Congenital smooth muscle hamartoma explained

Synonyms:CSMH

Congenital smooth muscle hamartoma is typically a skin colored or lightly pigmented patch or plaque with hypertrichosis.[1] [2] Congenital smooth muscle hamartoma was originally reported in 1969 by Sourreil et al.[3]

Signs and symptoms

Although the clinical presentation of congenital smooth muscle hamartoma varies, it typically takes the form of an irregularly shaped, skin-colored, or slightly hyperpigmented patch or plaque on the trunk or extremities that is accompanied by noticeable vellus hairs. Often, it is located in the lumbosacral region.[4]

Causes

Congenital smooth muscle hamartoma most likely arises from an abnormal development that occurs during mesodermal maturation, primarily in the arrector pili muscle.[5] It is hypothesized that hypertrichosis results from the CD34 + dermal dendritic cells in the hamartoma stimulating the bulge's epithelial cells.[6]

There have been reports of familial cases recently, which raise the possibility of a genetic susceptibility.[7]

Rarely do diffuse forms of congenital smooth cell hamartoma cause the skin to fold.[5] It may indicate systemic involvement and is described as a symptom of the Michelin tire infant syndrome.[8]

Diagnosis

Histologically, the main characteristics of CSMH include reticular dermal smooth muscle hyperplasia with differently orientated, clearly defined bundles of smooth muscle[9] [10] that may extend into subcutaneous adipose tissue. Hair follicles and smooth muscle proliferation are frequently tightly related.[11] Increased epidermal pigment is a sign of hyperpigmentation.[9] [10] Immunohistochemical methods have recently clarified a few CSMH markers. It has been observed that CD34-positive dermal dendritic cells are an inherent component of smooth muscle hamartomas.[6] Furthermore, to distinguish clearly between skin cancers of myofibroblastic or fibroblastic origin and spindled smooth muscle cell soft tissue tumors, the cytoskeletal protein h-caldesmon has been employed as a smooth muscle cell-specific marker.[12]

See also

Further reading

External links

Notes and References

  1. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. .
  2. Book: Rapini, Ronald P. . Bolognia, Jean L. . Jorizzo, Joseph L. . Dermatology: 2-Volume Set . Mosby . St. Louis . 2007 . 1835–6 . 978-1-4160-2999-1 .
  3. Goldman . Mitchel P. . Kaplan . Richard P. . Heng . Madalene C. Y. . Congenital Smooth-Muscle Hamartoma . International Journal of Dermatology . Wiley . 26 . 7 . 1987 . 0011-9059 . 10.1111/j.1365-4362.1987.tb00589.x . 448–452. 3654038 . 7812091 .
  4. Metzker . A. . Amir . J. . Rotem . A. . Merlob . P. . Congenital Smooth Muscle Hamartoma of the Skin . Pediatric Dermatology . Wiley . 2 . 1 . 1984 . 0736-8046 . 10.1111/j.1525-1470.1984.tb00441.x . 45–48. 6504777 . 263416250 .
  5. Web site: Raboudi . Asma . Litaiem . Noureddine . Congenital Smooth Muscle Hamartoma . StatPearls Publishing . 2023-06-26 . 31424772 . 2024-02-17.
  6. CD34-positive dendritic cells are an intrinsic part of smooth muscle hamartoma . British Journal of Dermatology . Oxford University Press (OUP) . 140 . 1 . 1999 . 0007-0963 . 10.1046/j.1365-2133.1999.02633.x . 172–174 . Koizumi . Kodama . Tsuji . Matsumura . Nabeshima . Ohkawara . 10215795 . 28839250 .
  7. García-Gavín . Juan . Pérez-Pérez . Lidia . Allegue . Francisco . Pérez-Pedrosa . Alberto . Ortíz-Rey . Jose Antonio . Zulaica . Ánder . Multiple congenital familial smooth muscle hamartoma in two siblings . Dermatology Online Journal . 18 . 5 . 2012-05-01 . 1087-2108 . 10.5070/D39N52G204 .
  8. Janicke . Elise C. . Nazareth . Michael R. . Rothman . Ilene L. . Generalized Smooth Muscle Hamartoma with Multiple Congenital Anomalies without the "Michelin Tire Baby" Phenotype . Pediatric Dermatology . Wiley . 31 . 6 . 2014-01-03 . 0736-8046 . 10.1111/pde.12280 . 731–733. 24383769 . 39284436 .
  9. Prendiville . Julie . Esterly . Nancy B. . Congenital smooth muscle hamartoma . The Journal of Pediatrics . Elsevier BV . 110 . 5 . 1987 . 0022-3476 . 10.1016/s0022-3476(87)80015-x . 742–744. 3572626 .
  10. Truhan . A. P. . Hypertrichotic skin-colored patches in an infant. Congenital smooth-muscle hamartoma (CSMH) . Archives of Dermatology . American Medical Association (AMA) . 121 . 9 . 1985-09-01 . 0003-987X . 10.1001/archderm.121.9.1997 . 1997, 1200–1. 4037849 .
  11. Huffman . D W . Mallory . S B . Congenital smooth muscle hamartoma . American Family Physician . June 1989 . 39 . 6 . 117–120 . 2729037.
  12. D'Addario . Stephen F. . Morgan . Michael . Talley . Lori . Smoller . Bruce R. . h-Caldesmon as a specific marker of smooth muscle cell differentiation in some soft tissue tumors of the skin . Journal of Cutaneous Pathology . Wiley . 29 . 7 . 2002-07-25 . 0303-6987 . 10.1034/j.1600-0560.2002.290707.x . 426–429. 12139638 . 22091736 .

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