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E-CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 4  |  Page : 423
Angiolymphoid hyperplasia with eosinophilia mimicking multiple cylindromas: a rare case report


1 Department of Dermatology, Venereology and Leprosy, Sheri-Kashmir Institute of Medical Sciences, Medical College Hospital, Srinagar, Kashmir, India
2 Department of Dermatology, Venereology and Leprosy, Sheri Kashmir Institute of Medical Sciences, Medical College Hospital, Srinagar, Kashmir, India

Date of Web Publication27-Jun-2014

Correspondence Address:
Dr. Gh Mohiuddin Wani
Department of Dermatology, Venereology and Leprosy, SKIMS, Srinagar - 190 017, Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.135542

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   Abstract 

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare and idiopathic vascular disorder. It is characterized by red to brown papules and nodules, typically localized on the head and neck, particularly around the ear as singular or multiple lesions. Although ALHE is a benign disease, lesions are often persistent and difficult to eradicate. Young to middle age women are more commonly affected. The histological examination corresponds to a florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate. We describe an elderly male with multiple nodular lesions over the scalp mimicking cylindromas; the histological examination was consistent with ALHE.


Keywords: Angiolymphoid hyperplasia, eosinophilia, epitheloid hemangioma, scalp


How to cite this article:
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

How to cite this URL:
Ahmad SM, Wani GM, Khursheed B, Qayoom S. Angiolymphoid hyperplasia with eosinophilia mimicking multiple cylindromas: a rare case report. Indian J Dermatol [serial online] 2014 [cited 2019 Jun 18];59:423. Available from: http://www.e-ijd.org/text.asp?2014/59/4/423/135542

What was known?br> Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign disease and the scalp might be involved as well.



   Introduction Top


Angiolymphoid hyperplasia with eosinophilia (ALHE) or Epitheloid hemangioma is an uncommon vascular disorder. Inflammatory processes such as allergic reactions, trauma or autoimmune disorders are identified as possible causes. ALHE can occur in all races, but it is reported more frequently in Asians. Young and middle-aged women are more commonly affected. Typically, patients present with smooth surfaced red to brown papules and nodules on the head and neck; most nodules are localized near the ear. Other less common sites include the trunk, extremities, genitalia, lips and oral mucosa; extra cutaneous involvement is rare. [1],[2]

The main differential diagnosis is Kimura disease, [3] but other disorders such as facial granuloma, insect bite reaction, pyogenic granuloma, cutaneous lymphoma or sarcoidosis may imitate the clinical picture of ALHE. [2]


   Case Report Top


A 75-year-old, turban wearing man presented to our outpatient clinic with a 6-month history of multiple, enlarging and itchy nodules over the scalp. His medical history was unremarkable except hypertension controlled with anti-hypertensive medication (amlodipine 5mg/day). A general physical examination was normal and our first skin examination revealed multiple, skin colored to dull red, dome shaped nodules localized on the scalp, more on the right posterior-lateral aspect. The lesions were discrete, 5-30 mm in diameter and most of them had a smooth hairless surface [Figure 1]. The lesions were firm in consistency, mobile from underlying structures, non-tender and slightly blanching. The regional lymph-nodes were not enlarged. The rest of the skin, mucous membranes and nails were normal.
Figure 1: Angiolymphoid hyperplasia with eosinophilia showing multiple nodular lesions over the scalp

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His routine laboratory investigations, including a complete blood cell count, erythrocyte sedimentation rate, urine analysis and blood chemistry, demonstrated no abnormalities. The total eosinophil count was within normal range. His chest radiography and ultrasonography were also normal.

Histopathological examination of one of the nodular skin lesion showed marked dermal vascular proliferation, lined with plump endothelial cells and surrounded by numerous eosinophils and lymphocytes [Figure 2] and [Figure 3]. No atypical mitotic figures were observed. The histopathologic findings were consistent with ALHE.
Figure 2: Histopathology of a nodular lesion, showing proliferation of vascular channels and dense, chronic, inflammatory infiltrate rich in eosinophils in dermis (H and E, x40)

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Figure 3: Proliferated vascular channels lined by plump endothelial cells and infiltration by eosinophils, lymphocytes and histiocytes better appreciated at higher magnification (H and E, x100)

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The patient was treated with intralesional corticosteroid injections: 2 ml of triamcinolone acetonide (20 mg/ml) once a month. After the third month, the lesions showed visible reduction in size. The patient is being followed-up. He is on no other additional topical treatment.


   Discussion Top


Angiolymphoid hyperplasia with eosinophilia, first described by Wells and Whimster in 1969, is a rare benign vascular tumor. [4] It is characterized by one or more purplish, brownish papules and nodules with a predilection for head and neck region. Other tissues such as the orbit, heart, bone, liver and spleen may also be involved. It is more common in middle aged females. Contrary to what the name suggests, peripheral blood eosinophilia is not a constant finding in ALHE. [5] Histopathologically, ALHE is characterized by numerous thick and thin walled vessels lined with characteristic edematous endothelial cells associated with variable lymphocytic and eosinophilic infiltrate. [6] Our patient had characteristic histological findings of ALHE like epitheloid endothelial cells and prominent eosinophilic infiltrate, differentiating it from other conditions like cylindroma, angio-sarcoma and cutaneous lymphoma.

The etiopathogenesis of ALHE is not well known. Trauma, hormonal changes and infections (Human T-cell lymphotropic virus (HTLV) or Human Herpesvirus 8 (HHV-8)) have been suggested to play a role in the pathogenesis. [7] Associations of ALHE with nephrotic syndrome and pregnancy have been described. [7],[8] Over-expression of estrogen and progesterone receptors was detected in pregnant women. [9] Interleukin 5 and vascular endothelial growth factors were also found to be increased in some cases. [10]

Since they share many clinical and histopathological similarities, ALHE should be differentiated from Kimura's disease. Kimura's disease occurs in younger patients, the lesions show deeper localization and association with lymphadenopathy. Histologically, it contains sclerosis at any stage, but does not have epitheloid endothelial cells. [11]

The lesions in our case clinically suggested multiple cylindromas from the predilection of lesions for the scalp, the multiplicity of lesions and their gross appearance as hemispherical, hairless, smooth surfaced tumors. No other family member was affected and the histopathology was, of course, distinguishing.

The most common therapeutic options are surgical excision and pulsed dye laser. Cryotherapy, irradiation, steroid injection, carbon dioxide laser have also been reported as therapeutic options with variable levels of success. [12] We preferred intralesional corticosteroid injections in our patient. Surgery would not have been feasible as he had numerous lesions involving almost the whole scalp. Treatment with intralesional corticosteroids is a traditional therapeutic approach that still offers the advantage of being simple to perform and is much less invasive than a surgical approach. It achieves similar results in terms of recurrence and has an even better aesthetic outcome. [13]

We report and emphasize this case considering the unusually high number of lesions (more than 60) and their resemblance to multiple cylindromas of the scalp.

The older, male patient of ALHE presented here had an atypical appearance. He had numerous lesions involving large area of the scalp, which has not been reported in literature. Pre-auricular area and ears were spared. We suggest that ALHE should be considered in the differential diagnosis of multiple cylindromas of the scalp.

 
   References Top

1.Endo M, Shibata M, Iwamoto I, Suzuki H, Hayakawa M. Angiolymphoid hyperplasia with eosinophilia. Eur J Dermatol 1998;8:175-7.  Back to cited text no. 1
    
2.El Sayed F, Dhaybi R, Ammoury A, Chababi M. Angiolymphoid hyperplasia with eosinophilia: Efficacy of isotretinoin? Head Face Med 2006;2:32-6.  Back to cited text no. 2
    
3.Chun SI, Ji HG. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Clinical and histopathologic differences. J Am Acad Dermatol 1992;27:954-8.  Back to cited text no. 3
    
4.Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 1969;81:1-14.  Back to cited text no. 4
    
5.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. 5
    
6.Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia: A clinicopathologic study of 116 patients. J Am Acad Dermatol 1985;12:781-96.  Back to cited text no. 6
    
7.Hollo P, Marschalko M, Sikos G, Harsing J, Horvath A. Angiolymphoid hyperplasia with eosinophilia in pregnancy. J Eur Acad Dermatol Venereol 2005;19:645-6.  Back to cited text no. 7
    
8.Azizzadeh M, Namazi MR, Dastghaib L, Sari-Aslani F. Angiolymphoid hyperplasia with eosinophilia and nephrotic syndrome. Int J Dermatol 2005;44:242-4.  Back to cited text no. 8
    
9.Moy RL, Luftman DB, Nguyen QH, Amenta JS. Estrogen receptors and the response to sex hormones in angiolymphoid hyperplasia with eosinophilia. Arch Dermatol 1992;128:825-8.  Back to cited text no. 9
    
10.Aoki M, Kimura Y, Kusunoki T, Tahara S, Kawanah S. Angiolymphoid hyperplasia with eosinophilia associated with anomalous dilatation of occipital artery: IL-5 and VEGF expression of lesional mast cells. Arch Dermatol 2002;138:982-4.  Back to cited text no. 10
    
11.Calonje E, MacKie RM. Soft tissue tumors and tumor like conditions. In: Breathnach S, Cox N, Griffiths C, editors. Rook's Text book of dermatology. 7 th ed. Oxford: Blackwell Publishing; 2004. p. 53.20.   Back to cited text no. 11
    
12.Karabudak O, Taskapan O, Bozdogan O, Dogan B. Angiolymphoid hyperplasia with eosinophilia: Atypical appearance in an older patient. Indian J Dermatol 2008;53:144-5.  Back to cited text no. 12
[PUBMED]  Medknow Journal  
13.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. 13
    

What is new?
This is the first case report showing the involvement of scalp with numerous lesions of angiolymphoid hyperplasia with eosinophilia (ALHE) and their resemblance to multiple cylindromas of the scalp.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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