Skin nonmelanocytic tumor

Cysts

Steatocystoma



Last author update: 14 February 2024
Last staff update: 14 February 2024

Copyright: 2002-2024, PathologyOutlines.com, Inc.

PubMed Search: Steatocystoma

Claire Mazahery, M.D., Ph.D.
Bethany R. Rohr, M.D.
Page views in 2023: 18,481
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Cite this page: Mazahery C, Rohr BR. Steatocystoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticsteatocystoma.html. Accessed April 14th, 2024.
Definition / general
  • Benign cysts with squamous epithelial lining and associated sebaceous glands
  • Can present as single or multiple flesh colored papules
Essential features
  • Benign, sebaceous gland associated cysts, with a characteristic hyaline cuticle and jagged squamous epithelial lining
  • Can occur as sporadic lesions or in an autosomal dominant inheritance pattern
  • Keratin 17 (KRT17) is the most common mutation
Terminology
  • Steatocystoma simplex (single lesion clinically)
  • Steatocystoma multiplex (multiple lesions clinically)
ICD coding
  • ICD-10: L72.2 - steatocystoma multiplex
Epidemiology
  • No gender predominance
Sites
  • Face and neck, chest, arms, axillae
Etiology
  • Sporadic (simplex and multiplex) (J Dermatol 2002;29:152)
  • Autosomal dominant mutation in keratin 17 (multiplex)
Clinical features
Diagnosis
  • Generally a clinical diagnosis aided by histopathology
  • May be incidentally found on imaging
Radiology description
  • Not required for diagnosis
  • Well circumscribed, subepidermal fat signals, generally found incidentally on ultrasound, mammogram or noncontrast magnetic resonance imaging (MRI) (Breast J 2021;27:389, Cureus 2022;14:e27756)
Prognostic factors
Case reports
Clinical images

Images hosted on other servers:
Skin colored papules

Skin colored papules

Single yellow hued papule

Single yellow hued papule

Multiple papules on cheek

Multiple papules on cheek

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Bethany R. Rohr, M.D. and Claire Mazahery, M.D., Ph.D.
sebaceous glands in lining

Sebaceous glands in lining

sawtooth hyaline cuticle

Sawtooth hyaline cuticle

characteristic lining, debris, gland

Characteristic lining, debris, gland

sebaceous glands in wall

Sebaceous glands in wall

multiple sebaceous glands

Multiple sebaceous glands

sebaceous glands abutting lumen

Sebaceous glands abutting lumen

Virtual slides

Images hosted on other servers:
Skin, steatocystoma

Skin, steatocystoma

Positive stains
Videos

Steatocystoma: 5 minute pathology pearls by Dr. Jerad Gardner

Sample pathology report
  • Skin, left proximal forearm, shave biopsy:
    • Steatocystoma (see comment)
    • Comment: In the dermis, there is a cyst with a stratified squamous epithelial lining lacking a granular layer. There is a sawtoothed pattern cuticle and scattered sebaceous glands in the cyst lining.
Differential diagnosis
Board review style question #1

A 30 year old woman presented with a flesh colored papule on the arm. A biopsy was performed. What is the predominant inheritance pattern of the familial form of this entity?

  1. Autosomal dominant mutations in keratin 17
  2. Autosomal dominant mutations in keratin 18
  3. Autosomal recessive mutations in keratin 17
  4. Sporadic lesions only
Board review style answer #1
A. Autosomal dominant mutations in keratin 17. The solitary lesion in the question above is steatocystoma simplex. Steatocystoma multiplex can be seen as a sporadic or an autosomal dominant disease presenting with multiple steatocystomas. The heritable form is thought to be due to mutations in keratin 17. Answer D is incorrect because although the patient in the question stem has a single lesion (steatocystoma simplex, which is sporadic), steatocystoma multiplex has identical lesions on histology. Answer C is incorrect because steatocystoma multiplex is an autosomal dominant (not recessive) mutation in keratin 17. Answer B is incorrect because the mutated gene is keratin 17, not keratin 18.

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Reference: Steatocystoma
Board review style question #2
A 40 year old man presents with a longstanding history of multiple asymptomatic, firm, flesh colored papules across the chest and upper back. He notes several family members with similar skin lesions. A close physical examination revealed thickened nails that he says have been a source of embarrassment since childhood. What adnexal structure is associated with his skin lesions?

  1. Apocrine glands
  2. Eccrine glands
  3. Follicular structures
  4. Sebaceous glands
Board review style answer #2
D. Sebaceous glands. The patient's nail changes present since early life are suggestive of pachyonychia congenita, which can co-occur with familial steatocystoma multiplex (keratin 17 mutations present in both diseases), accounting for the skin findings in this patient and his family members. The cysts of steatocystoma multiplex are associated with sebaceous glands. Answers A and B are incorrect because these may be seen in hidrocystoma. Answer C is incorrect because these are found in follicular based cysts (such as pilar cyst, vellus cyst or epidermal inclusion cyst).

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Reference: Steatocystoma
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