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Acne inversa

MedGen UID:
57993
Concept ID:
C0162836
Disease or Syndrome
Synonyms: Hidradenitis suppurativa
SNOMED CT: Suppurative hidradenitis (59393003); Hidradenitis suppurativa (59393003); Hidradenitis axillaris (59393003); Apocrine acne (59393003); Verneuil's disease (59393003)
 
HPO: HP:0040154

Definition

A chronic suppurative and cicatricial disease of the apocrine glands occurring chiefly in the axillae in women and in the groin and anal regions in men. It is characterized by poral occlusion with secondary bacterial infection, evolving into abscesses which eventually rupture. As the disease becomes chronic, ulcers appear, sinus tracts enlarge, fistulas develop, and fibrosis and scarring become evident. [from MeSH]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcne inversa

Conditions with this feature

Hidradenitis suppurativa, familial
MedGen UID:
326766
Concept ID:
C1840560
Disease or Syndrome
Acne inversa is a chronic relapsing inflammatory skin disease characterized by recurrent draining sinuses and abscesses, predominantly in skin folds that carry terminal hairs and apocrine glands. Healing occurs with substantial scarring (summary by Jansen et al., 2001). Jansen et al. (2001) provided a detailed history and review of the disorder. Genetic Heterogeneity of Familial Acne Inversa Familial acne inversa-2 with or without Dowling-Degos disease (ACNINV2; 613736) is caused by mutation in the PSENEN gene (607632) on chromosome 19q13, and familial acne inversa-3 (ACNINV3; 613737) is caused by mutation in the PSEN1 gene (104311) on chromosome 14q24.
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). For a discussion of genetic heterogeneity of hypohidrotic/anhidrotic ectodermal dysplasia, see 305100.

Recent clinical studies

Etiology

Nikolakis G, Liakou AI, Bonovas S, Seltmann H, Bonitsis N, Join-Lambert O, Wild T, Karagiannidis I, Zolke-Fischer S, Langner K, Zouboulis CC
Acta Derm Venereol 2017 Apr 6;97(4):493-498. doi: 10.2340/00015555-2591. PMID: 27882387
Nedomansky J, Weiss D, Willinger B, Nickl S, Steininger C
Infection 2016 Apr;44(2):247-9. Epub 2015 Jul 1 doi: 10.1007/s15010-015-0814-6. PMID: 26129687
Nikolakis G, Join-Lambert O, Karagiannidis I, Guet-Revillet H, Zouboulis CC, Nassif A
J Am Acad Dermatol 2015 Nov;73(5 Suppl 1):S12-8. doi: 10.1016/j.jaad.2015.07.041. PMID: 26470608
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
CESKO E, KORBER A, DISSEMOND J
Eur J Dermatol 2009 Sep-Oct;19(5):490-3. Epub 2009 Jun 15 doi: 10.1684/ejd.2009.0710. PMID: 19527985

Diagnosis

Nikolakis G, Liakou AI, Bonovas S, Seltmann H, Bonitsis N, Join-Lambert O, Wild T, Karagiannidis I, Zolke-Fischer S, Langner K, Zouboulis CC
Acta Derm Venereol 2017 Apr 6;97(4):493-498. doi: 10.2340/00015555-2591. PMID: 27882387
Nedomansky J, Weiss D, Willinger B, Nickl S, Steininger C
Infection 2016 Apr;44(2):247-9. Epub 2015 Jul 1 doi: 10.1007/s15010-015-0814-6. PMID: 26129687
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
von Laffert M, Stadie V, Wohlrab J, Marsch WC
Br J Dermatol 2011 Feb;164(2):367-71. Epub 2010 Nov 23 doi: 10.1111/j.1365-2133.2010.10034.x. PMID: 20831631
CESKO E, KORBER A, DISSEMOND J
Eur J Dermatol 2009 Sep-Oct;19(5):490-3. Epub 2009 Jun 15 doi: 10.1684/ejd.2009.0710. PMID: 19527985

Therapy

Zouboulis CC
Expert Rev Clin Immunol 2016 Oct;12(10):1015-26. Epub 2016 Aug 29 doi: 10.1080/1744666X.2016.1221762. PMID: 27531618
Nikolakis G, Join-Lambert O, Karagiannidis I, Guet-Revillet H, Zouboulis CC, Nassif A
J Am Acad Dermatol 2015 Nov;73(5 Suppl 1):S12-8. doi: 10.1016/j.jaad.2015.07.041. PMID: 26470608
Zouboulis CC, Del Marmol V, Mrowietz U, Prens EP, Tzellos T, Jemec GB
Dermatology 2015;231(2):184-90. Epub 2015 Jun 30 doi: 10.1159/000431175. PMID: 26139027
CESKO E, KORBER A, DISSEMOND J
Eur J Dermatol 2009 Sep-Oct;19(5):490-3. Epub 2009 Jun 15 doi: 10.1684/ejd.2009.0710. PMID: 19527985
Goertz RS, Konturek PC, Naegel A, Janka R, Amann K, Maennlein G, Wein A, Hahn EG, Boxberger F
Med Sci Monit 2009 Jan;15(1):CS14-8. PMID: 19114971

Prognosis

Nikolakis G, Liakou AI, Bonovas S, Seltmann H, Bonitsis N, Join-Lambert O, Wild T, Karagiannidis I, Zolke-Fischer S, Langner K, Zouboulis CC
Acta Derm Venereol 2017 Apr 6;97(4):493-498. doi: 10.2340/00015555-2591. PMID: 27882387
Giamarellos-Bourboulis EJ, Platzer M, Karagiannidis I, Kanni T, Nikolakis G, Ulrich J, Bellutti M, Gollnick H, Bauer M, Zouboulis CC, Huse K
J Invest Dermatol 2016 Aug;136(8):1592-8. Epub 2016 May 7 doi: 10.1016/j.jid.2016.04.021. PMID: 27164300
Danby FW
J Am Acad Dermatol 2015 Nov;73(5 Suppl 1):S52-4. doi: 10.1016/j.jaad.2015.07.042. PMID: 26470617
Nikolakis G, Join-Lambert O, Karagiannidis I, Guet-Revillet H, Zouboulis CC, Nassif A
J Am Acad Dermatol 2015 Nov;73(5 Suppl 1):S12-8. doi: 10.1016/j.jaad.2015.07.041. PMID: 26470608
Goertz RS, Konturek PC, Naegel A, Janka R, Amann K, Maennlein G, Wein A, Hahn EG, Boxberger F
Med Sci Monit 2009 Jan;15(1):CS14-8. PMID: 19114971

Clinical prediction guides

Tsaousi A, Witte E, Witte K, Röwert-Huber HJ, Volk HD, Sterry W, Wolk K, Schneider-Burrus S, Sabat R
Mediators Inflamm 2016;2016:4097574. Epub 2016 Oct 23 doi: 10.1155/2016/4097574. PMID: 27843200Free PMC Article
Karagiannidis I, Nikolakis G, Sabat R, Zouboulis CC
Rev Endocr Metab Disord 2016 Sep;17(3):335-341. doi: 10.1007/s11154-016-9366-z. PMID: 27294593
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
Danby FW
J Am Acad Dermatol 2015 Nov;73(5 Suppl 1):S52-4. doi: 10.1016/j.jaad.2015.07.042. PMID: 26470617
Kurek A, Johanne Peters EM, Sabat R, Sterry W, Schneider-Burrus S
J Dtsch Dermatol Ges 2013 Aug;11(8):743-9, 743-50. Epub 2013 Apr 9 doi: 10.1111/ddg.12067. PMID: 23565584

Recent systematic reviews

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
Woodruff CM, Charlie AM, Leslie KS
Mayo Clin Proc 2015 Dec;90(12):1679-93. doi: 10.1016/j.mayocp.2015.08.020. PMID: 26653298
Gerard AJ, Feldman SR, Strowd L
J Cutan Med Surg 2015 Jul-Aug;19(4):391-6. Epub 2015 Mar 5 doi: 10.1177/1203475415575013. PMID: 25775617
Scheinfeld N
Dermatol Online J 2015 Jan 15;21(1) PMID: 25612117
Haslund P, Lee RA, Jemec GB
Acta Derm Venereol 2009 Nov;89(6):595-600. doi: 10.2340/00015555-0747. PMID: 19997689

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