A Case of Kimura's Disease as Giant Pedunculated Tumors.

Sir,

The patient, a 41-year-old woman, had noticed swelling in the periauricular region in her teen; her tumors were diagnosed at the age of 23 as Kimura's disease. They shrunk after 4-month treatment with oral prednisolone 15 mg/day. Over 18 years since she visited our clinic, she presented with a 13 × 7 × 6.5 cm pedunculated tumor in the pre-auricular area and 6-, 5-, and 4-cm tumors from the post-auricular to submandibular regions [Figure 1]; they occasionally itched. The overlying skin was pigmented with partial depigmentation. There was no swelling lymph node in this case. Her white blood cell count and serum IgE level were 6340/μl with 32.5% eosinophils and 34,456 U/ml, respectively. Biopsy of a post-auricular tumor showed reactive lymphoid follicles and eosinophil infiltration associated with fibrosis in the deep dermis and subcutaneous tissue [Figure 2] and nearly complete loss of elastic fibers in the dermis [Figure 3]. The tumors were excised and she received local monthly injections of 20 mg corticosteroid. There was no relapse in 18 months.

Figure 1

Large pedunculated tumors in the same regions at age 41

Figure 2

Reactive lymphoid follicles and infiltration of eosinophils associated with fibrosis in the deep dermis and subcutaneous tissue (H and E, ×12.5, ×200)

Figure 3

Comparison with a site-matched control reveals nearly complete loss of elastic fibers in the dermis of the pedunculated tumor (Verhoeff-van Gieson stain). Left: Control and right: Patient

Kimura's disease, a chronic inflammatory disorder, was first reported as eosinophilic hyperplastic lymphogranuloma.[1] Kimura et al. reported two Japanese patients with unusual granulation combined with hyperplastic changes of lymphatic tissues and in 1949 Iizuka et al. detailed the clinical and pathologic features of 11 patients.[23]

Kimura's disease, endemic in Asia, is commonly seen in young males. It is characterized by slowly progressive solitary or multiple subcutaneous painless masses primarily on the head and neck. They are sometimes pruritic. Histologically, there are reactive lymphoid follicles with eosinophilic infiltration, dense fibrosis, and abnormal vascular proliferation in the deep dermis or subcutaneous tissue. Vascular changes are capillary proliferation and increased numbers of thin-walled blood vessels often accompanied by regional lymphadenopathy.

The largest pedunculated tumor in our patient measured 13 × 7 × 6.5 cm. Our literature review found two patients with Kimura's disease presenting as 13 × 7 × 6.5 cm and 13 × 7 × 6.5 cm pedunculated tumors in a 38-year-old man and a 42-year-old woman.[45] We compared the elastic fibers of the lesion with the cutis laxa where elastic fibers disappear to be replaced by fine dust-like granules. Electron microscopic (EM) findings are the presence in the vicinity of the elastic fibers of electron-dense amorphous or granular aggregates that are thought to represent a defect in the synthesis of elastic fibers. In our case, Verhoeff-van Gieson staining showed that the elastic fibers had almost completely disappeared [Figure 3]; EM study disclosed no amorphous or granular materials, suggesting degeneration or destruction of the fibers by chronic mechanical stress or elastolytic factors derived from infiltrating components.

Previously-reported tumors were excised with or without the prior administration of systemic corticosteroids. We removed the tumors surgically and injected topically. Others treated Kimura's disease with intralesional or systemic corticosteroids, cyclosporine, interferon-α, chemotherapy, electrodessication, curettage, cryotherapy, radiotherapy, and local excision alone or in combination. While drugs are effective in reducing the size of the tumors, they often recur after the drugs are stopped. We recommend combined excision and lesional corticosteroids to treat giant, especially pedunculated tumors of Kimura's disease.