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Table of Contents 
CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 5  |  Page : 510-512
Epitheloid hemangioma: A report of two cases


Department of Skin and VD, Sriram Chandra Bhanj Medical College, Cuttack, Odisha, India

Date of Web Publication1-Sep-2014

Correspondence Address:
Basanti Devi
Department of Skin and Veneral Diseases, B 175, Pragyan Bihar, Baramunda, Bhubaneswar - 751 003, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.139918

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   Abstract 

We report two cases of epitheloid hemangioma presented with multiple nodular lesions over head and neck region. One of them gave history of bleeding on minor trauma. Pyogenic granuloma was considered as a differential diagnosis from the morphological appearance and history of bleeding. Nodular leprosy, sarcoidosis, and secondary syphilis were also considered. Histopathological examination of both was typical of epitheloid hemangioma, an entity commonly overlooked clinically due to its rarity.


Keywords: Epitheloid hemangioma, Kimura′s disease, pyogenic granuloma


How to cite this article:
Devi B, Jena S, Kar D, Patro S, Behera B. Epitheloid hemangioma: A report of two cases. Indian J Dermatol 2014;59:510-2

How to cite this URL:
Devi B, Jena S, Kar D, Patro S, Behera B. Epitheloid hemangioma: A report of two cases. Indian J Dermatol [serial online] 2014 [cited 2019 Jun 26];59:510-2. Available from: http://www.e-ijd.org/text.asp?2014/59/5/510/139918

What was known?
Epitheloid hemangioma is a benign vascular tumor with a characteristic histopathological appearance of numerous thin and thick walled vessels lined with characteristic edematous endothelial cells associated with variable eosinophilic and lymphocytic infiltrates.



   Introduction Top


Epitheloid hemangioma, otherwise known as ALHE, i.e., angiolymphoid hyperplasia with eosinophilia, is a rare benign vasoproliferative disease of unknown etiology. It was first described by Wells and Whimstar in 1961 [1] who considered it to be a late stage of Kimura's disease, but now both are considered to be separate entities. [2] The condition commonly presents in middle age adults as discrete or grouped papules, plaques, and nodules in the head and neck region [3] with slight female preponderance for periauricular lesions.

We report two cases of epitheloid hemangioma usually misdiagnosed clinically due to its rarity.


   Case Reports Top


Case 1

A 45-year-old man presented to us with 10-15 nodules on scalp with history of bleeding following minor trauma.Dermatological examination revealed multiple soft to firm erythematous, non-tender, non-pulsatile plaques, and nodules of size 0.5-1.5 cm arranged linearly on the scalp [Figure 1]. Few had hemorrhagic crust on surface. There was no regional lymphadenopathy. Systemic examination was within normal limits. Provisional diagnosis of pyogenic granuloma was made because of the morphology and history of bleeding. Other differential diagnoses considered were nodular lepromatous leprosy, nodular sarcoidosis, and secondary syphilis.Hemogram was within normal limits. Venereal Disease Research Laboratory (VDRL) test and Acid-Fast Bacilli (AFB) were negative. Chest X-ray revealed no abnormality.
Figure 1: Soft to firm nodules over scalp (Case 1)

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Case 2

A 35-year-old man presented to us with multiple asymptomatic papules and nodules over left auricle and adjacent area of scalp since 4 months. There was no history of pain, bleeding, or ulceration. Dermatological examination revealed multiple skin colored to slightly erythematous papules and nodules over the left auricle. The nodules were soft to firm, non-tender, non-pulsatile, free from underlying structures without any surface change. Only the largest had some superficial crust. There was no mucosal involvement or regional lymphadenopathy. Systemic examination was within normal limits. Differential diagnosis of nodular lepromatous leprosy, nodular sarcoidosis, and secondary syphilis were considered. Hemogram was normal except eosinophilia (11%), VDRL was non-reactive, and AFB was negative while chest X-ray was normal.

Histopathological examination was confirmatory in both cases which revealed circumscribed lobules with clusters of proliferating capillaries.Thick blood vessels lined by plump endothelial cells with perivascular infiltration of lymphocytes and eosinophils [Figure 2].
Figure 2: HP showing capillary proliferation with plump endothelial cells and eosinophilic infiltration (×40)

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Based on typical histopathological studies both cases were diagnosed as epitheloid hemangioma.

Larger lesions in both the cases were surgically excised. Radiofrequency ablation was done for smaller lesions and the base of larger lesions, following surgical excision. There has been no recurrence after a follow up of 3 months.


   Discussion Top


Epitheloid hemangioma, otherwise known as angiolymphoid hyperplasia with eosinophilia, is a rare benign vascular tumor with a characteristic histopathological appearance of proliferation of small to medium-sized blood vessels often showing a lobular architecture. Many of these vascular channels are lined by enlarged (epitheloid) endothelial cells with perivascular inflammatory cell infiltrate composed mainly of lymphocytes and eosinophils. Nodular areas of a lymphocytic infiltrate with rare follicle formation. [4] A rare variant of intravascular epithelioid hemangioma is described in which the whole of the lesion is intravascular. [5] Being relatively rare, it may be commonly misdiagnosed as nodular lepromatous leprosy, pyogenic granuloma, sarcoidosis, and secondary syphilis.Peripheral blood eosinophilia is not a constant finding, [6] and serum IgE is not routinely available. So, morphology and histopathology remains the mainstay of diagnosis.

ALHE is usually used synonymously with Kimura's disease due to common features of male predominance, predilection for head and neck and infiltration in dermis by lymphocytes and eosinophils [7] but Rosaiet al., suggested both to be distinct clinicopathological entity. [8] Kimura's disease lesions are subcutaneous nodules while ALHE superficial erythematous papule and nodule, which bleeds easily with minor trauma. Regional lymphadenopathy is present in Kimura's disease but absent in ALHE. Pathologically, lymphoid follicles with eosinophilic infiltration are more prominent in Kimura's disease while capillary proliferations with epitheloid changes are conspicuous in ALHE. Peripheral eosinophilia, raised IgE level are found in most cases of Kimura's disease but only in <10% cases in ALHE. [9],[10]

The pathogenesis of ALHE remains unclear. Some authors consider ALHE as a neoplasm developing from endothelial cells; others suggest that it is secondary to an inflammatory vascular reaction secondary to complex immunologic mechanisms. Other hypotheses implicating environmental factors such as insect bite, trauma, and infections have been reported. Some authors consider that arterio-venous shunt is the main etiopathogenetic mechanism observed in 42% of the cases. [10],[11] The predominance of T-lymphocytes and a rearrangement of T-Cell Receptor (TCR), in some cases, made some authors suppose that ALHE is a low-grade neoplastic disease secondary to various stimuli. [11],[12],[13]

Different treatment modalities have been attempted including cryosurgery, intralesional corticosteroids, lasers, intralesional interferon [9],[14] but upto a third of all treated cases do recur, due to its deeper vascular component. Complete surgical excision has been shown to lower recurrence rates. [15]

Epitheloid hemangioma is a rare affliction with a challenging diagnosis and treatment. In spite of the benignity of this disease, it causes a therapeutic dilemma because of the cosmetic defects and recurrence following treatment.

 
   References Top

1.Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 1969;81:1-14.  Back to cited text no. 1
    
2.Googe PB, Harris NL, Mihm MC Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Two distinct histopathological entities. J Cutan Pathol 1987;14:263-71.  Back to cited text no. 2
    
3.Grimwood R, Swineheart JM, Aelin JL. Angiolymphoid hyperplasia with eosinophilia. Arch Dermatol 1979;115:205-7.  Back to cited text no. 3
    
4.Calonje E. Vascular tumors: Tumors and tumor-like conditions of blood vessels and lymphatics. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, Xu X, editors. Lever's Histopathology of the Skin. 10 th ed. Philadelphia: Lippincott Williams and Wilkins; 2009. p. 1023-24.  Back to cited text no. 4
    
5.Rosai J, Ackerman LR.Intravenous atypical vascular proliferation. A cutaneous lesion simulating a malignant blood vessel tumor. ArchDermatol 1974;109:714.  Back to cited text no. 5
    
6.Zarrin-Khameh N, Spoden JE, Tran RM. Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: A case report and review of the literature. Arch Pathol Lab Med 2005;129:1168-71.  Back to cited text no. 6
    
7.Maheshwari V, Sharma R, Alam K, Khan A. Angiolymphoid hyperplasia with eosinophilia (alhe) versus Kimura's disease: Changing concepts. Indian J Dermatol Venereol Leprol 1999;65:186-8.  Back to cited text no. 7
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8.Rosai J, Gold J, Landy R. The histiocytoid hemangiomas: A unifying concept embracing several previously described entities of skin, soft tissue, large vessels, bone, and heart. Hum Pathol 1979;10:707-30.  Back to cited text no. 8
    
9.Khandpur S, Ramam M. Skin tumors. In: Valia RG, Valia A, editors. IADVL Textbook of Dermatology. 3 rd ed. Mumbai: Bhilani Publishing house; 2008. p. 1520-21.  Back to cited text no. 9
    
10.Calonje E. Soft-tissue tumours and tumour-like conditions. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8 th ed. Oxford: Wiley-Blackwell; 2010. p. 56.27-8.  Back to cited text no. 10
    
11.Demitsu T, Nagato H, Inoue T. Angiolymphoid hyperplasia with eosinophilia: Its character and therapy. Skin Surg 2000;9:8-16.  Back to cited text no. 11
    
12.Ramchandani PL, Sabesan T, Hussein K. Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura disease. Br J Oral Maxillofac Surg 2005;43:249-52.  Back to cited text no. 12
    
13.Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia: A clinicopathlogic study of 116 patients. J Am Acad Dermatol 1985;12:781-96.  Back to cited text no. 13
    
14.Rampini P, Semino M, Drago F, Rampini E. Angiolymphoid hyperplasia with eosinophilia: Successful treatment with interferon alpha 2b. Dermatology 2001;202:343.  Back to cited text no. 14
    
15.Calhoun KH, Newton RC, Sanchez RL. Angiolymphoid hyperplasia with eosinophilia. Arch Otolarygol Head Neck Surg 1998;114:1474-6.  Back to cited text no. 15
    

What is new?
Multiple nodular lesions in head and neck region especially periauricular helps in clinical diagnosis of this rare condition.


    Figures

  [Figure 1], [Figure 2]



 

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