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Steatocystoma multiplexL72.20

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 21.12.2021

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Synonym(s)

Sebaceous cysts; Sebocystomatosis Günther; Steatoma multiplex; Steatomatosis; Steatoms

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HistoryThis section has been translated automatically.

Jamieson, 1873

DefinitionThis section has been translated automatically.

Nevoid cystic adnexal tumors with sebaceous gland differentiation. Steatocystoma multiplex may occur in association with multiple vellus hair cysts as well as pachyonychia congenita, both of which may share the same genetic defect.

EtiopathogenesisThis section has been translated automatically.

Autosomal-dominantly inherited mutation of the gene KRT17, which is mapped on the gene locus 17q12-q21, with consecutive disturbance of keratin 17. In such cases, other developmental anomalies of the tissues derived from the ectoderm may occur simultaneously, such as on nails, hair and teeth (see Fig).

Pathogenetically, there is an enlargement of sebaceous gland follicles with rudimentary sebaceous gland infundibulum without continuous connection to the skin surface. A closed sterile cyst develops from the underlying sebaceous gland acini, ducts and hair systems, so that there is usually no inflammation.

Only in the clinical picture of Steatocystoma multiplex conglobatum chronic inflammatory processes are observed. In this case, clinical forms can develop as in hidradenitis suppurativa (Alotaibi L et al. 2019).

In addition to autosomal dominant inheritance, sporadic forms of steatocystoma multiplex also occur (Jiang M et al. 2020).

ManifestationThis section has been translated automatically.

Occurring during puberty.

LocalizationThis section has been translated automatically.

Especially the chest, armpits and back are affected. Rarely also appearing on the forehead or scrotum.

Clinical featuresThis section has been translated automatically.

Mostly numerous, disseminated, 1-2 mm to 1.0 cm large, deeply dermal, skin-coloured, sometimes bluish shimmering cysts.

HistologyThis section has been translated automatically.

Alternating large epithelial cyst, dermal and subcutaneous. Cyst wall with sawtooth-like cuticular horny layer. No stratum granulosum, storage of sebaceous gland cells or sebaceous azini. In the lumen: corneal cell masses, sebum, often hair.

Differential diagnosisThis section has been translated automatically.

Closed comedones; apocrine sweat gland tumors; acne cystica; tricholemic cyst.

Complication(s)This section has been translated automatically.

Steatocystoma multiplex conglobatum.

TherapyThis section has been translated automatically.

Cosmetically disturbing cysts can be excised. Due to the large number of cysts, complete removal is often not possible. Isotretinoin (e.g. isotretinoin-ratiopharm; acne normin) 0.5 mg/kg bw/day is helpful for some patients.

Note(s)This section has been translated automatically.

A congenital linear form was reported.

LiteratureThis section has been translated automatically.

  1. Alotaibi L et al (2019) Steatocystoma Multiplex Suppurativa: A Case with Unusual Giant Cysts over the Scalp and Neck. Case Rep Dermatol 11:71-76.
  2. Cho S et al (2002) Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol 29: 152-156
  3. Jamieson WA (1873) Case of numerous cutaneous cysts scattered over the body. Edin Med J 19: 223-225
  4. Rollins T et al (2000) Acral steatocystoma multiplex. J Am Acad Dermatol 43: 396-369
  5. Rongioletti F et al (2002) Late onset vulvar steatocystoma multiplex. Clin Exp Dermatol 27: 445-447
  6. Rossi R et al (2003) CO2 laser therapy in a case of steatocystoma multiplex with prominent nodules on the face and neck. Int J Dermatol 42: 302-304
  7. Sardana K et al (2002) Facial steatocystoma multiplex associated with pilar cyst and bilateral preauricular sinus. J Dermatol 29: 157-159
  8. Schmook T (2001) Surgical pearl: mini-incisions for the extraction of steatocystoma multiplex. J Am Acad Dermatol 44: 1041-1042.
  9. Shin NY et al (2019) Steatocystoma multiplex: A case report of a rare entity. Imaging Sci Dent 49:317-321.

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Last updated on: 21.12.2021

Author: Prof. Dr. med. Peter Altmeyer