Eccrine angiomatous hamartoma (EAH), first described by Lotzbeck in 1859, is a rare benign vascular hamartoma characterized histologically by a proliferation of eccrine and vascular components.[1][2] EAH exists on a spectrum of cutaneous tumors that include eccrine nevus, mucinous eccrine nevus and EAH. Each diagnostic subtype is characterized by an increase in the number as well as size of mature eccrine glands or ducts, with EAH being distinguished by the added vascular component.[3][4]
Patients with EAH may present with complaints of pain or increased sweating (hyperhidrosis) associated with stress or exercise, or without any associated symptoms.[3][4] It usually appears as a solitary nodular lesion on the acral areas of the extremities, particularly the palms and soles.[5] Onset of EAH most commonly arises in children prior to puberty as a solitary, unilateral, large, red to violaceous nodule or plaque located on the extremities.[3][4][6][7] Although rare, there have been reports of multiple EAH lesions occurring within a single patient in a linear, grouped, agminated or blaschkoid distribution.[8]
EAH most commonly presents as a solitary papule or plaque on the extremities of children and are frequently congenital, although they can appear in adulthood as well.[2][7] Rarely, multiple EAH lesions have been reported in a single patient, most often in an agminated pattern located on the extremities. A predisposition of EAH for facial and truncal involvement is not commonly seen. Some reports have demonstrated lesion predominance on the distal extremities.[6][7][9][10][11][12] Fewer accounts detail distribution on the head, neck and lower back.[3][8][13] Cases reporting lesions in uncommon locations, such as the trunk or abdomen, typically involved only a solitary lesion. Whereas EAH occurring as multiple lesions was more often reported in classic sites of involvement such as the arm or leg.[14][15]
Although EAH is often asymptomatic, it is known to cause variable levels of pain. This is thought to occur as a result of small nerves that are seen on electron microscopy in close proximity to the eccrine and vascular structures.[16]Hypertrichosis of the tumor is encountered in most cases. Hyperhidrosis is an additional diagnostic feature that is seen in under half of reported cases.[16] There is also significant cosmetic concern in some instances.[8][17]
Causesedit
The pathophysiologic mechanism underlying the hamartoma is thought to involve a biochemical fault in the interactions between differentiating epithelium and subjacent mesenchyme giving rise to an abnormal proliferation of adnexal and vascular structures.[2][17][18]
Diagnosisedit
A skin biopsy is typically performed for definitive diagnosis. The histopathologic hallmarks of EAH include the presence of an increased number of eccrine glands in the mid- and lower dermis along with ectatic or collapsed vessels that are seen in close approximation to the hyperplastic eccrine units. The overlying epidermis may be normal or may show acanthosis or papillomatosis.[3][4][6][16]
A recent report of EAH located on the neck described dermatoscopic features of multiple yellow, confluent nodules in a popcorn-like shape over a background of erythema and linear, arborizing vessels.[8] Dermoscopy is minimally invasive, inexpensive and may provide another diagnostic modality in the differentiation of EAH from other diagnoses, but has yet to be validated.[8]
Differential Diagnosisedit
Vascular malformations:
Eccrine nevus – Characterized histopathologically by an increase in eccrine structures but not capillaries. Clinical hallmark is hyperhidrosis in most cases.
Smooth muscle hamartoma – These flesh-colored plaques may have associated hypertrichosis. A "chicken-skin" appearance (pseudo-Darier sign) may be seen with piloerection.
Glomus tumor – Painful bluish papules, single or multiple, are encountered, mainly on acral areas of the body.
Blue rubber bleb nevus
Sudoriparous angioma – Another rare, benign tumor where eccrine glands of normal number are seen lying close to vascular structures in the dermis; these have a larger caliber than those seen in EAH.
EAH is a benign hamartoma and if there is no associated pain or cosmetic concern or disfiguration, EAH may be observed only. Treatment is often unnecessary.[7] Most cases of symptomatic EAH have been treated with surgical resection, with a few efficacious alternative treatments available.[19][20]
^Lotzbeck (January 1859). "Ein Fall von Schweissdrüsengeschwulst an der Wange". Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin. 16 (1–2): 160–165. doi:10.1007/bf01945254. ISSN 0945-6317. S2CID 21220116.
^ abcGarcía-Arpa, Mónica; Rodríguez-Vázquez, María; Cortina-de la Calle, Pilar; Romero-Aguilera, Guillermo; López-Pérez, Rafael (2005-01-01). "Multiple and Familial Eccrine Angiomatous Hamartoma". Acta Dermato-Venereologica. -1 (1): 355–7. doi:10.1080/00015550510027072. ISSN 0001-5555. PMID 16191863.
^ abcdeTempark, T.; Shwayder, T. (2012-12-18). "Mucinous eccrine naevus: case report and review of the literature". Clinical and Experimental Dermatology. 38 (1): 1–6. doi:10.1111/ced.12034. ISSN 0307-6938. PMID 23252751.
^ abcdLarralde, Margarita; Bazzolo, Eleonora; Boggio, Paula; Abad, María Eugenia; Santos Muñoz, Andrea (May 2009). "Eccrine Angiomatous Hamartoma: Report of Five Congenital Cases". Pediatric Dermatology. 26 (3): 316–319. doi:10.1111/j.1525-1470.2008.00777.x. ISSN 0736-8046. PMID 19706095. S2CID 26198059.
^James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 581. ISBN0-7216-2921-0.
^ abcSulica, R. Lucien; Kao, Grace F.; Sulica, Virginia I.; Penneys, Neal S. (February 1994). "Eccrine angiomatous hamartoma (nevus):. Immunohistochemical findings and review of the literature". Journal of Cutaneous Pathology. 21 (1): 71–75. doi:10.1111/j.1600-0560.1994.tb00694.x. ISSN 0303-6987. PMID 7514619. S2CID 40831123.
^ abcdNakatsui, Thomas C.; Schloss, Eric; Krol, Alfons; Lin, Andrew N. (July 1999). "Eccrine angiomatous hamartoma: Report of a case and literature review". Journal of the American Academy of Dermatology. 41 (1): 109–111. doi:10.1016/s0190-9622(99)70416-0. ISSN 0190-9622. PMID 10411421.
^Sanmartin, Onofre; Botella, Rafael; Alegre, Victor; Martinez, Antonio; Aliaga, Adolfo (April 1992). "Congenital Eccrine Angiomatous Hamartoma". The American Journal of Dermatopathology. 14 (2): 161–164. doi:10.1097/00000372-199204000-00015. ISSN 0193-1091. PMID 1566976.
^Shin, Jaeyoung; Jang, Yong Hyun; Kim, Soo-Chan; Kim, You Chan (2013). "Eccrine Angiomatous Hamartoma: A Review of Ten Cases". Annals of Dermatology. 25 (2): 208–12. doi:10.5021/ad.2013.25.2.208. ISSN 1013-9087. PMC3662915. PMID 23717013.
^Jorge-Finnigan, Conrado; Conejero, Claudia; Hernández-Martín, Angela; Sánchez-Gómez, Julian; Noguera-Morel, Lucero (March 2015). "Congenital Erythematous Plaques and Papules on the Right Arm". Pediatric Dermatology. 32 (2): 285–286. doi:10.1111/pde.12463. ISSN 0736-8046. PMID 25801080. S2CID 35363409.
^ abPelle, Michelle T.; Pride, Howard B.; Tyler, William B. (September 2002). "Eccrine angiomatous hamartoma". Journal of the American Academy of Dermatology. 47 (3): 429–435. doi:10.1067/mjd.2002.121030. ISSN 0190-9622. PMID 12196755.
^Foshee, J. B.; Grau, Renee H.; Adelson, David M.; Crowson, Neil (July 2006). "Eccrine Angiomatous Harmartoma in an Infant". Pediatric Dermatology. 23 (4): 365–368. doi:10.1111/j.1525-1470.2006.00255.x. ISSN 0736-8046. PMID 16918635. S2CID 6352879.
^Barco, Didac (2009-03-01). "Successful Treatment of Eccrine Angiomatous Hamartoma With Botulinum Toxin". Archives of Dermatology. 145 (3): 241–3. doi:10.1001/archdermatol.2008.575. ISSN 0003-987X. PMID 19289750.
^Felgueiras, João; del Pozo, Jesús; Sacristán, Felipe; Bonet, Maria del Mar (March 2015). "Eccrine Angiomatous Hamartoma". Dermatologic Surgery. 41 (3): 428–430. doi:10.1097/dss.0000000000000297. ISSN 1076-0512. PMID 25705946. S2CID 41880499.
January 01, 1970
eccrine, angiomatous, hamartoma, this, article, technical, most, readers, understand, please, help, improve, make, understandable, experts, without, removing, technical, details, october, 2020, learn, when, remove, this, message, first, described, lotzbeck, 18. This article may be too technical for most readers to understand Please help improve it to make it understandable to non experts without removing the technical details October 2020 Learn how and when to remove this message Eccrine angiomatous hamartoma EAH first described by Lotzbeck in 1859 is a rare benign vascular hamartoma characterized histologically by a proliferation of eccrine and vascular components 1 2 EAH exists on a spectrum of cutaneous tumors that include eccrine nevus mucinous eccrine nevus and EAH Each diagnostic subtype is characterized by an increase in the number as well as size of mature eccrine glands or ducts with EAH being distinguished by the added vascular component 3 4 Patients with EAH may present with complaints of pain or increased sweating hyperhidrosis associated with stress or exercise or without any associated symptoms 3 4 It usually appears as a solitary nodular lesion on the acral areas of the extremities particularly the palms and soles 5 Onset of EAH most commonly arises in children prior to puberty as a solitary unilateral large red to violaceous nodule or plaque located on the extremities 3 4 6 7 Although rare there have been reports of multiple EAH lesions occurring within a single patient in a linear grouped agminated or blaschkoid distribution 8 Contents 1 Signs and Symptoms 2 Causes 3 Diagnosis 3 1 Differential Diagnosis 4 Treatment 5 See also 6 ReferencesSigns and Symptoms editEAH most commonly presents as a solitary papule or plaque on the extremities of children and are frequently congenital although they can appear in adulthood as well 2 7 Rarely multiple EAH lesions have been reported in a single patient most often in an agminated pattern located on the extremities A predisposition of EAH for facial and truncal involvement is not commonly seen Some reports have demonstrated lesion predominance on the distal extremities 6 7 9 10 11 12 Fewer accounts detail distribution on the head neck and lower back 3 8 13 Cases reporting lesions in uncommon locations such as the trunk or abdomen typically involved only a solitary lesion Whereas EAH occurring as multiple lesions was more often reported in classic sites of involvement such as the arm or leg 14 15 Although EAH is often asymptomatic it is known to cause variable levels of pain This is thought to occur as a result of small nerves that are seen on electron microscopy in close proximity to the eccrine and vascular structures 16 Hypertrichosis of the tumor is encountered in most cases Hyperhidrosis is an additional diagnostic feature that is seen in under half of reported cases 16 There is also significant cosmetic concern in some instances 8 17 Causes editThe pathophysiologic mechanism underlying the hamartoma is thought to involve a biochemical fault in the interactions between differentiating epithelium and subjacent mesenchyme giving rise to an abnormal proliferation of adnexal and vascular structures 2 17 18 Diagnosis editA skin biopsy is typically performed for definitive diagnosis The histopathologic hallmarks of EAH include the presence of an increased number of eccrine glands in the mid and lower dermis along with ectatic or collapsed vessels that are seen in close approximation to the hyperplastic eccrine units The overlying epidermis may be normal or may show acanthosis or papillomatosis 3 4 6 16 A recent report of EAH located on the neck described dermatoscopic features of multiple yellow confluent nodules in a popcorn like shape over a background of erythema and linear arborizing vessels 8 Dermoscopy is minimally invasive inexpensive and may provide another diagnostic modality in the differentiation of EAH from other diagnoses but has yet to be validated 8 Differential Diagnosis edit Vascular malformations Eccrine nevus Characterized histopathologically by an increase in eccrine structures but not capillaries Clinical hallmark is hyperhidrosis in most cases Tufted angioma Smooth muscle hamartoma These flesh colored plaques may have associated hypertrichosis A chicken skin appearance pseudo Darier sign may be seen with piloerection Glomus tumor Painful bluish papules single or multiple are encountered mainly on acral areas of the body Blue rubber bleb nevus Sudoriparous angioma Another rare benign tumor where eccrine glands of normal number are seen lying close to vascular structures in the dermis these have a larger caliber than those seen in EAH Macules Nevus flammeus Angioma serpiginosum Telangiectasia macularis eruptive perstansTreatment editEAH is a benign hamartoma and if there is no associated pain or cosmetic concern or disfiguration EAH may be observed only Treatment is often unnecessary 7 Most cases of symptomatic EAH have been treated with surgical resection with a few efficacious alternative treatments available 19 20 See also editHamartomas Skin lesion Eccrine NevusReferences edit Lotzbeck January 1859 Ein Fall von Schweissdrusengeschwulst an der Wange Archiv fur Pathologische Anatomie und Physiologie und fur Klinische Medicin 16 1 2 160 165 doi 10 1007 bf01945254 ISSN 0945 6317 S2CID 21220116 a b c Garcia Arpa Monica Rodriguez Vazquez Maria Cortina de la Calle Pilar Romero Aguilera Guillermo Lopez Perez Rafael 2005 01 01 Multiple and Familial Eccrine Angiomatous Hamartoma Acta Dermato Venereologica 1 1 355 7 doi 10 1080 00015550510027072 ISSN 0001 5555 PMID 16191863 a b c d e Tempark T Shwayder T 2012 12 18 Mucinous eccrine naevus case report and review of the literature Clinical and Experimental Dermatology 38 1 1 6 doi 10 1111 ced 12034 ISSN 0307 6938 PMID 23252751 a b c d Larralde Margarita Bazzolo Eleonora Boggio Paula Abad Maria Eugenia Santos Munoz Andrea May 2009 Eccrine Angiomatous Hamartoma Report of Five Congenital Cases Pediatric Dermatology 26 3 316 319 doi 10 1111 j 1525 1470 2008 00777 x ISSN 0736 8046 PMID 19706095 S2CID 26198059 James William Berger Timothy Elston Dirk 2005 Andrews Diseases of the Skin Clinical Dermatology 10th ed Saunders Page 581 ISBN 0 7216 2921 0 a b c Sulica R Lucien Kao Grace F Sulica Virginia I Penneys Neal S February 1994 Eccrine angiomatous hamartoma nevus Immunohistochemical findings and review of the literature Journal of Cutaneous Pathology 21 1 71 75 doi 10 1111 j 1600 0560 1994 tb00694 x ISSN 0303 6987 PMID 7514619 S2CID 40831123 a b c d Nakatsui Thomas C Schloss Eric Krol Alfons Lin Andrew N July 1999 Eccrine angiomatous hamartoma Report of a case and literature review Journal of the American Academy of Dermatology 41 1 109 111 doi 10 1016 s0190 9622 99 70416 0 ISSN 0190 9622 PMID 10411421 a b c d e Garcia Garcia Sandra Cecilia Saeb Lima Marcela Villarreal Martinez Alejandra Vazquez Martinez Osvaldo Tomas Lopez Carrera Yuri Igor Ocampo Candiani Jorge Gomez Flores Minerva March 2018 Dermoscopy of eccrine angiomatous hamartoma The popcorn pattern JAAD Case Reports 4 2 165 167 doi 10 1016 j jdcr 2017 08 014 ISSN 2352 5126 PMC 5789522 PMID 29387774 Zeller D J Goldman R L 1971 Eccrine Pilar Angiomatous Hamartoma Dermatology 143 2 100 104 doi 10 1159 000252176 ISSN 1018 8665 Kikuchi Ichiro Kuroki Yasumasa Inoue Shouhei August 1982 Painful Eccrine Angiomatous Nevus on the Sole The Journal of Dermatology 9 4 329 332 doi 10 1111 j 1346 8138 1982 tb02642 x ISSN 0385 2407 PMID 6759552 S2CID 10085945 Velasco J A Almeida V 1988 Eccrine Pilar Angiomatous Nevus Dermatology 177 5 317 322 doi 10 1159 000248587 ISSN 1018 8665 PMID 3072225 Sanmartin Onofre Botella Rafael Alegre Victor Martinez Antonio Aliaga Adolfo April 1992 Congenital Eccrine Angiomatous Hamartoma The American Journal of Dermatopathology 14 2 161 164 doi 10 1097 00000372 199204000 00015 ISSN 0193 1091 PMID 1566976 Shin Jaeyoung Jang Yong Hyun Kim Soo Chan Kim You Chan 2013 Eccrine Angiomatous Hamartoma A Review of Ten Cases Annals of Dermatology 25 2 208 12 doi 10 5021 ad 2013 25 2 208 ISSN 1013 9087 PMC 3662915 PMID 23717013 Jorge Finnigan Conrado Conejero Claudia Hernandez Martin Angela Sanchez Gomez Julian Noguera Morel Lucero March 2015 Congenital Erythematous Plaques and Papules on the Right Arm Pediatric Dermatology 32 2 285 286 doi 10 1111 pde 12463 ISSN 0736 8046 PMID 25801080 S2CID 35363409 CEBREIRO SANCHEZ AGUILAR GoMEZ CENTENO FERNANDEZ REDONDO TORIBIO November 1998 Eccrine angiomatous hamartoma report of seven cases Clinical and Experimental Dermatology 23 6 267 270 doi 10 1046 j 1365 2230 1998 00391 x ISSN 0307 6938 PMID 10233623 S2CID 2426476 a b c VisualDx Eccrine angiomatous hamartoma VisualDx Retrieved 2018 09 29 a b Pelle Michelle T Pride Howard B Tyler William B September 2002 Eccrine angiomatous hamartoma Journal of the American Academy of Dermatology 47 3 429 435 doi 10 1067 mjd 2002 121030 ISSN 0190 9622 PMID 12196755 Foshee J B Grau Renee H Adelson David M Crowson Neil July 2006 Eccrine Angiomatous Harmartoma in an Infant Pediatric Dermatology 23 4 365 368 doi 10 1111 j 1525 1470 2006 00255 x ISSN 0736 8046 PMID 16918635 S2CID 6352879 Barco Didac 2009 03 01 Successful Treatment of Eccrine Angiomatous Hamartoma With Botulinum Toxin Archives of Dermatology 145 3 241 3 doi 10 1001 archdermatol 2008 575 ISSN 0003 987X PMID 19289750 Felgueiras Joao del Pozo Jesus Sacristan Felipe Bonet Maria del Mar March 2015 Eccrine Angiomatous Hamartoma Dermatologic Surgery 41 3 428 430 doi 10 1097 dss 0000000000000297 ISSN 1076 0512 PMID 25705946 S2CID 41880499 Retrieved from https en wikipedia org w index php title Eccrine angiomatous hamartoma amp oldid 1171070594, wikipedia, wiki, book, books, library,
