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CASE REPORT
Year : 2016  |  Volume : 17  |  Issue : 3  |  Page : 196-198

Angiolymphoid hyperplasia with eosinophilia of oral mucosa in a child treated with imiquimod


Department of Dermatology, Government Stanley Medical College and Hospital, Chennai, Tamil Nadu, India

Date of Web Publication5-Jul-2016

Correspondence Address:
Rashmi Singh
Department of Dermatology, Government Stanley Medical College and Hospital, Royapuram, Chennai - 600 001, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.179640

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  Abstract 

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare and idiopathic disorder of blood vessels which is common in head and neck area of middle-aged women. We report a case of angiolymphoid hyperplasia of lower lip in a 7-year-old child, confirmed by histopathological findings of hyperplastic lymphoid follicles, eosinophilia, and proliferation of vessels and positive staining with CD3 and CD20 in immunohistochemistry. Lesion was treated with imiquimod for 16 weeks following which it resolved completely. We present this case for the rarity of the case in this age group on an atypical rare site. To the best of our knowledge, this is the first reported case of ALHE on oral mucosa of a male child.

Keywords: Angiolymphoid hyperplasia, mucous membrane, nodule


How to cite this article:
Venkatesan A, Singh R. Angiolymphoid hyperplasia with eosinophilia of oral mucosa in a child treated with imiquimod. Indian J Paediatr Dermatol 2016;17:196-8

How to cite this URL:
Venkatesan A, Singh R. Angiolymphoid hyperplasia with eosinophilia of oral mucosa in a child treated with imiquimod. Indian J Paediatr Dermatol [serial online] 2016 [cited 2019 Aug 23];17:196-8. Available from: http://www.ijpd.in/text.asp?2016/17/3/196/179640


  Introduction Top


Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare and idiopathic vascular disorder. It is characterized by red to brown papules or nodules dislocated in the dermis or subcutaneous tissue. These lesions are typically localized on the head and neck, particularly around the ear as singular or multiple nodules.[1] Lesions in the oral mucosa are extremely rare. It occurs predominantly in Caucasian women during the third and fourth decades, and it is very rare in children.[2]


  Case Report Top


A 7-year-old, otherwise fit boy, born out of a nonconsanguineous marriage, presented with a solitary, hard, nodule on the left side of his lower lip since 2 months. History of lip biting was present.

On physical examination, the lesion in the left lower lip was fleshy, nodular, firm, nontender, dome-shaped, and it measured 7 mm × 8 mm in diameter [Figure 1]. Lesion was bleeding on manipulation. There was no regional lymphadenopathy. The case, which was previously treated by a pediatrician with topical antibiotics without any improvement, was clinically diagnosed by us as granuloma pyogenicum. Subsequent to that attempts of treatment were done by topical timolol, and then sclerosant (sodium dodecyl sulfate) with not so satisfactory results. A biopsy was taken by a pediatric surgeon, and the sections of the biopsy specimen were stained with hematoxylin and eosin, which revealed lymphoid follicular hyperplasia, mixed infiltrate with prominent eosinophilia, and hyalinized blood vessels with prominent endothelial cells [Figure 2]. No mitoses or atypical cells were observed. Immunohistochemistry showed positive staining with CD3 and CD20, and low positive for Ki67 with few cells staining for CD34. A diagnosis of ALHE was made based on the above evidence.
Figure 1: (a) Firm, nontender, dome-shaped, and it measured 7 mm × 8 mm in diameter nodule on left lower lip. (b) Complete resolution after 16 weeks of imiquimod

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Figure 2: (a) Histopathology with H and E stain showing hyperplasia of lymphoid follicles. (b) Histopathology with H and E stain showing eosinophilia and hyalinized blood vessels of small caliber

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The patient was then put on treatment with topical 5% imiquimod, 5 days a week with 2 days gap to promote healing of erosions. The lesion regressed completely, and the patient was released from treatment after 16 weeks. No recurrence has been reported after 1 year of follow-up.


  Discussion Top


First described in 1969 by Wells and Whimster, ALHE is a rare and idiopathic vascular disorder.[3] The main contention remains controversial whether it is a neoplastic or an atopic hypersensitivity reaction (unusual reactive process). Several reports also indicate the possibility that ALHE may be secondary to infection or trauma.[4] In our case, lip biting appears to be the precipitating cause resulting in inflammation and the development of lesion.

ALHE is seen most commonly in Asians, followed by whites and less common in blacks. ALHE is slightly more prevalent in females though a male predominance has been noted in selected Asian studies. It presents most commonly in patients aged 20–50 years, with a mean onset of 30–33 years.[5]

Clinically, ALHE presents as single or multiple pink to reddish-brown papules or subcutaneous nodules that are usually located on the head and neck (85%), and especially in the preauricular region.[2] Lesions in the oral mucosa are extremely rare, and when it occurs, it most commonly affects young male adults.[6] To the best of our knowledge, there has been no reported case of ALHE of oral mucosa in children.

ALHE shows characteristic histologic features including a proliferation of small blood vessels, many of which are lined by enlarged endothelial cells with uniform ovoid nuclei and intracytoplasmic vacuoles. These distinctive endothelial cells have been described as having a cobblestone appearance. In addition, a perivascular and interstitial infiltrate composed primarily of lymphocytes and eosinophils is present. Eosinophils typically comprise 5–15% of an infiltrate. Rarely, they can account for as much as 50% of an infiltrate. Occasionally, an infiltrate is devoid of eosinophils. Lymphoid aggregates with or without follicle formation are typical. Immunohistochemistry of ALHE is positive for CD31, CD34, and factor VII markers suggesting the origin of cell to be either fibrohistiocytic or endothelial.

The most common therapeutic options are surgical excision and pulsed dye laser. Other options include radiotherapy, curettage, shave excision with electrodessication, cryotherapy, corticosteroids (topical, systemic, or intralesional preparation), and laser therapy. Continuous wave carbon dioxide and argon lasers have been successfully used, but there is a risk of scarring after treatment. Anecdotal reports have described other successful therapeutic options: Intralesional interferon alpha-2a, indometacin farnesil, pentoxifylline, chemotherapeutic agents such as vinblastine, mepolizumab (anti-interleukine-5), and imiquimod. Furthermore, oral retinoids have been employed with different results.[1]

In this case, we used imiquimod 5% which showed a successful resolution of the lesion without relapse in the follow-up period. Imiquimod is a toll-like receptor-7 agonist enhancing innate and acquired immune response. It also activates TH-1 cells which provide a long-term memory of immune response to viral/tumor cells. It also promotes expression of apoptotic receptors which causes tumor regression.


  Conclusion Top


To the best of our knowledge, this is the first reported case of angiolymphoid hyperplasia on the oral mucosa of a male child.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Lembo S, Balato A, Cirillo T, Balato N. A long-term follow-up of angiolymphoid hyperplasia with eosinophilia treated by corticosteroids: When a traditional therapy is still up-to-date. Case Rep Dermatol 2011;3:64-7.  Back to cited text no. 1
    
2.
Jeon EK, Cho AY, Kim MY, Lee Y, Seo YJ, Park JK, et al. Angiolymphoid hyperplasia with eosinophilia that was possibly induced by vaccination in a child. Ann Dermatol 2009;21:71-4.  Back to cited text no. 2
    
3.
Ahmad SM, Wani GM, Khursheed B, Qayoom S. Angiolymphoid hyperplasia with eosinophilia mimicking multiple cylindromas: A rare case report. Indian J Dermatol 2014;59:423.  Back to cited text no. 3
    
4.
Al-Muharraqi MA, Faqi MK, Uddin F, Ladak K, Darwish A. Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the face: An unusual presentation. Int J Surg Case Rep 2011;2:258-60.  Back to cited text no. 4
    
5.
Shafer, Hine, Levy. A Textbook of Oral Pathology. 5th ed. India: Elsevier; 2006. p. 33-4.  Back to cited text no. 5
    
6.
Aggarwal A, Keluskar V. Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in the oral mucosa. Indian J Dent Res 2012;23:271-4.  Back to cited text no. 6
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