U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Congenital smooth muscle hamartoma

MedGen UID:
590701
Concept ID:
C0406819
Neoplastic Process
Synonyms: Congenital arrector pili hamartoma; Congenital erector pili hamartoma; congenital smooth muscle hamartoma; Nevus of pilar muscle; Smooth muscle nevus
SNOMED CT: Congenital arrector pili hamartoma (239144007); Nevus of pilar muscle (239144007); Smooth muscle nevus (239144007); Congenital smooth muscle hamartoma (239144007); Congenital erector pili hamartoma (239144007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016986
Orphanet: ORPHA263435

Definition

Congenital smooth muscle hamartoma (CSMH) is a rare cutaneous hamartomatous lesion most often located on the lumbosacral area or proximal limbs (but rarely on atypical areas such as scalp, eyelid or foot) and characterized by a disorganized proliferation of smooth muscle fibres of arrector pili presenting usually as a localized skin-colored or hyperpigmented plaque (up to 10 cm in diameter) with prominent vellus hairs (most common classic form) or less commonly by multiple skin-colored papules that can coalesce to form irregularly shaped plaques. With time, hyperpigmentation and vellus hairs usually diminish and neither malignant transformation nor associated systemic involvement has been reported. [from ORDO]

Professional guidelines

Recent clinical studies

Etiology

Meza V, Aranibar L, Wortsman X
J Ultrasound Med 2025 Apr;44(4):779-785. Epub 2024 Dec 18 doi: 10.1002/jum.16636. PMID: 39692078
Kim YJ, Roh MR, Lee JH, Na JI, Ko JY, Jung JM, Lee JH, Chang SE
Int J Dermatol 2018 Jan;57(1):55-61. Epub 2017 Oct 31 doi: 10.1111/ijd.13779. PMID: 29090468
Palit A, Inamadar AC, Athanikar SB, Sampagavi VV, Deshmukh NS, Yelikar BR
Indian J Dermatol Venereol Leprol 2005 May-Jun;71(3):217-8. doi: 10.4103/0378-6323.16252. PMID: 16394425
Zvulunov A, Rotem A, Merlob P, Metzker A
Am J Dis Child 1990 Jul;144(7):782-4. doi: 10.1001/archpedi.1990.02150310050024. PMID: 2356798
Huffman DW, Mallory SB
Am Fam Physician 1989 Jun;39(6):117-20. PMID: 2729037

Diagnosis

Meza V, Aranibar L, Wortsman X
J Ultrasound Med 2025 Apr;44(4):779-785. Epub 2024 Dec 18 doi: 10.1002/jum.16636. PMID: 39692078
Kim HS, Kim YC
Am J Dermatopathol 2024 Jan 1;46(1):68. Epub 2023 Oct 26 doi: 10.1097/DAD.0000000000002585. PMID: 37883970
Bilgiç Ö, Tunçez Akyürek F, Altınyazar HC
J Pediatr 2016 Feb;169:318. Epub 2015 Nov 25 doi: 10.1016/j.jpeds.2015.10.084. PMID: 26621046
Ustüner P, Aşkin U, Seçkin D, Ozen O
Indian J Dermatol Venereol Leprol 2008 Nov-Dec;74(6):701. doi: 10.4103/0378-6323.45145. PMID: 19180688
Johnson MD, Jacobs AH
Arch Dermatol 1989 Jun;125(6):820-2. doi: 10.1001/archderm.125.6.820. PMID: 2658846

Therapy

Szychta P, Dixon M, Raine C
Anal Quant Cytopathol Histpathol 2012 Oct;34(5):285-8. PMID: 23301388
Johnson MD, Jacobs AH
Arch Dermatol 1989 Jun;125(6):820-2. doi: 10.1001/archderm.125.6.820. PMID: 2658846

Prognosis

Kim YJ, Roh MR, Lee JH, Na JI, Ko JY, Jung JM, Lee JH, Chang SE
Int J Dermatol 2018 Jan;57(1):55-61. Epub 2017 Oct 31 doi: 10.1111/ijd.13779. PMID: 29090468
Palit A, Inamadar AC, Athanikar SB, Sampagavi VV, Deshmukh NS, Yelikar BR
Indian J Dermatol Venereol Leprol 2005 May-Jun;71(3):217-8. doi: 10.4103/0378-6323.16252. PMID: 16394425
Zvulunov A, Rotem A, Merlob P, Metzker A
Am J Dis Child 1990 Jul;144(7):782-4. doi: 10.1001/archpedi.1990.02150310050024. PMID: 2356798

Clinical prediction guides

Kim YJ, Roh MR, Lee JH, Na JI, Ko JY, Jung JM, Lee JH, Chang SE
Int J Dermatol 2018 Jan;57(1):55-61. Epub 2017 Oct 31 doi: 10.1111/ijd.13779. PMID: 29090468
Szychta P, Dixon M, Raine C
Anal Quant Cytopathol Histpathol 2012 Oct;34(5):285-8. PMID: 23301388
van Kooten EO, Hage JJ, Meinhardt W, Horenblas S, Mooi WJ
J Cutan Pathol 2004 May;31(5):388-92. doi: 10.1111/j.0303-6987.2004.00191.x. PMID: 15059225
Zvulunov A, Rotem A, Merlob P, Metzker A
Am J Dis Child 1990 Jul;144(7):782-4. doi: 10.1001/archpedi.1990.02150310050024. PMID: 2356798
Johnson MD, Jacobs AH
Arch Dermatol 1989 Jun;125(6):820-2. doi: 10.1001/archderm.125.6.820. PMID: 2658846

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...