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Cover page of the Journal of Health Sciences


 
 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 11  |  Issue : 1  |  Page : 97-100

Case of a nodular ear


Department of Dermatology, Dr. D. Y. Patil Medical College, Mumbai, Maharashtra, India

Date of Web Publication17-Jan-2018

Correspondence Address:
Dr. Palak Jayesh Sheth
Department of Dermatology, Dr. D. Y. Patil Medical College, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kleuhsj.kleuhsj_158_17

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  Abstract 


Angiolymphoid hyperplasia with eosinophilia is a benign condition which presents as vascular nodules or plaques, which are often multiple and grouped, located in the head and neck region, especially around the ears. It is associated with arteriovenous shunts in many cases, and they may be painful, pruritic, or pulsatile. On histology, it is characterized by proliferation of blood vessels with epithelioid cells surrounding the larger vessels and accompanied by eosinophils. Various treatment modalities have been described. Here, we present the case of a 30-year-old female with angiomatous nodules over the left ear,which were successfully treated.

Keywords: Angiolymphoid hyperplasia with eosinophilia, Kimura's disease, nodular ear


How to cite this article:
Apurwa A, Sheth PJ, Nadkarni N, Patil S. Case of a nodular ear. Indian J Health Sci Biomed Res 2018;11:97-100

How to cite this URL:
Apurwa A, Sheth PJ, Nadkarni N, Patil S. Case of a nodular ear. Indian J Health Sci Biomed Res [serial online] 2018 [cited 2019 Aug 24];11:97-100. Available from: http://www.ijournalhs.org/text.asp?2018/11/1/97/223422




  Introduction Top


Angiolymphoid hyperplasia with eosinophilia (ALHE) (papular angioplasia, inflammatory angiomatous nodule, atypical granuloma, inflammatory arteriovenous hemangioma, pseudo-pyogenic granuloma, and histiocytoid hemangioma) is an uncommon, benign, reactive vasoproliferative disease, presenting with painless, vascular nodules in the dermal and subcutaneous tissues of the head and neck, particularly around the ear.[1] Other sites include the hands, shoulders, breast, penis, oral cavity, orbit, eyelids, and lacrimal glands.[2] The lesions may be asymptomatic, painful, or pruritic. Lymphadenopathy and peripheral eosinophilia may be present.[3]

It commonly affects women between 20 and 40 years old.[4] Underlying vascular malformation and local trauma are suggested as possible causes. ALHE is very often mistaken for Kimura's disease but these two are now identified as two separate entities.


  Case Report Top


A 38-year-old female attended the outpatient department with complaint of multiple gradually increasing painless swellings over the left ear for 3 years' duration [Figure 1].
Figure 1: Multiple well-defined nodules present on the pinna of the left ear

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The patient gives a history of itching over the lesion on and off, leading to discrete ulcerations and bleeding due to scratching. She denied constitutional symptoms and local trauma.

There was no history of similar lesions elsewhere on the body or similar complaints in the past.

There was no history of diabetes mellitus, thyroid illness, hypertension, tuberculosis, bronchial asthma, respiratory infection, and menstrual irregularities.

On cutaneous examination, multiple grouped erythematous papular-nodular lesions were observed, with diameter ranging from 0.3 to 0.8 cm located on the pinna of the left ear. They were soft to firm in consistency, slightly tender, noncompressible, and immobile. There was no associated regional lymphadenopathy. Systemic examination revealed no abnormality.

Complete blood count and peripheral smear show eosinophilia, while urine routine and microscopy were within normal limits.

A differential diagnosis of Kimura's disease, ALHE, pyogenic granuloma, pseudolymphoma, and sarcoidosis was considered.

A biopsy was done to confirm the diagnosis which showed epidermal atrophy with effacement of rete ridges. Areas of sparing were present below the epidermis with nodular aggregates of lymphocytes and eosinophils around the blood vessels. Multiple smaller blood vessels were present in the upper and mid dermis [Figure 2], [Figure 3], [Figure 4].
Figure 2: Multiple dilated blood vessels seen in the dermis along with inflammatory infiltrate on H and E, slide (×4)

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Figure 3: Dilated blood vessels and neutrophils and few eosinophilic infiltrates present in the upper dermis on an H and E, slide (×10)

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Figure 4: Lymphocytic infiltrate along with few eosinophils and dilated blood vessels present in the upper dermis on H and E, slide (×40)

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Hence, a diagnosis of ALHE was made.

The patient was given intralesional triamcinolone acetonide 40 mg/ml, thrice each at an interval of 3 weeks. The patient had a dramatic response with complete resolution at the end of 9 weeks [Figure 5].
Figure 5: Posttreatment image of the patient with resolution of the lesions

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  Discussion Top


ALHE is a rare condition involving the muscular arteries, most commonly occurring in the head and neck region. It was first described in 1969 by Wells and Whimster.[5] Initially, it had been described as pseudo or atypical pyogenic granuloma, subcutaneous angioblastic lymphoid hyperplasia with eosinophilia, and papular angioplasia. Due to several similarities, it was thought to be related to Kimura's disease. However, recent studies indicate that Kimura's disease differs from ALHE in several clinical and histopathologic characteristics.

[Table 1] shows the differences between ALHE and Kimura's disease.[6]
Table 1: Differences between Kimura's disease and angiolymphoid hyperplasia with eosinophilia

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It usually occurs in young- to middle-aged adults, with a female preponderance. It is more common in Japan than other countries and less commonly seen in African americans. It is rarely seen in adults.[3]

The pathogenesis of ALHE is unclear. It is considered to be due to or in association with an underlying vascular pathology, as a reactive phenomenon. It can be associated with a history of trauma, insect bite, and hyperestrogenemias such as pregnancy, and oral contraceptive pill intake is also noticed in some cases.[7]

The predominance of T lymphocytes and a rearrangement of T-cell receptor in some cases made some authors suppose that ALHE is a low-grade neoplastic disease secondary to various stimuli.[8]

Diagnosis is made upon histological findings. Serum hypereosinophilia is inconstant seen only in 21% of the affected individuals.

Histologically, the lesions show proliferation of small blood vessels with peripheral inflammatory infiltrates mainly composed of mononuclear cells and eosinophils.

The blood vessels are lined predominantly by endothelial cells which have a histiocytoid or epithelioid appearance.

Immunohistochemical stains often show large number of T lymphocytes,[3] with occasional B cells forming the lymphoid follicles.[9] Radiological investigations such as magnetic resonance imaging or angiography may be done to confirm the diagnosis.

The lesions may either increase progressively or decrease spontaneously if treatment is not administered. Surgical treatment with Mohs' surgery is effective. Recurrences, essentially after incomplete excision, are observed in 30% of the cases.[8] No metastatic cases have been reported. Several therapeutic procedures which are done with good results include cryotherapy, electrode section, intralesional injection of corticosteroids, systemic corticosteroids, topical tacrolimus, interferon alpha-2b therapy, sclerotherapy, intralesional bleomycin, radiofrequency excision, radiotherapy, and lasers.

Imiquimod, which inhibits the production of interleukin-5 (IL-5), and mepolizumab, which inhibits the reaction of IL5 with its receptor, were reported to be effective.[10]

This case being reported as ALHE is a rare condition, which has a challenging diagnosis. Although the disease is of a benign nature, it can present as a therapeutic dilemma due to the cosmetic defects and often resistance to treatment.

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 Top

1.
Botet MV, Sánchez JL. Angiolymphoid hyperplasia with eosinophilia: Report of a case and a review of the literature. J Dermatol Surg Oncol 1978;4:931-6.  Back to cited text no. 1
    
2.
Taylor SK, Meyerle JH, Glusac EJ. Angiolymphoid hyperplasia with eosinophilia (eMedicine Website). Available from: http://emedicine.medscape.com/article/1082603-overview 2008. [Last accessed on 2016 Jul 14].  Back to cited text no. 2
    
3.
Kempf W1, Haeffner AC, Zepter K, Sander CA, Flaig MJ, Mueller B, et al. Angiolymphoid hyperplasia with eosinophilia: Evidence for a T-cell lymphoproliferative origin. Hum Pathol 2002;33:1023-9.  Back to cited text no. 3
    
4.
Moran CA, Suster S. Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the lung: A clinicopathologic and immunohistochemical study of two cases. Am J Clin Pathol 2005;123:762-5.   Back to cited text no. 4
[PUBMED]    
5.
5. Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 1969;81:1-14.  Back to cited text no. 5
    
6.
Reddy PS, Prasad AS, Sumathy TK, Shivaswamy KN, Ranganathan C. An overlap of angiolymphoid hyperplasia with eosinophilia and Kimura's disease: Successful treatment of skin lesions with cryotherapy. Indian J Dermatol 2015;60:216.  Back to cited text no. 6
    
7.
Leiferman KM, Peters MS, Eosinophils in cutaneous disease. Fitzpatricks dermatology in general medicine 7th edition New York : Mc Gray Hill 2008 p 307-17.  Back to cited text no. 7
    
8.
Ramchandani PL. Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura diseases Br J Oral Maxillofac Surg. 2005. Br J Oral Maxillofac Surg. 2005;43:249-52.  Back to cited text no. 8
    
9.
Olsen TG. J Am Acad Dermatol. Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 116 patients. J Am Acad Dermatol. 1985;12:781-96.  Back to cited text no. 9
    
10.
Braun-Falco M. Br J Dermatol. 2004. Angiolymphoid hyperplasia with eosinophilia treated with anti- interleukin-5 antibody (mepolizumab) Br J Dermatol. 2004;151:1103-4.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1]



 

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