Kimura’s disease: case report of an Italian young male and response to oral cyclosporine A in an 8 years follow-up

Kimura’s disease is a benign chronic inflammatory disease, common in Asian males and rare in Western people. Clinically, Kimura’s disease is characterized by subcutaneous nodular lesions, usually localised in head and neck, often associated with regional lymphadenopathy. Peripheral blood eosinophilia and elevated serum IgE are often observed. We report a case of a 40-year-old Italian patient presenting with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological and laboratory findings, a diagnosis of Kimura’s disease was made. The patient was treated with very low doses of cyclosporine A with no evidence of disease recurrence over the following 8 years. However, the discontinuation of cyclosporine A determined a relapse of the disease. The relevance of this case is due to the rarity of the disease in Italy, to its peculiar clinical presentation and, moreover, it is the first case in literature that has a good response to treatment with low doses of cyclosporine A, documented in an 8-year follow-up.

     

Kimura’s disease: case report of an Italian young male and response to oral cyclosporine A in an 8 years follow-up

Kimura’s disease is a benign chronic inflammatory disease, common in Asian males and rare in Western people. Clinically, Kimura’s disease is characterized by subcutaneous nodular lesions, usually localised in head and neck, often associated with regional lymphadenopathy. Peripheral blood eosinophilia and elevated serum IgE are often observed. We report a case of a 40-year-old Italian patient presenting with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological and laboratory findings, a diagnosis of Kimura’s disease was made. The patient was treated with very low doses of cyclosporine A with no evidence of disease recurrence over the following 8 years. However, the discontinuation of cyclosporine A determined a relapse of the disease. The relevance of this case is due to the rarity of the disease in Italy, to its peculiar clinical presentation and, moreover, it is the first case in literature that has a good response to treatment with low doses of cyclosporine A, documented in an 8-year follow-up.     

Lobular panniculitis with small vessel vasculitis associated with ulcerative colitis

We report a rare case of lobular panniculitis with small vessel vasculitis, presenting with fever, cutaneous lesions, and systemic manifestations involving the visceral fat and associated with ulcerative colitis. The patient was treated with cyclophosphamide and prednisolone, which successfully cured the systemic disease, with resolution of the inflammatory infiltrates.