Angiolymphoid hyperplasia with eosinophilia: A report of a rare entity in an elderly male

Anushruti Aggarwal; Sharang Gupta; Dimple Chopra; Raina Arora

doi:10.4103/jiag.jiag_3_23

CASE REPORT

Year : 2023 | Volume : 19 | Issue : 1 | Page : 76-78

Angiolymphoid hyperplasia with eosinophilia: A report of a rare entity in an elderly male

Anushruti Aggarwal, Sharang Gupta, Dimple Chopra, Raina Arora
Department of Dermatology, Government Medical College, Patiala, Punjab, India

Date of Submission	14-Jan-2023
Date of Decision	30-Jan-2023
Date of Acceptance	21-Feb-2023
Date of Web Publication	17-Mar-2023

Correspondence Address:
Sharang Gupta
Department of Dermatology, Government Medical College, Patiala, Punjab
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiag.jiag_3_23

Abstract

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign proliferation of blood vessels of uncertain etiology. It primarily affects the head-and-neck region. Histologically, it is characterized by the prominent proliferation of plump endothelial cells, and accompanying eosinophilic and lymphocytic infiltration. Herein, we report the case of ALHE in a 65-year-old male.

Keywords: Angiolymphoid hyperplasia with eosinophilia, epithelioid hemangioma, histiocytoid hemangioma

How to cite this article:
Aggarwal A, Gupta S, Chopra D, Arora R. Angiolymphoid hyperplasia with eosinophilia: A report of a rare entity in an elderly male. J Indian Acad Geriatr 2023;19:76-8

How to cite this URL:
Aggarwal A, Gupta S, Chopra D, Arora R. Angiolymphoid hyperplasia with eosinophilia: A report of a rare entity in an elderly male. J Indian Acad Geriatr [serial online] 2023 [cited 2023 Mar 22];19:76-8. Available from: http://www.jiag.com/text.asp?2023/19/1/76/371902

Introduction

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasoproliferative disorder that presents with solitary or multiple, pink to red-brown, dome-shaped papules or nodules, most frequently located in the head-and-neck region. The term was coined by Wells and Whimster in 1969.^[1] It is known by various other names such as epithelioid hemangioma, histiocytoid hemangioma, and inflammatory angiomatous nodule.^[2] Peripheral eosinophilia occurs in up to 20% of patients with ALHE. ALHE has been confused with Kimura disease (KD) for a long. In recent years, researchers have tried to differentiate between these two entities. Elevated serum immunoglobulin E (IgE) levels are not a feature of ALHE but are more commonly present in KD.

Description of the Case

A 65-year-old male presented with a history of an erythematous nodular lesion on the right ear lobe for 7 years. It was a single firm nodule on the ear lobule which slowly increased in size over the years [Figure 1]. The patient did not give any history of trauma before the onset of the lesion. The patient denied any history of bleeding from the lesion. There was no history of any difficulty in hearing or any other ear complaints. No regional lymphadenopathy was present. Systemic examination revealed no abnormalities.

Figure 1: Erythematous firm nodule involving the right earlobe

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The routine laboratory investigations including complete hemogram and renal and liver function tests were within normal limits. HIV serology was nonreactive. The serum IgE levels were within normal limits.

Skin biopsy showed mild spongiosis in the dermis with an infiltrate of lymphocytes, plasma cells, and macrophages with a marked increase in eosinophils. Adnexal structures were surrounded by dense inflammatory infiltrate with many dilated and congested blood vessels. Proliferation of blood vessels was present and blood vessels were lined by plump endothelial cells [Figure 2] and [Figure 3].

Figure 2: Epidermis showing acanthosis and spongiosis

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Figure 3: Dermis showing moderate inflammatory infiltrate comprising lymphocytes, plasma cells, and macrophages with a marked increase in eosinophils. Adnexal structures are surrounded by dense inflammatory infiltrates with many dilated and congested blood vessels

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Differential diagnoses included KD, pseudolymphoma, epithelioid hemangioendothelioma, and epithelioid angiosarcoma.

Discussion

ALHE is a rare and benign vascular tumor of uncertain etiology. It is commonly seen in young to middle-aged adults. Lesions are solitary, few, or multiple, sometimes grouped, erythematous, violaceous, or brown papules, plaques, or nodules of the dermis or subcutaneous tissues.^[3] The head and neck are the most common site involved, although other sites such as the trunk, extremities, penis, colon, and conjunctiva can also be affected in certain cases.^{[4],[5],[6],[7],[8]}

ALHE shows slight female preponderance. It usually presents in the third to fifth decade of life. The pathogenesis of ALHE, as mentioned before, is unknown. Various hypotheses have been put forward, including a reactive process,^[9] neoplastic process,^[10],[11] and infectious mechanisms, however, none of these have been established. It has been considered a vascular proliferation arising in response to or in association with underlying vascular malformation. The lesions may pulsate, and/or maybe pruritic or painful.

ALHE tends to be chronic, lasting over months to years. The dominant histological feature is prominent vascular proliferation with large epithelioid or histiocytoid endothelial cells that contain abundant eosinophilic cytoplasm, often with cytoplasmic vacuoles in the dermis and/or subcutis. There are variable numbers of eosinophils and lymphocytes.

Differentiation from KD is essential [Table 1]. Lesions of ALHE generally are smaller, more superficial, and more numerous than those of KD, and often are symptomatic. Although KD may exhibit some vascularity, it lacks the large epithelioid endothelial cells that are a key feature of ALHE.

Table 1: Salient differences between angiolymphoid hyperplasia with eosinophilia and Kimura disease

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The choice of treatment is dictated in part by the number, location, size of lesions, and the patient's general health. Patients with solitary or a few small lesions may benefit from excision or Mohs surgery, but recurrence at the surgical site remains a problem.^[12] A variety of other treatment modalities have been used with success, including systemic and intralesional glucocorticoid administration, Interferon (INF)-α therapy, cryotherapy, laser therapy, and topical application of tacrolimus.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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