Kimura’s Disease Presenting as Steroid-Responsive Thromboangiitis Obliterans
A 60-year-old Japanese man presented with severe numbness in the fingers of both hands, which were cold and pale in appearance. He was found to have marked eosinophilia (leukocyte count 18 500 /μL with 58.3% eosinophils) and was admitted immediately to the nearby hospital. Treatment with intravenous methylprednisolone pulse (1 g for 3 days) was initiated, followed by oral prednisone 60 mg daily. Although his blood eosinophilia normalized rapidly, gangrene developed in his fingers (Figure 1). Bone marrow examination showed no blast cell proliferation. Tests for antinuclear and antiphospholipid antibodies, rheumatoid factor, antineutrophil cytoplasmic antibodies, and cryoglobulins were all negative. Protein S and protein C were within the normal range. After prednisone was tapered gradually and discontinued, he was referred to our hospital with a diagnosis of thromboangiitis obliterans (TAO). Arteriography of his extremities showed diffuse narrowing and obliterations of both radial and ulnar arteries (Figure 2). In addition, nodular pools of the contrast media were detected in both upper arms (Figure 3). These lesions were tangible as subcutaneous nodules by careful palpation. Although the patient initially noticed the nodules in his thirties, they gradually increased in size and number a few months ago. He also noted that these nodules became smaller after steroid treatment. The histology of the left amputated fingers showed the obliteration of small digital arteries by organized thrombus; there was no evidence of vasculitis (Figure 4). One week after discontinuation of prednisone, the patient developed generalized pruritic papules, persistent nonproductive cough, and pulmonary infiltrates in his lower lungs, along with the recurrence of eosinophilia and a high serum titer of immunoglobulin E (2829 U/mL). Because incisional biopsy of a subcutaneous nodule in his left upper arm established the diagnosis of Kimura’s disease (Figure 5), treatment with dexamethasone 5 mg daily was initiated. Eosinophilia, skin eruption, and dry cough disappeared quickly, whereas digital gangrene of his right thumb and index finger improved gradually (Figure 6). Kimura’s disease is a rare, benign, chronic inflammatory disorder that affects predominantly young Asian men and is characterized by marked eosinophilia, elevated immunoglobulin E level, and recurrent subcutaneous nodules around head and neck.1 Usually the lesions remain unchanged for years with no systemic manifestations, which sharply contrasts with idiopathic hypereosinophilic syndrome. The latter is often accompanied by thromboembolic phenomena, persistent dry cough, pulmonary infiltrates, and skin rash.2 Uncommon location of the nodules, the disappearance of the nodules after initial steroid treatment, an atypical age of the disease onset, and thromboembolic phenomena allowed the accurate diagnosis of Kimura’s disease in the present case challenging.3 Recently, 2 cases of steroid-responsive TAO were reported.4 The clinical characteristics of these 2 cases and the present case are similar, but quite different from those of typical TAO cases, particularly considering the patient’s advanced age, abstinence from smoking at the disease onset, and remarkable eosinophilia. Considered together, TAO with eosinophilia could be a subtype of Kimura’s disease. We propose an evaluation of whether a reported case5 of Kimura’s disease presenting with Raynaud’s phenomenon and digital infarcts is a subtype of Kimura’s disease.