Clinical Challenge: Mildly pruritic pink papules a patient’s forehead

Angiolymphoid hyperplasia with eosinophilia (ALHE), also known as epithelioid hemangioma, is an uncommon idiopathic eruption of dome-shaped papules, plaques or nodules.1 Lesions may be skin-colored, tan-to-brown, pink or dull red in color, and predominately occur on the periauricular area, forehead...

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Angiolymphoid hyperplasia with eosinophilia (ALHE), also known as epithelioid hemangioma, is an uncommon idiopathic eruption of dome-shaped papules, plaques or nodules.¹ Lesions may be skin-colored, tan-to-brown, pink or dull red in color, and predominately occur on the periauricular area, forehead and scalp.¹ Intraoral, genital and extremity lesions have also been described, but are less common. 

AHLE may appear as a single lesion or grouped lesions in a single anatomic distribution. ALHE occurs in young- to middle-aged adults and has a slight female predominance.^1,2  The condition occurs most often in people of Asian race/ethnicity, but also occurs in whites and less often, blacks.

Lesions may be asymptomatic, pruritic and painful. Some lesions may be pulsatile, and in rare cases, bleed. Approximately 20% of patients have an accompanying peripheral eosinophilia.³

The pathogenesis is not entirely clear, but a preceding trauma has been identified in many cases and may serve as a precipitating factor for the formation of ALHE lesions. Vascular damage or rupture with subsequent formation of an arteriovenous shunt, fistula or malformation is commonly present at the base of the lesions, further suggesting a pathogenic role for trauma.^1,2  

Because of this association, many consider ALHE to be a reactive process. Others maintain that ALHE is simply a benign neoplastic phenomenon. Lesions may slowly expand overtime, but to date there have been no reported cases of malignant transformation.

Diagnosis

Patients with ALHE present with distinctive histopathologic features, and a skin biopsy is required to make the diagnosis. The epidermis is uninvolved in ALHE; however, circumscribed roughly lobular proliferations of vessels and inflammation appear in the dermis and subcutaneous tissue.⁴ 

There is a diffuse or rich nodular infiltrate composed of lymphocytes and eosinophils, with occasional mast cells and plasma cells.¹ “Cobblestone” or “hobnail” enlarged endothelial cells line the vascular lumens and create a scalloped appearance.^1,4  The synonym “epithelioid hemangioma” refers to these epithelioid endothelial cells.¹

Treatment

Patients are often bothered by the cosmetic appearance of AHLE lesions, prompting them to seek medical advice, but lesions do not typically spontaneously regress. 

Managing AHLE cases can be challenging. Unfortunately, there is no single treatment option that is universally successful. Lesions can be surgically excised, but slowly return in one-third of cases.^1,2  This is especially true in cases where the underlying vascular anomaly has not also been addressed. 

Several other treatment options have been tried with varying success, including: intralesional corticosteroids, pulsed dye laser, cyrotherapy, electrodesiccation, pentoxifyllin and indomethacin.² 

In cases that are particularly resistant to treatment, chemotherapeutic agents, such as interferon or vinblastine, may be beneficial.²       

Kristy Fleming, MD, is a third year dermatology resident at Baylor College of Medicine.     

References

1. J Bolognia, JL Jorizzo, RP Rapini. Dermatology. 2nd Ed. Elsevier; 2008: 1774-1775.

2. James WD, Berger TG, Elston DM. Andrew’s Diseases of the Skin Clinical Dermatology. 10th ed. Pennsylvania: Saunders Elsevier; 2006:590-591.

3. Gilchrest, Goldsmith, Katz et al. “Angiolymphoid Hyperplasia with Eosinophilia and Kimura Disease.” Fitzpatrick’s Dermatology in General Medicine. Vol 1. 7th ed. New York, NY: McGraw-Hill Medical; 2008:313-14.

4. Rapini R. Practical Dermatopathology. Elsevier; 2005: 324.