|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 2 | Page : 94-96
Unilateral angiolymphoid hyperplasia with eosinophilia
Vijayalaxmi Suranagi, Prakash R Malur, Manjunath Swamy
Departments of Pathology and Dermatology, Jawaharlal Nehru Medical College, Karnataka Lingayat Education Society (KLES) Dr. Prabhakar Kore Hospital and Medical Research Centre (MRC), Belgaum, Karnataka, India
|Date of Web Publication||18-Dec-2014|
Departments of Pathology and Dermatology, Jawaharlal Nehru Medical College, Karnataka Lingayat Education Society (KLES) Dr. Prabhakar Kore Hospital and Medical Research Centre (MRC), Belgaum, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Suranagi V, Malur PR, Swamy M. Unilateral angiolymphoid hyperplasia with eosinophilia. Indian J Dermatopathol Diagn Dermatol 2014;1:94-6
|How to cite this URL:|
Suranagi V, Malur PR, Swamy M. Unilateral angiolymphoid hyperplasia with eosinophilia. Indian J Dermatopathol Diagn Dermatol [serial online] 2014 [cited 2020 Oct 28];1:94-6. Available from: https://www.ijdpdd.com/text.asp?2014/1/2/94/147315
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare idiopathic condition that manifests in adults as isolated or grouped pruritic papules, plaques, or nodules in the skin of the head and neck region. Similar cases have been reported under various names such as inflammatory angiomatous nodule, pseudopyogenic granuloma, intravenous atypical vascular proliferation, histiocytoid hemangioma, and epithelioid hemangioma.  Most patients present with lesions in the periauricular region, forehead, or scalp.  Rare sites of involvement include the arms, hands, trunk, extremities, breasts, axilla, penis, oral cavity, tongue, lymph node, testis, and orbit. ,, We report a case of ALHE with multiple lesions limited to one side of the face, involving the fore head, inner canthus of eye, and angle of the mouth, and give a brief review of the literature with emphasis on the pathogenesis as well as the features distinguishing ALHE and Kimura's disease.
A 25-year-old woman presented with multiple itchy lesions on the left side of the face that were distributed in the regions of inner canthus of eye, eyebrow, nose, and angle of the mouth, with the age of the lesions varying from 6 months to 2 years [Figure 1]a and 1b. Examination revealed multiple discrete, erythematous to hyperpigmented smooth-surfaced papules and nodules, varying in size from 0.5 to 1 cm, which were soft in consistency, slightly tender, and compressible, with bleeding due to scratching. There was no associated regional lymphadenopathy. Systemic examination revealed no abnormality. Blood and urine analysis, chest X-ray, ultrasonography of abdomen and pelvis, and ECG were normal except for eosinophilia of 10% and an absolute eosinophil count of 545 cells/mm 3 . An excision biopsy of the lesion was performed.
|Figure 1: (a and b) Dome-shaped, dark-colored lesions near the inner canthus of eye and eyebrow; lesions seen on one side of the face|
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Microscopic examination revealed normal epidermis. Dermis showed proliferation of thick-walled blood vessels lined by plump endothelial cells protruding into the lumen [Figure 2], giving the typical cobblestone appearance [Figure 3]. Perivascular area showed infiltrate comprising of lymphocytes and eosinophils [Figure 4]. Some of the vessels showed mucoid stroma around them. A histological diagnosis of ALHE was made on excision biopsy of one of the lesions.
|Figure 2: Dermis showing proliferating thick-walled blood vessels with prominent endothelial cells and lymphoid aggregates (H and E, ×100)|
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|Figure 3: Typical cobblestone appearance of endothelial cells (H and E, ×400)|
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|Figure 4: Cell infiltrate comprising lymphocytes and eosinophils (H and E, ×400)|
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The disease was first described by Kimura et al. in 1948.  ALHE is seen most commonly in Asians, more common in Japan than in other countries, followed by Whites. It is slightly more common in females. ALHE presents most commonly in patients aged 20-50 years. It typically presents with an expanding nodule or a group of nodules, usually in the vicinity of the ear. The lesions may be associated with pain or pruritus. The lesions range from erythematous to brown, and may be eroded or crusted. ,
Most lesions arise superficially in the dermis in young to middle-aged females. Blood eosinophilia has been reported in up to 15-20% of patients.  The etiology of ALHE is unknown. It is not clear if it is primarily a vascular neoplasm, a lymphoproliferative process, a reactive proliferation secondary to diverse stimuli, including trauma, or a heterogeneous group of entities. Owing to the associated eosinophilia and elevated lgE levels, the pathogenesis of ALHE is presumed by some authors to be a hypersensitive response; however, the stimulus is unknown. A prior traumatic insult was reported in only 9% of 116 patients with ALHE.  There is some evidence that it may be related to traumatic pseudoaneurysm or an arteriovenous shunt supporting a vascular origin. , Antigenic stimulation following insect bites has been postulated by some authors. 
It has been suggested that ALHE may be a primary lymphoproliferative process, as evidenced by the findings of T-cell gene rearrangements. However, data supporting a primary lymphoproliferative process are few. Recently, monoclonality was confirmed by automated high-resolution polymerase chain reaction (PCR) fragment analysis. All these data raise the question of whether ALHE or a subset of ALHE represents either a true T-cell lymphoma of low-grade malignancy or a specific variant of reactive lymphoid hyperplasia.  According to Kempf et al., clonal lesional lymphocytes cannot be considered synonymous with malignant potency or overt malignancy, but it does shed new light on the pathogenic aspects of this disorder. 
Histologically, eosinophils typically comprise 5-15% of the infiltrate. Rarely, they may account for as much as 50% of the infiltrate. Lymphoid aggregates with or without follicle formation are typical. , In about 50% of cases, evidence of involvement of medium to large-sized arteries can be found. Rarely, the whole lesion is intravascular.  The differential diagnoses for ALHE include angiosarcoma (presence of nuclear atypia, mitotic activity), benign angiomas or ectasias (absence of an intrinsic inflammatory cell infiltrate and enlarged endothelial cells), insect bite reactions (vascular proliferation is rarely exuberant), and reaction to injected vaccines ( less marked vascular hyperplasia, absence of epithelioid endothelial cells, presence of aluminum-containing histiocytes). 
When ALHE was first described in Western Europe, similarities to Kimura's disease reported in Asia were noted. Indeed, many authors thought that both conditions might be part of one disease spectrum. However, more recently, most authorities emphasize differences between the two entities.  In Kimura's disease, male predominance, tendency for more extensive lesions, and involvement of salivary tissue and lymph nodes, skeletal muscles, and sites distant from the head and neck region are observed. Authors from Asia have stressed the histologic differences, the most important of which are the lesser degree of exuberant vascular hyperplasia, lacking prominent endothelial cells, absence of uncanalized blood vessels and lesions centered on damaged arteries, and presence of eosinophilic abscesses and marked fibrosis around the lesion in Kimura's disease.  The lesions are deep-seated, and peripheral blood eosinophilia and elevated serum IgE are said to be more common in Kimura's disease.
Treatment of ALHE is often challenging. Intralesional corticosteroids and irradiation have been used, but are not very effective. Other treatments that have been reported include topical imiquimod, topical tacrolimus, isotretinoin, and interferon alfa-2b.The lesions tend to recur. Moh's micrographic surgery, pulsed-dye laser, and carbon dioxide laser have been used with some success. Cryosurgery and electrosurgery have also been reported. ,
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]