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Dermpath-India

Pathology of Steatocystoma

Multiplex

Dr Sampurna Roy MD           

 

                                                                                                                      

 

Steatocystoma presents as a solitary lesion (steatocystoma simplex) or multiple lesions (steatocystoma multiplex) in adolescence or young adults.  

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Steatocystoma multiplex is a rare benign autosomal dominant disorder of the pilosebaceous unit. 

Mutations in keratin 17 have been found in some cases.

Site:  These lesions can appear virtually anywhere on the body but are more common in areas where the pilo-sebaceous apparatus is well developed, such as the trunk (especially the presternal area), neck, axilla, inguinal region, scalp, and proximal extremities.

Gross:
Macroscopically these are small, smooth, fleshy, cystic nodule.
  

Microscopic features:

Microscopically the lesion is located in the mid dermis.

The cyst has a wavy wall, lined by squamous epithelium with a corrugated eosinophilic cuticle surface.

No granular layer is usually seen.

Characteristic feature:   Presence of sebaceous glands within or adjacent to the cyst wall. These are flattened lobules of sebaceous glands of varying size.

The cystic space contains, keratin, vellous hair, sebum etc.

Each cyst is attached to overlying normal epidermis by thin strands of undifferentiated epithelial cells.

 

Differential diagnosis :  Eruptive vellous hair cyst, dermoid cyst, cystic sebaceous hyperplasia.

 

Further reading

A novel missense mutation of keratin 17 gene in a chinese family with steatocystoma multiplex.

Steatocystoma.

Increased FDG Uptake in Scrotal Steatocystoma Multiplex With Calcification.

A rare case of facial steatocystoma multiplex.

Acral steatocystoma multiplex.

Steatocystoma multiplex-a rare genetic disorder: a case report and review of the literature.

Acral subcutaneous steatocystoma multiplex: a distinct subtype of the disease?

Syringoma masquerading as steatocystoma multiplex.

Atypical steatocystoma multiplex with calcification.

Steatocystoma multiplex confined to the scalp with concurrent alopecia.

[Steatocystoma multiplex in 39 year-old female].

Case for diagnosis. Steatocystoma multiplex.

Steatocystoma multiplex--an uncommon lesion with special emphasis on cytological features and cyto-histological correlation.

Localized forms of steatocystoma multiplex: Case report and review of the literature.

Steatocystoma multiplex.

Clinical and histologic features of 64 cases of steatocystoma multiplex.

 

 


Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

                                                                                                                                       


 

 

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