BACKGROUND: Kimura disease (KD) is a rare lymphoproliferative inflammatory disease of unknown etiology. Data regarding therapeutic modalities and pathophysiology are scarce. OBJECTIVES: Analyze therapeutic and follow-up data and compare KD with cutaneous IgG4-related disease (IgG4-RD). METHODS: Multicentric retrospective study of 25 KD patients with analysis of treatment, follow-up and IgG4 immunostaining. Comparison with published cases of cutaneous IgG4-RD. RESULTS: Patients were mostly male (84%), median-aged 42 years with lymph node, lacrimal/salivary gland and kidney involvements in 45, 24 and 12%, respectively. Surgical excision had 100% complete response and 60% relapse. Oral corticosteroids had 100% response with 50% relapse. Thalidomide, cyclosporine or interferon-α had 100% response, but 100, 20 and 50% relapse, respectively. KD showed clinicopathological similarities with 27 published cases of cutaneous IgG4-RD. CONCLUSION: Surgery may be used in resectable KD cases, whereas cyclosporine or thalidomide may represent interesting alternatives to oral corticosteroids in other cases. KD shares features with cutaneous IgG4-RD.
BACKGROUND:Kimura disease (KD) is a rare lymphoproliferative inflammatory disease of unknown etiology. Data regarding therapeutic modalities and pathophysiology are scarce. OBJECTIVES: Analyze therapeutic and follow-up data and compare KD with cutaneous IgG4-related disease (IgG4-RD). METHODS: Multicentric retrospective study of 25 KD patients with analysis of treatment, follow-up and IgG4 immunostaining. Comparison with published cases of cutaneous IgG4-RD. RESULTS:Patients were mostly male (84%), median-aged 42 years with lymph node, lacrimal/salivary gland and kidney involvements in 45, 24 and 12%, respectively. Surgical excision had 100% complete response and 60% relapse. Oral corticosteroids had 100% response with 50% relapse. Thalidomide, cyclosporine or interferon-α had 100% response, but 100, 20 and 50% relapse, respectively. KD showed clinicopathological similarities with 27 published cases of cutaneous IgG4-RD. CONCLUSION: Surgery may be used in resectable KD cases, whereas cyclosporine or thalidomide may represent interesting alternatives to oral corticosteroids in other cases. KD shares features with cutaneous IgG4-RD.