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Angiolymphoid hyperplasia with eosinophilia (ALHE) is characterized by angiomatoid papules and nodules localized mostly on the head, face and neck. This inflammatory tumor was first categorized by Mehregan et al.1 and recognized as a distinct entity from Kimura’s disease.2 ALHE frequently occurs on the head, face and neck in young adults, and the trunk and extremities have been reported as rare sites of the lesion.3 Here, we report a case of ALHE occurring on the leg with a good therapeutic response to suplatast tosilate.

A 63-year-old man was referred to our hospital in October 2008 with a 3-month history of a dark reddish lesion on the right calf (Fig. 1a). He had been treated with topical steroids without therapeutic effect. On examination, there was an irregularly elevated, dark reddish plaque, 4.1 cm × 3.5 cm in diameter on the right upper calf. There was no regional lymphadenopathy or other pathological findings. A biopsy specimen from the lesion revealed that small blood vessels proliferated in the dermis and were accompanied by densely infiltrating eosinophils and lymphocytes (Fig. 2a). No germinal centers were formed in the dermis. Some of the vascular channels were lined with enlarged endothelial cells (Fig. 2b). Laboratory data including eosinophil count and total serum immunoglobulin (Ig)E were within normal limits. We diagnosed the lesion as ALHE.

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Figure 1.  Clinical appearance of skin lesion on the upper calf before (a) and after 5 month’s treatment (b) with suplatast tosilate.

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Figure 2.  (a) Skin biopsy specimen showing proliferation of small blood vessels and infiltration of eosinophils in the dermis and (b) swollen endothelial cells of vascular channels (hematoxylin–eosin, original magnification ×100).

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Because he had been treated with topical steroids without satisfactory effect, p.o. administration of suplatast tosilate was started along with the topical ointment. The lesion was dramatically improved after the 4-week administration (Fig. 1b), and completely disappeared 4 months after the beginning of the treatment. No recurrence was observed even 15 months later.

Angiolymphoid hyperplasia with eosinophilia arises mostly on the head and neck regions with the predilection sites of the external ear and external auditory canal. The occurrence of ALHE outside the head and neck area has been noted as a rare instance.4–6 Olsen et al.3 reviewed 116 cases of ALHE and found four cases (3.4%) occurring on the leg.

Treatment of ALHE is still challenging. Although various therapeutic modalities have been reported to be successful,7–10 there is no definite proposal for the first choice. Topical steroids, tacrolimus7 or imiquimod8 may be effective but sometimes unsatisfactory without combination of other therapies. Surgical removal is inappropriate for the large and non-circumscribed lesions, and cosmetically problematic. Photodynamic therapy9 and laser therapy10 may be another choice when they are available and the lesions are located on the limited areas.

Suplatast tosilate is a unique anti-allergic agent, which has been developed as a T-helper cell (Th)2 cytokine inhibitor in Japan. It exerts an inhibitory effect on interleukin (IL)-4 and IL-5 production by Th2 cells, and reduces IgE production by B cells.11 A successful therapeutic result with this agent has been reported in another case of ALHE.12 Although the mechanism underlying ALHE remains uncertain, the good therapeutic response to suplatast tosilate may provide further evidence for the pathogenic role of Th2 cells in ALHE. In relation to its inhibitory effect on Th2 cells, this drug is known to depress eosinophil infiltration, leading to further improvement of ALHE.13 Our case of ALHE is unique in not only its location but also its clinical appearance, exhibiting an irregularly configured nodule on a plaque. This morphology may be related to the inflammatory property of ALHE rather than the neoplastic moiety that is represented by pseudopyogenic granuloma. It is possible that suplatast is effective for the inflammatory type of ALHE as seen in our patient. Further investigation may clarify the efficacy of suplatast tosilate for ALHE.

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