Steatocystoma multiplex

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Steatocystoma multiplex
SkinTumors-P6260388.JPG
Specialty Dermatology Edit this on Wikidata

Steatocystoma multiplex, also known as epidermal polycystic disease and sebocystomatosis,[1]:781 is a benign, autosomal dominant congenital condition resulting in multiple cysts on a person's body. Steatocystoma simplex is the solitary counterpart to steatocystoma multiplex.[2]

Causes and genetics[edit]

Steatocystoma multiplex has an autosomal dominant pattern of inheritance.

It is associated with defects in Keratin 17.[3] The condition is inherited in an autosomal dominant manner. This indicates that the defective gene responsible for a disorder is located on an autosome, and only one copy of the defective gene is sufficient to cause the disorder, when inherited from a parent who has the disorder.

However, a solitary case can also emerge in a family with no prior history of the disorder due to the occurrence of a mutation (often referred to as a sporadic or spontaneous mutation).[citation needed]

Diagnosis[edit]

Treatment[edit]

The cysts can be removed via excision, though conventional cyst excision techniques have proven impractical, and a specialized regimen is required.[4]

See also[edit]

References[edit]

  1. ^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  2. ^ Davey, Mathew. "Steatocystoma Multiplex". Retrieved 25 May 2011. 
  3. ^ Smith FJ, Corden LD, Rugg EL, et al. (1997). "Missense mutations in keratin 17 cause either pachyonychia congenita type 2 or a phenotype resembling steatocystoma multiplex". J. Invest. Dermatol. 108 (2): 220–3. doi:10.1111/1523-1747.ep12335315. PMID 9008238. 
  4. ^ Pamoukian VN, Westreich M (1997). "Five generations with steatocystoma multiplex congenita: a treatment regimen". Plast. Reconstr. Surg. 99 (4): 1142–6. doi:10.1097/00006534-199704000-00036. PMID 9091916. 

External links[edit]

Classification
External resources