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Year : 2016  |  Volume : 17  |  Issue : 4  |  Page : 287-289

Angiolymphoid hyperplasia with eosinophilia: Report of two cases in children

Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India

Date of Web Publication7-Oct-2016

Correspondence Address:
Anupam Das
“Prerana” 19, Phoolbagan, Kolkata - 700 086, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.179481

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Angiolymphoid hyperplasia with eosinophilia (ALHE) is apparently benign, locally proliferating lesion composed of vascular channels with surrounding infiltrate of lymphocytes and eosinophils. We report two cases with long-standing papulonodular lesions on the left ear who presented to us on account of trauma-induced bleeding and cosmetic issues. Peripheral blood eosinophilia was present, and histological findings were consistent with a diagnosis of ALHE. The rarity of this disease in the pediatric age group prompted the present report.

Keywords: Angiolymphoid hyperplasia, children, peripheral eosinophilia

How to cite this article:
Das A, Ghosh A, Gharami RC. Angiolymphoid hyperplasia with eosinophilia: Report of two cases in children. Indian J Paediatr Dermatol 2016;17:287-9

How to cite this URL:
Das A, Ghosh A, Gharami RC. Angiolymphoid hyperplasia with eosinophilia: Report of two cases in children. Indian J Paediatr Dermatol [serial online] 2016 [cited 2021 Nov 28];17:287-9. Available from: https://www.ijpd.in/text.asp?2016/17/4/287/179481

  Introduction Top

Long back, there used to be a difficulty with the nomenclature of angiolymphoid hyperplasia with eosinophilia (ALHE). The term“Kimura's disease” had been applied, but this condition is now viewed as distinct from ALHE. In Kimura's disease, the lesions are deeper-seated, associated with lymphadenopathy, without any initial overlying skin lesions and do not contain epithelioid endothelial cells. A classical case of ALHE is characterized by diametrically opposite features. Peripheral blood eosinophilia is much more common in Kimura's disease. Exceptionally, ALHE may coexist with Kimura's disease.[1] We hereby report two children with ALHE.

  Case Reports Top

Case 1

A 14-year-old girl presented with multiple elevated lesions on the left ear, present over the preceding 5 years. The patient did not give any history of hearing problem or discharge from the ear. She complained of easy bleeding on trivial trauma and the parents stressed on the cosmetic aspect. On examination, there were multiple firm nontender and noncompressible nodules of 1.0–2.5 cm diameter on the left ear, with the involvement of external auditory canal [Figure 1]. They were fixed to the underlying structures as well as the overlying skin. Few nodules were surmounted by a hemorrhagic crust. There was no local rise of temperature. Regional lymphadenopathy was absent, and examination of the salivary glands did not reveal any abnormality. The examination of other organ systems, including chest, eye and ear, nose, and throat evaluation, was found to be normal.
Figure 1: Multiple firm papulo-nodules of 1.0–2.5 cm diameter on the left ear (Case 1)

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Case 2

A 10-year-old girl presented with multiple papulonodular lesions on the left ear that bled to touch. It started spontaneously as a single firm nodule on the concha of the left ear, which slowly increased in size and number. On examination, there were multiple nodules on her left ear (pinna), most of which had coalesced to form plaques. The lesions had a slight erythematous to violaceous hue. A clot was also visible over the lesion, suggestive of recent bleeding [Figure 2]. Regional lymphadenopathy was absent, and examination of other organ systems was found to be normal.
Figure 2: Multiple papules and nodules on the left ear coalescing to form plaques. Note the erythematous to violaceous hue. A clot is also visible (Case 2)

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For both the cases, the clinical differentials were Kimura's disease, cutaneous lymphoid hyperplasia, lymphoma cutis, arteriovenous malformations, sarcoidosis, etc. Routine laboratory investigations including a complete hemogram, hepatic profile, renal profile were done. Eosinophilia of 18% was notable while other parameters were within normal limits. The serology for antibodies against human immunodeficiency virus was negative. Fungal culture did not show any growth. Histopathological examination of the excised nodule showed angioproliferative cells with prominent fibrosis, lymphocytic infiltrates, and extravasation. There was no nuclear atypia, hyperchromasia, or increased mitotic activity [Figure 3] and [Figure 4]. This finding was substantial enough to label our case as ALHE.
Figure 3: Photomicrograph showing abundant proliferation of blood vessels lined by plump endothelial cells. Note the infiltrate of lymphocytes and eosinophils (H and E, ×10)

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Figure 4: Photomicrograph showing abundant proliferation of blood vessels lined by plump endothelial cells. Note the infiltrate of lymphocytes and eosinophils (H and E, ×100)

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  Discussion Top

ALHE was first described in 1969 by Wells and Whimster. They considered ALHE to be a late stage of Kimura's disease, described in the Japanese literature in 1948. It is now generally accepted that these are two separate entities. It occurs in the third to fourth decade of life, with a female preponderance. A history of trauma can be elicited in some cases. ALHE has been given numerous names in the literature and has been recently classified under cutaneous vascular hyperplasias, along with pyogenic granuloma and bacillary angiomatosis.[2] It is an uncommon benign but potentially disfiguring vascular proliferation. It has a particular predilection for head and neck area, especially for the ears. The condition presents with erythematous or skin-colored dome-shaped dermal papules or nodules, often associated with spontaneous bleeding, pain, pulsation, pruritus, and growth. Eosinophilia is only present in 20% of the cases, and lymphadenopathy is uncommon, unlike Kimura's disease. Extracutaneous involvement is rare. Various uncommon presentations have been reported. One of them includes ALHE at the lower lip.[3] Another unusual case of ALHE has been reported that arose on the lower back in a zosteriform distribution.[4]

The frequent presence of mural damage or rupture in intralesional large vessels of ALHE has suggested a role of trauma or arteriovenous shunting in its pathogenesis.[5] Cases have been reported in association with arteriovenous fistulae and malformations. Our cases were classically characterized by all the features of ALHE including female preponderance, peripheral blood eosinophilia, trauma-induced bleeding, and histopathological findings. The peculiarity of our cases was the age at presentation. ALHE has been rarely reported in children. A case of a 7-year-old child with a painless mass on his penis was reported, which was eventually diagnosed as ALHE.[6] Another case of ALHE on conjunctiva was reported in an 11-year-old girl.[7]

Pin-point diagnosis is essentially histopathology based. The proliferation of small to medium-sized blood vessels often showing a lobular architecture, many of which are lined by greatly enlarged (epithelioid) endothelial cells. A perivascular inflammatory cell infiltrate composed mainly of lymphocytes, and eosinophils is an important finding. Histologically, it appears as an angiomatous lesion with abundant proliferating blood vessels lined by prominent endothelial cells with a“histiocytoid” or“epitheloid” appearance and vacuolated cytoplasm. There is diffuse infiltration by lymphocytes and eosinophils.[5] The endothelial cells stain positive for CD34, von Willebrand factor and CD31.

Treatment of ALHE is dictated in part by the number, location, and size of the lesions. Rare instances of spontaneous regression have been reported. The natural history of the lesion is such that if a confident diagnosis is made on a small lesion, it is reasonable to observe the lesion for 3–6 months and await spontaneous regression. Patients with solitary or a few small lesions may benefit from excision or Moh's surgery.[8] About one-third of lesions recur after excision. Other treatment modalities used successfully include systemic and intralesional steroid administration, interferon therapy, cryotherapy, laser therapy, and topical application of tacrolimus.[9] There is an anecdotal report of response to imiquimod cream.[10] Our patients have been referred to the Department of Pediatric Surgery for excision and repair of the lesions.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Calonje E. Soft-tissue tumours and tumour-like conditions. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8th ed. West Sussex: Wiley Blackwell Publishers; 2010. p. 56.27-8.  Back to cited text no. 1
Al-Mutairi N, Manchanda Y. Angiolymphoid hyperplasia with eosinophilia associated with hepatitis C antibodies. Indian J Dermatol Venereol Leprol 2007;73:367.  Back to cited text no. 2
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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. 3
  Medknow Journal  
Kurihara Y, Inoue H, Kiryu H, Furue M. Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in zosteriform distribution. Indian J Dermatol 2012;57:401-3.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
Calonje E. Vascular tumors: Tumours and tumour-like conditions of blood vessels and lymphatics. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, Xu X, editors. Lever's Histopathology of Skin. 10th ed. Philadelphia: Lippincott Williams and Wilkins Publishers; 2009. p. 1024-5.  Back to cited text no. 5
Nishi M, Matsumoto K, Fujita T, Satoh T, Iwabuchi K, Iwamura M, et al. Angiolymphoid hyperplasia with eosinophilia on penile skin in a 7-year-old child. J Pediatr Surg 2011;46:559-61.  Back to cited text no. 6
Huang M, Lloyd WC 3rd, O'Hara M. Angiolymphoid hyperplasia with eosinophilia: An unusual presentation in a child. J AAPOS 2008;12:302-4.  Back to cited text no. 7
Leiferman KM, Peters MS. Angiolymphoid hyperplasia with eosinophilia and Kimura disease. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's Dermatology in General Medicine. 7th ed. New York: McGraw-Hill; 2008. p. 313-4.  Back to cited text no. 8
Kumar JV, Guruprasad KY. Angiolymphoid hyperplasia with eosinophilia. Indian J Dermatol Venereol Leprol 1998;64:133-4.  Back to cited text no. 9
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Gencoglan G, Karaca S, Ertekin B. Angiolymphoid hyperplasia with eosinophilia successfully treated with imiquimod. A case report. Dermatology 2007;215:233-5.  Back to cited text no. 10


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

This article has been cited by
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