Angiolymphoid hyperplasia with eosinophilia

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Angiolymphoid hyperplasia with eosinophilia (ALHE), also known as epithelioid hemangioma, is an uncommon disease. ALHE is a benign vascular proliferation of unknown etiology with a characteristic component of epithelioid endothelial cells.

Recent literature review shows that ALHE usually affects patients in their third to fourth decade with approximately an equal male to female ratio. Cutaneous lesions present as single or multiple cutaneous papules or subcutaneous nodules, sometimes with inflammatory features. The ear and periauricular area, face, and scalp are most commonly affected. Peripheral blood eosinophilia is noted in 10%–20%. Regional lymphadenopathy is observed in 5%–10%. Although considered benign in nature, the etiology of ALHE is unknown. Neoplastic proliferation of vascular tissue, arteriovenous shunting, or reactive hyperplasia of vascular tissue secondary to trauma (such as ear piercing), infection (human T-lymphotropic virus [HTLV] or herpes virus 8), renin, or hyperestrogenic states have all been proposed as causal factors. ALHE may cause symptoms such as cosmetic disfigurement, bleeding, itch, and pain. It has been associated with various lymphoproliferative conditions, supporting the contention that it may represent a monoclonal T-cell process.

The differential diagnoses of ALHE include Kimura disease, pyogenic granuloma, lymphocytoma cutis, insect bite, sarcoidosis, Kaposi sarcoma, and angiosarcoma. ALHE and Kimura disease are generally regarded as separate clinicopathological entities. Kimura disease is a chronic inflammatory condition of unknown etiology often affecting young Asian men, and typically presenting as cervical lymphadenopathy and subcutaneous nodules in the head and neck region. However, coexistence of ALHE and Kimura disease in the same patient has been reported.

Management Strategy

Spontaneous remission is rare in ALHE. Complete surgical excision is preferred for persistent lesions. Recurrence may occur if excision is incomplete. Laser therapy and radiofrequency ablation are alternative surgical options. Cryotherapy, inexpensive and easily available, is a conventional treatment although reports on its efficacy are lacking. Other treatments reported effective include topical and intralesional corticosteroids, propranolol, topical imiquimod, topical tacrolimus, isotretinoin, methotrexate, suplatast tosilate, intralesional interferon-α _2b, thalidomide, and photodynamic therapy . Whatever treatment method, recurrence and incomplete resolution remain frequent. Although treatment failure is lowest with excision, it is still noted in about 40.8%.

Specific Investigations

Histopathology

Dermoscopy

Imaging

Histopathological features of ALHE are characterized by a proliferation of blood vessels of varying sizes lined by plump endothelial cells. These histiocytoid endothelial cells show abundant eosinophilic or clear cytoplasm and large vesicular nuclei. The cells are mostly cuboidal with occasional ‘hobnailing’, which is related to the presence of cytoplasmic vacuoles in these cells, causing cytoplasmic protrusion into lumina. There is also an accompanying perivascular lymphocytic and eosinophilic infiltrate.

Dermoscopy may help to differentiate ALHE from other vascular tumors such as angiosarcoma or Kaposi sarcoma. Under polarized light, ALHE shows polymorphous vascular pattern composed of dotted and linear vessels with a regular distribution over a pale-reddish to light-pink background.

The location and extent of underlying vascular anomalies may be assessed by angiography, angio-computed tomography, and angio-magnetic resonance imaging.

First-Line Therapies

Surgery	D
Laser therapy	D
Corticosteroid, topical or intralesional	E

Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia (ALHE): a systematic review

Adler BL, Krausz AE, Minuti A, et al. J Am Acad Dermatol 2016; 74: 506–12.

After reviewing 416 studies detailing 908 patients, the authors concluded that surgical excision was the best option, albeit suboptimal, with a treatment failure rate of 40.8%, defined as incomplete resolution of disease or recurrence after treatment. Pulsed dye lasers, or other lasers, may represent other reasonable therapeutic options. The treatment failure rates for different lasers ranged from 50% to 66.7%.

Facial angiolymphoid hyperplasia with eosinophilia: sustained remission following treatment with carbon dioxide laser

Ali FR, Madan V. Clin Exp Dermatol 2016; 41: 96–8.

A 35-year-old woman presented with biopsy-proven ALHE lesions on her left temple, which had developed during pregnancy 5 years previously. Ablation with CO ₂ laser (15W, 4-mm spot, 10600 nm) was undertaken in three sessions over the course of 8 months, with an excellent clinical response. There was no recurrence after 30 months.

Combined pulsed dye and CO ₂ lasers in the treatment of angiolymphoid hyperplasia with eosinophilia

Sagi L, Halachmi S, Levi A, et al. Lasers Med Sci 2016; 31(6): 1093–6.

The authors reported combination of pulsed dye laser and CO ₂ laser in treating 14 patients with ALHE. All patients exhibited clinical response after a mean of 2.4 ± 0.4 treatment sessions. They did not recommend surgery for risk of recurrence or development of new lesions and that further surgical excision would result in a new scar.