
 
HPO: HP:0040154
Monarch Initiative: MONDO:0006559
Orphanet: ORPHA387

Definition

A chronic skin condition involving the inflammation of the apocrine sweat glands, forming pimple-like bumps known as abscesses. [from HPO]

Conditions with this feature

Acute febrile neutrophilic dermatosis
MedGen UID:
43097
Concept ID:
C0085077
Disease or Syndrome
Acute febrile neutrophilic dermatosis (AFND) is an autosomal dominant autoinflammatory disorder characterized by onset of recurrent fever and dermatologic abnormalities in childhood. Laboratory studies show elevated acute-phase reactants and activation of the inflammatory response, particularly IL1B (147720). Additional more variable features may include myalgia and arthralgia (summary by Masters et al., 2016).
Follicular atrophoderma and basal cell epitheliomata
MedGen UID:
87539
Concept ID:
C0346104
Neoplastic Process
Bazex-Dupre-Christol syndrome (BDCS) is an X-linked dominant disorder characterized by a triad of congenital hypotrichosis, follicular atrophoderma affecting the dorsa of the hands and feet, the face, and extensor surfaces of the elbows or knees, and the development of basal cell neoplasms, including basal cell nevi and basal cell carcinomas from the second decade onward (Yung and Newton-Bishop, 2005). Rombo syndrome (180730) has similar features, but shows autosomal dominant inheritance.
Granulocytopenia with immunoglobulin abnormality
MedGen UID:
383874
Concept ID:
C1856263
Disease or Syndrome
Immunodeficiency-59 and hypoglycemia (IMD59) is an autosomal recessive primary immunologic disorder characterized by combined immunodeficiency and recurrent septic infections of the respiratory tract, skin, and mucous membranes, as well as disturbed glucose metabolism. Granulocytopenia and B-cell and dendritic cell deficiency are present (Haapaniemi et al., 2017).
Acne inversa, familial, 2
MedGen UID:
462387
Concept ID:
C3151037
Disease or Syndrome
Acne inversa is a chronic inflammatory disease of the hair follicles whose characteristic features include draining sinuses, painful skin abscesses, and disfiguring scars. Manifestations typically appear after puberty. Familial acne inversa is genetically heterogeneous (summary by Wang et al., 2010). Some patients with PSENEN-associated acne inversa also exhibit reticulate hyperpigmentation consistent with Dowling-Degos disease (DDD; see 179850) (Zhou et al., 2016). For a general phenotypic description and a discussion of genetic heterogeneity of acne inversa, see 142690.
Acne inversa, familial, 3
MedGen UID:
462388
Concept ID:
C3151038
Disease or Syndrome
Acne inversa is a chronic inflammatory disease of the hair follicles whose characteristic features include draining sinuses, painful skin abscesses, and disfiguring scars. Manifestations typically appear after puberty. Familial acne inversa is genetically heterogeneous (summary by Wang et al., 2010). For a general phenotypic description and a discussion of genetic heterogeneity of familial acne inversa, see 142690.
Ectodermal dysplasia 12, hypohidrotic/hair/tooth/nail type
MedGen UID:
934583
Concept ID:
C4310616
Disease or Syndrome
Some ectodermal dysplasias are here classified as congenital disorders characterized by abnormal development in 2 or more ectodermal structures (hair, nails, teeth, and sweat glands) without other systemic findings. Hypohidrotic, or anhidrotic, ectodermal dysplasia (HED/EDA) is characterized by a triad of signs comprising sparse hair (hypotrichosis), abnormal or missing teeth (anodontia or hypodontia), and inability to sweat (anhidrosis or hypohidrosis). Typical clinical manifestations also include dryness of the skin, eyes, airways, and mucous membranes presumably due to the defective development of several exocrine glands. Hypohidrotic ectodermal dysplasia can be associated with dysmorphic features (forehead bumps, rings under the eyes, everted nose, and prominent lips) and occasionally with absent nipples (summary by Cluzeau et al., 2011).
Acne inversa, familial, 1
MedGen UID:
1631104
Concept ID:
C4551962
Disease or Syndrome
Any familial acne inversa in which the cause of the disease is a mutation in the NCSTN gene.
Immunodeficiency 15a
MedGen UID:
1648385
Concept ID:
C4748694
Disease or Syndrome
Immunodeficiency 15A (IMD15A) is an autosomal dominant primary immunodeficiency disorder characterized by relatively late onset of recurrent respiratory tract infections and lymphopenia, combined with immune activation of both CD4+ and CD8+ T cells. One patient presented with inflammatory disease and possible ectodermal defect.

Professional guidelines

PubMed

Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):76-90. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.067. PMID: 30872156Free PMC Article
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):91-101. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.068. PMID: 30872149Free PMC Article
Zouboulis CC, Desai N, Emtestam L, Hunger RE, Ioannides D, Juhász I, Lapins J, Matusiak L, Prens EP, Revuz J, Schneider-Burrus S, Szepietowski JC, van der Zee HH, Jemec GB
J Eur Acad Dermatol Venereol 2015 Apr;29(4):619-44. Epub 2015 Jan 30 doi: 10.1111/jdv.12966. PMID: 25640693

Recent clinical studies

Etiology

Zouboulis CC, Benhadou F, Byrd AS, Chandran NS, Giamarellos-Bourboulis EJ, Fabbrocini G, Frew JW, Fujita H, González-López MA, Guillem P, Gulliver WPF, Hamzavi I, Hayran Y, Hórvath B, Hüe S, Hunger RE, Ingram JR, Jemec GBE, Ju Q, Kimball AB, Kirby JS, Konstantinou MP, Lowes MA, MacLeod AS, Martorell A, Marzano AV, Matusiak Ł, Nassif A, Nikiphorou E, Nikolakis G, Nogueira da Costa A, Okun MM, Orenstein LAV, Pascual JC, Paus R, Perin B, Prens EP, Röhn TA, Szegedi A, Szepietowski JC, Tzellos T, Wang B, van der Zee HH
Exp Dermatol 2020 Dec;29(12):1154-1170. doi: 10.1111/exd.14214. PMID: 33058306
Tchero H, Herlin C, Bekara F, Fluieraru S, Teot L
Indian J Dermatol Venereol Leprol 2019 May-Jun;85(3):248-257. doi: 10.4103/ijdvl.IJDVL_69_18. PMID: 30924446
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):76-90. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.067. PMID: 30872156Free PMC Article
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):91-101. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.068. PMID: 30872149Free PMC Article
Zouboulis CC, Desai N, Emtestam L, Hunger RE, Ioannides D, Juhász I, Lapins J, Matusiak L, Prens EP, Revuz J, Schneider-Burrus S, Szepietowski JC, van der Zee HH, Jemec GB
J Eur Acad Dermatol Venereol 2015 Apr;29(4):619-44. Epub 2015 Jan 30 doi: 10.1111/jdv.12966. PMID: 25640693

Diagnosis

Jenkins T, Isaac J, Edwards A, Okoye GA
Dermatol Clin 2023 Jul;41(3):471-479. Epub 2023 Apr 15 doi: 10.1016/j.det.2023.02.001. PMID: 37236715
Hendricks AJ, Hsiao JL, Lowes MA, Shi VY
Dermatology 2021;237(1):81-96. Epub 2019 Oct 23 doi: 10.1159/000503605. PMID: 31645040
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):76-90. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.067. PMID: 30872156Free PMC Article
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):91-101. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.068. PMID: 30872149Free PMC Article
Zouboulis CC, Desai N, Emtestam L, Hunger RE, Ioannides D, Juhász I, Lapins J, Matusiak L, Prens EP, Revuz J, Schneider-Burrus S, Szepietowski JC, van der Zee HH, Jemec GB
J Eur Acad Dermatol Venereol 2015 Apr;29(4):619-44. Epub 2015 Jan 30 doi: 10.1111/jdv.12966. PMID: 25640693

Therapy

Zouboulis CC, Benhadou F, Byrd AS, Chandran NS, Giamarellos-Bourboulis EJ, Fabbrocini G, Frew JW, Fujita H, González-López MA, Guillem P, Gulliver WPF, Hamzavi I, Hayran Y, Hórvath B, Hüe S, Hunger RE, Ingram JR, Jemec GBE, Ju Q, Kimball AB, Kirby JS, Konstantinou MP, Lowes MA, MacLeod AS, Martorell A, Marzano AV, Matusiak Ł, Nassif A, Nikiphorou E, Nikolakis G, Nogueira da Costa A, Okun MM, Orenstein LAV, Pascual JC, Paus R, Perin B, Prens EP, Röhn TA, Szegedi A, Szepietowski JC, Tzellos T, Wang B, van der Zee HH
Exp Dermatol 2020 Dec;29(12):1154-1170. doi: 10.1111/exd.14214. PMID: 33058306
Sabat R, Jemec GBE, Matusiak Ł, Kimball AB, Prens E, Wolk K
Nat Rev Dis Primers 2020 Mar 12;6(1):18. doi: 10.1038/s41572-020-0149-1. PMID: 32165620
Tchero H, Herlin C, Bekara F, Fluieraru S, Teot L
Indian J Dermatol Venereol Leprol 2019 May-Jun;85(3):248-257. doi: 10.4103/ijdvl.IJDVL_69_18. PMID: 30924446
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):76-90. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.067. PMID: 30872156Free PMC Article
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):91-101. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.068. PMID: 30872149Free PMC Article

Prognosis

Jfri A, Nassim D, O'Brien E, Gulliver W, Nikolakis G, Zouboulis CC
JAMA Dermatol 2021 Aug 1;157(8):924-931. doi: 10.1001/jamadermatol.2021.1677. PMID: 34037678Free PMC Article
Alikhan A, Sayed C, Alavi A, Alhusayen R, Brassard A, Burkhart C, Crowell K, Eisen DB, Gottlieb AB, Hamzavi I, Hazen PG, Jaleel T, Kimball AB, Kirby J, Lowes MA, Micheletti R, Miller A, Naik HB, Orgill D, Poulin Y
J Am Acad Dermatol 2019 Jul;81(1):91-101. Epub 2019 Mar 11 doi: 10.1016/j.jaad.2019.02.068. PMID: 30872149Free PMC Article
Prens E, Deckers I
J Am Acad Dermatol 2015 Nov;73(5 Suppl 1):S8-11. doi: 10.1016/j.jaad.2015.07.045. PMID: 26470623
Buimer MG, Wobbes T, Klinkenbijl JH
Br J Surg 2009 Apr;96(4):350-60. doi: 10.1002/bjs.6569. PMID: 19283748
Jansen T, Plewig G
Int J Dermatol 1998 Feb;37(2):96-100. doi: 10.1046/j.1365-4362.1998.00414.x. PMID: 9542661

Clinical prediction guides

Wu KK, Dao H Jr
J Dermatolog Treat 2022 Feb;33(1):41-47. Epub 2020 Mar 9 doi: 10.1080/09546634.2020.1737638. PMID: 32116066
Jfri A, Nassim D, O'Brien E, Gulliver W, Nikolakis G, Zouboulis CC
JAMA Dermatol 2021 Aug 1;157(8):924-931. doi: 10.1001/jamadermatol.2021.1677. PMID: 34037678Free PMC Article
Tchero H, Herlin C, Bekara F, Fluieraru S, Teot L
Indian J Dermatol Venereol Leprol 2019 May-Jun;85(3):248-257. doi: 10.4103/ijdvl.IJDVL_69_18. PMID: 30924446
Zouboulis CC, Tzellos T, Kyrgidis A, Jemec GBE, Bechara FG, Giamarellos-Bourboulis EJ, Ingram JR, Kanni T, Karagiannidis I, Martorell A, Matusiak Ł, Pinter A, Prens EP, Presser D, Schneider-Burrus S, von Stebut E, Szepietowski JC, van der Zee HH, Wilden SM, Sabat R; European Hidradenitis Suppurativa Foundation Investigator Group
Br J Dermatol 2017 Nov;177(5):1401-1409. Epub 2017 Oct 30 doi: 10.1111/bjd.15748. PMID: 28636793
Gulliver W, Zouboulis CC, Prens E, Jemec GB, Tzellos T
Rev Endocr Metab Disord 2016 Sep;17(3):343-351. doi: 10.1007/s11154-016-9328-5. PMID: 26831295Free PMC Article

Recent systematic reviews

Abu Rached N, Gambichler T, Dietrich JW, Ocker L, Seifert C, Stockfleth E, Bechara FG
Int J Mol Sci 2022 Dec 3;23(23) doi: 10.3390/ijms232315250. PMID: 36499573Free PMC Article
Phan K, Ng WHS, Lai B, Garg A, Smith SD
J Dermatolog Treat 2022 Jun;33(4):2309-2316. Epub 2021 Aug 9 doi: 10.1080/09546634.2021.1959502. PMID: 34289795
Jfri A, Nassim D, O'Brien E, Gulliver W, Nikolakis G, Zouboulis CC
JAMA Dermatol 2021 Aug 1;157(8):924-931. doi: 10.1001/jamadermatol.2021.1677. PMID: 34037678Free PMC Article
Tchero H, Herlin C, Bekara F, Fluieraru S, Teot L
Indian J Dermatol Venereol Leprol 2019 May-Jun;85(3):248-257. doi: 10.4103/ijdvl.IJDVL_69_18. PMID: 30924446
Zouboulis CC, Bechara FG, Dickinson-Blok JL, Gulliver W, Horváth B, Hughes R, Kimball AB, Kirby B, Martorell A, Podda M, Prens EP, Ring HC, Tzellos T, van der Zee HH, van Straalen KR, Vossen ARJV, Jemec GBE
J Eur Acad Dermatol Venereol 2019 Jan;33(1):19-31. Epub 2018 Oct 23 doi: 10.1111/jdv.15233. PMID: 30176066Free PMC Article

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