Abstract
A 70-year-old Japanese woman had been diagnosed and treated with oral administration of steroid along with potassium iodide in the division of gastroenterology and the division of dermatology of her hometown hospital for a ten-year history of Sweet disease (SD) accompanied by Crohn’s disease. Since 2003, she had been treated in the division of gastroenterology with maintenance therapy with oral prednisolone 7.5 mg daily along with potassium iodide 900 mg daily. On April, 2009, severe indurated and ulcerated erythema appeared on her trunk, expanding to her head and the fingers without exacerbation of CD. The histopathology revealed neutrophilic and lymphocytic infiltrations throughout the dermis. She was diagnosed as pyoderma gangrenosum (PG). Severe anemia and decreased platelets were associated problems. A bone marrow biopsy showed atypical plasmacytoid myeloma cells. In addition, she had an increased level of IgA lambda chain by serum electrophoresis and positive Bence-Jones protein by urine test. Thus the association of multiple myeloma (IgA lambda type) was established. The cutaneous manifestations were successfully treated with increased prednisolone 30 mg daily. In contrast, the MM has been refractory and complicated with persistent biphasic cytopenia, repeated pneumonitis, and dyspnea, despite treatment with MP (prednisolone, melphalan) therapy or intravenous bortezomib injection, so she is now under treatment. We believe this case falls within the spectrum of the neutrophilic dermatosis of myeloproliferative disorders. When PG develops without exacerbation of CD, we should consider and test for an association of malignancy of intestinal or other organs. Such a case of PG following MM in a patient with CD and associated with SD has never previously been described, to the best of our knowledge.
