Eccrine angiomatous hamartoma: A rare skin lesion ...

4/4/2017

Eccrine angiomatous hamartoma: A rare skin lesion with diverse histological features :[PAUTHORS], Indian Journal of Dermatology

CASE REPORT

Year : 2012 | Volume : 57 | Issue : 3 | Page : 225227

Eccrine angiomatous hamartoma: A rare skin lesion with diverse histological features Sumit Kar1 , Ajay Krishnan1 , Nitin Gangane2 , 1 Department of Dermatology, Venereology and Leprosy, MGIMS, Sewagram, Wardha, Maharashtra, India 2 Department of Pathology, MGIMS, Sewagram, Wardha, Maharashtra, India Correspondence Address: Sumit Kar Department of Dermatology, Venereology and Leprosy, MGIMS, Sewagram, Wardha, Maharashtra 442 012 India

Abstract Eccrine angiomatous hamartoma (EAH) is an exceedingly rare benign tumorlike lesion prevalent in childhood which may produce pain and marked sweating. The histological features include proliferation of eccrine sweat glands and angiomatous capillary channels. We report an 8yearold girl who had a single lesion on her left lower leg. Physical examination revealed a slightly elevated, 4×7 cm erythematous plaque on the lateral aspect of left leg. Sweating in the lesion was evoked by physical work or emotional stress. There was no pain or tenderness associated with the lesion. The patient had no history of trauma to the site. These lesions were clinically angiomatous, and we obtained the diagnosis by histopathological examination. Histopathological examination of the lesion showed increased numbers of eccrine glands, as well as dilated vascular channels in the deep dermis and subcutaneous tissue. These findings are consistent with EAH.

How to cite this article: Kar S, Krishnan A, Gangane N. Eccrine angiomatous hamartoma: A rare skin lesion with diverse histological features.Indian J Dermatol 2012;57:225227

How to cite this URL: Kar S, Krishnan A, Gangane N. Eccrine angiomatous hamartoma: A rare skin lesion with diverse histological features. Indian J Dermatol [serial online] 2012 [cited 2017 Apr 4 ];57:225227 Available from: http://www.eijd.org/text.asp?2012/57/3/225/96206

Full Text Introduction Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous tumor usually present at birth or during early infancy and childhood. It may produce pain and marked sweating. We report a case of EAH occurring in an 8 year old girl.

Case Report An 8yearold girl presented with bruiselike, nodules noted on her left lower leg for the last 2 years. http://www.eijd.org/printarticle.asp?issn=00195154;year=2012;volume=57;issue=3;spage=225;epage=227;aulast=Kar

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Her nodules were gradually increasing in size and now involved lateral aspect of the left ankle. She had an additional lesion on her left sole. Her lesions have increased in number and grown proportionately with the patient. There was no discharge or pain or any other symptoms on the lesion. She is otherwise developmentally normal and has no musculoskeletal abnormalities. Her past medical history was insignificant. Physical examination revealed an irregular, nodular plaque of 4×7 cm, with an overlying ecchymotic to dark surface beginning below the left ankle joint extending down to the lateral aspect of the dorsum of foot [Figure 1]. Similar lesion was present on the sole on the same side. Nodules were of varying firmness and had no pulsations or bruits. Stroking failed to elicit muscular contraction. Sweating was comparatively more on the skin over the lesions compared to the surrounding skin. No leg length discrepancy was present and the patient had full range of motion of both lower extremities. Examination of the hair, nails and mucous membranes were unremarkable.{Figure 1} Histopathological examination showed a thickened epidermis and an increased number of small and mediumsized blood vessels with thin muscular walls of uneven thickness. An increased number of eccrine glands with large coils composed of an increased number of tubules and ducts with a normal proportion of cells were noted in the dermis. There was abundant fibromyxomatous stroma in these coils. The dermis appeared to have increased thickness with mature fat cells [Figure 2] and [Figure 3]. Collagen bundles were thicker and in haphazard array. Some collagen bundles were oriented perpendicular to the skin surface. The clinical and histopathological findings supported a diagnosis of multiple EAH.{Figure 2}{Figure 3}

Discussion EAH have been reported in both sexes. It has also been reported to occur in older age group. [1] The exact pathogenesis is not know but various theories like abnormal induction of heterotypic dependency with resultant malformation of adnexal as well as mesenchymal elements have been proposed. [2] Clinically, EAH presents as an angiomatous lesion, usually solitary, although several cases with multiple lesions have been described. EAH, generally asymptomatic, occasionally presents with pain and focal hyperhidrosis (accounting for the designation of Sudoriferous angioma adopted by some authors). [3] The hyperhidrosis is presumably an expression of its eccrine component. Hyperhydrosis may be absent in some cases. [4] They have occurred at several sites, particularly the palm and sole, but also on other parts of the feet, on the face, neck and on the trunk. Generally, they enlarge very gradually; more rapid growth has been described during pregnancy [5] and adolescence. On histopathology, EAH is characterized by a dermal proliferation of welldifferentiated eccrine secretory and ductal elements closely associated with thinwalled angiomatous channels. In addition, unusual histopathological variants have been reported and include the infiltration of adipose tissue, [6] increased dermal mucin, [7] and the presence of apocrine glands or pilar structures. Where the angiomatous element predominates, the term sudoriparous angioma has been applied. The nevus sudoriferus type of eccrine nevus may represent the nonangiomatous end of the same spectrum of hamartomas. Clinically, this condition must be differentiated from other neonatal angiomatoses. Sometimes the clinical findings are nonspecific, whereas histological examination may exclude the other conditions. The differential diagnosis of EAH may include an eccrine nevus, tufted angioma, macular telangiectatic mastocytosis, nevus flammeus, glomus tumor and smooth muscle hamartoma. While definitive diagnosis is based upon histology, MRI [8] and ultrasound are useful to delineate the extent of soft tissue and vascular masses. Immunohistochemical staining for S100 and carcinoembryonic antigen (CEA), for eccrine glands and the antifactor VIIIrelated antigens, for vascular channels can also be done. The natural history of EAH is benign and typically slowgrowing. There have been no reported complications. Although aggressive treatment is generally unwarranted, simple excision is usually curative as was done here. Recently botulinum toxin [9] and intralesional sclerosants [10] have been successfully used in the treatment of this condition. It is important to recognize this condition because it is a benign lesion for which aggressive treatment is not indicated. This case is being reported as very few cases have been reported from India.

References

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References 1 2 3 4 5 6 7 8 9 10

Jeong E, Park HJ, Oh ST, Lee JY, Cho BK. Lateonset eccrine angiomatous hamartoma on the forehead. Int J Dermatol 2006;45:5989. Zeller DJ, Goldman RL. Eccrinepilar angiomatous hamartoma: A report of a unique case. Dermatologica 1971;143:1004. Sanmartin O, Botella R, Alegre V, Martinez A, Aliaga A. Congenital eccrine angiomatous hamartoma. Am J Dermatopathol 1992;14:1614. Challa VR, Jona J. Eccrine angiomatous hamartoma: A rare skin lesion with diverse histological features. Dermatologica 1977;155:2069. Gabrielsen TO, Elgjo K, Sommerschild H. Eccrine angiomatous hamartoma of the finger leading to amputation. Clin Exp Dermatol 1991;16:445. Chien AJ, Asgari M, Argenyi ZB. Eccrine angiomatous hamartoma with elements of an arterio venous malformation: A newly recognized variant. J Cutan Pathol 2006;33:4336. Tsunemi Y, Shimazu K, Saeki H, Ihn H, Tamaki K. Eccrine angiomatous hamartoma with massive mucin deposition. Eur J Dermatol 2005;15:2912. Laeng RH, Heilbrunner J, Itin PH. Lateonset eccrine angiomatous hamartoma: Clinical, histological and imaging findings. Dermatology 2001;203:704. Barco D, Baselga E, Alegre M, Curell R, Alomar A. Successful treatment of eccrine angiomatous hamartoma with botulinum toxin. Arch Dermatol 2009;145:2413. Kaliyadan F, Sundeep V, Hiran KR. Late onset eccrine angiomatous hamartoma treated with intralesional sclerosant: A case report. Ind J Dermatolo 2007;52:99101.

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