Multinucleate cell angiohistiocytoma

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Multinucleate cell angiohistiocytoma
SpecialtyDermatology

Multinucleate cell angiohistiocytoma (MCAH) is a cutaneous condition that presents as slowly growing, multiple, discrete but grouped, red to violaceous papules[1]

Signs and symptoms[edit]

Lesions are asymptomatic and most commonly found on the face and acral regions, however they have been described in other locations such as the trunk and, less frequently, the mucous membranes;[2] they are usually unilateral and appear as papules ranging in diameter from 2 to 15mm.[3]

They can be smooth and flat, or they can seem like reddish, pink, violaceous, or brown papules with a slightly elevated dome-shaped surface.[4] There have been a few cases reported with bilateral involvement and even widespread forms.[2] MCAH lesions do not seem to spontaneously remit; instead, they grow over weeks to months.[5]

Causes[edit]

While there are still many unanswered questions regarding the etiology and pathogenesis of MCAH, the general consensus is that lesions result from a reactive process rather than a neoplastic one.[3]

Mechanism[edit]

This disease is thought to be caused by enhanced cutaneous vascularity and an aggressive inflammatory response to intravascular macrophage migration.[6] Furthermore, impacted regions demonstrate an overabundance of estrogen receptor α.[7] Angiogenesis has been associated with estrogen signaling.[8] This discovery may help to explain why MCAH is more common in women and why it is extremely vascular.[6]

Diagnosis[edit]

Histopathologically, multinucleated cells with angular outlines in the dermis and vascular hyperplasia linked to a rise in factor XIIIa-positive fibrohistiocytic interstitial cells are the hallmarks of MCAH.[4] The most specific histological finding (3–10 hyperchromatic nuclei with basophilic cytoplasm) is the presence of multinucleated giant cells, albeit this finding does not necessarily indicate the presence of MCAH.[9][10]

Immunohistochemistry demonstrates that vascular endothelial cells are positive for Factor VIII, vimentin, CD31, and CD34 antibodies,[11][12] but negative for Bcl-2 and HHV-8 antigens.[13][14] Vimentin, Factor XIIIa, CD68, alpha-1-antitrypsin, and lysozyme are expressed by mononuclear cells,[11][13] although neither S100 nor CD1a are.[12] The MAC387 expression is not constant.[12][13] While multinucleate cells are negative for other monocyte/macrophage lineage markers, they are significantly positive for vimentin.[11][13] CD68 expression can be either positive[11] or negative.[13][12] In interstitial and perivascular spindle cells, as well as rarely in multinucleate cells, estrogen receptor α has significant expression.[7]

Angiofibroma/fibrous papule, dermatofibroma, microvenular hemangioma, Kaposi's sarcoma, and acroangiodermatitis are among the histopathologic differential diagnoses.[4]

Treatment[edit]

Treatment is not required because the lesion is benign, but it might be suggested for itchy or unappealing lesions.[4] The literature has reported on a number of treatment approaches, such as intense pulsed light, cryosurgery, corticosteroids, argon and carbon dioxide lasers, and surgical excision.[3]

See also[edit]

References[edit]

  1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  2. ^ a b Wang, Ming; Abdul-fattah, Bilal; Wang, Cuiyan; Zhao, Yun; Qu, Xiaoying; Al-Muriesh, Maher; Wang, Xia; Zhu, Li; Qian, Yue; Huang, Changzheng; Chen, Siyuan (2016-11-28). "Generalized multinucleate cell angiohistiocytoma: case report and literature review". Journal of Cutaneous Pathology. 44 (2). Wiley: 125–134. doi:10.1111/cup.12853. ISSN 0303-6987. PMID 27813137.
  3. ^ a b c Costa, Anderson Alves; Wedy, Glaucia Ferreira; Junior, Walter Belda; Criado, Paulo Ricardo (2020). "Multinucleate cell angiohistiocytoma: an uncommon cutaneous tumor". Anais Brasileiros de Dermatologia. 95 (4). Elsevier BV: 480–483. doi:10.1016/j.abd.2019.10.005. ISSN 0365-0596.
  4. ^ a b c d Applebaum, Danielle S.; Shuja, Fareesa; Hicks, Lindsey; Cockerell, Clay; Hsu, Sylvia (2014-05-16). "Multinucleate cell angiohistiocytoma: a case report and review of the literature". Dermatology Online Journal. 20 (5): 22610. doi:10.5070/D3205022610. ISSN 1087-2108. PMID 24852770.
  5. ^ Richer, V.; Lui, H. (2015-08-12). "Facial multinucleate cell angiohistiocytoma: long-term remission with 585 nm pulsed dye laser". Clinical and Experimental Dermatology. 41 (3). Oxford University Press (OUP): 312–313. doi:10.1111/ced.12717. ISSN 0307-6938. PMID 26269389.
  6. ^ a b Frew, John W. (2015). "Multinucleate Cell Angiohistiocytoma". The American Journal of Dermatopathology. 37 (3). Ovid Technologies (Wolters Kluwer Health): 222–228. doi:10.1097/dad.0000000000000075. ISSN 0193-1091. PMID 25140659.
  7. ^ a b Cesinaro, Anna Maria; Roncati, Luca; Maiorana, Antonio (2010). "Estrogen Receptor Alpha Overexpression In Multinucleate Cell Angiohistiocytoma: New Insights Into the Pathogenesis of a Reactive Process". The American Journal of Dermatopathology. 32 (7): 655–659. doi:10.1097/DAD.0b013e3181d3ca49. ISSN 0193-1091. PMID 20644465.
  8. ^ Losordo, Douglas W.; Isner, Jeffrey M. (2001). "Estrogen and Angiogenesis". Arteriosclerosis, Thrombosis, and Vascular Biology. 21 (1). Ovid Technologies (Wolters Kluwer Health): 6–12. doi:10.1161/01.atv.21.1.6. ISSN 1079-5642. PMID 11145928.
  9. ^ Blanco Barrios, S; Rodríguez Díaz, E; Alvarez Cuesta, C; Galache Osuna, C; Requena Caballero, C; Martínez Merino, A; Requena Caballero, L; Kutzner, H (2005-02-17). "Multinucleate cell angiohistiocytoma: a new case report". Journal of the European Academy of Dermatology and Venereology. 19 (2). Wiley: 208–211. doi:10.1111/j.1468-3083.2005.00983.x. ISSN 0926-9959. PMID 15752293.
  10. ^ Väkevä, Liisa; Saksela, Olli; Kariniemi, Arja-Leena (2003-01-01). "Multinucleate Cell Angiohistiocytoma: A Report of Four Cases in Finland". Acta Dermato-Venereologica. 83 (3). Medical Journals Sweden AB: 222–237. doi:10.1080/00015550310007265. ISSN 0001-5555. PMID 12816161.
  11. ^ a b c d Pérez, Lidia Pérez; Zulaica, Ander; Rodríguez, Luis; Campo, M. Carmen; Peñaranda, José Manuel Suárez; Fernández-Redondo, Virginia; Toribio, Jaime (2006-04-25). "Multinucleate cell angiohistiocytoma. Report of five cases". Journal of Cutaneous Pathology. 33 (5). Wiley: 349–352. doi:10.1111/j.0303-6987.2006.00428.x. ISSN 0303-6987. PMID 16640541.
  12. ^ a b c d Jaconelli, Laure; Kanitakis, Jean; Ktiouet, Salma; Faure, Michel; Claudy, Alain (2009-02-15). "Multinucleate cell angiohistiocytoma: report of three new cases and literature review". Dermatology Online Journal. 15 (2): 4. doi:10.5070/D30Z27T0XZ. ISSN 1087-2108. PMID 19336021.
  13. ^ a b c d e Rawal, Y. B.; Anderson, K. M.; Rawal, S. Y. (2009). "Multinucleate cell angiohistiocytoma: an uncommon mucosal tumour". Clinical and Experimental Dermatology. 34 (3). Oxford University Press (OUP): 333–336. doi:10.1111/j.1365-2230.2008.02939.x. ISSN 0307-6938. PMID 19040523.
  14. ^ Gómez-Mateo, María del Carmen; Monteagudo, Carlos (2013). "Nonepithelial skin tumors with multinucleated giant cells". Seminars in Diagnostic Pathology. 30 (1). Elsevier BV: 58–72. doi:10.1053/j.semdp.2012.01.004. ISSN 0740-2570. PMID 23327730.

Further reading[edit]

  • Roy, Simon F.; Dong, Danni; Myung, Peggy; McNiff, Jennifer M. (2019). "Multinucleate cell angiohistiocytoma: A clinicopathologic study of 62 cases and proposed diagnostic criteria". Journal of Cutaneous Pathology. 46 (8): 563–569. doi:10.1111/cup.13463. ISSN 0303-6987. PMID 30927287.
  • Edgerton, Bryn; Ladha, Malika A.; Hunter, Charlene; Devani, Alim R.; Prajapati, Vimal H. (2019). "Successful treatment of multinucleate cell angiohistiocytoma in an adult male patient with potassium-titanyl-phosphate laser in combination with intralesional corticosteroids". JAAD Case Reports. 5 (10). Elsevier BV: 880–882. doi:10.1016/j.jdcr.2019.08.001. ISSN 2352-5126.

External links[edit]