Sir.Sweet syndrome is characterized by tender, erythematous plaques, most often on the head, neck or extremities. It is associated with haematological malignancies in more than 20% of cases (1. 2). Relapsing polychondritis (RP) is a rare rheumatological disorder in which recurrent episodes of inflammation result in destniction of cartilage of the ears and nose (3). Myelodysplastic syndrome (MDS) is a clonal disorder of haematopoiesis characterized by peripheral cytopaenias and dysplastic bone marrow, which is usually hypercellular. Diebold et al. (4) demonstrated that there was frequent evidence of MDS in their bone marrow study of RP patients. We report here a case of a patient with Japanese Sweet syndrome found subsequent to RP and MDS.
CASE REPORTAn 80-year-old Japanese man presented with a 1 -month history of flu-like illness followed by vertigo, joint pain and auricular swelling. Physical examination at our hospital revealed fresh red areas of erythema, with swelling and tenderness on the left ear. exeept ihe earlobe (Fig. la). Histopathologically. dense inflammatory infiltrations on the subcutaneous cartilage were observed along with degeneration ofthe marginal chondrocytes in the cartilage {Fig. lb). Ophthalmological and otolaryngologicai examination revealed uveitis and moderate sensorineural deafness. In laboratory examinations, biochemical tests detected high levels of C-reactive protein ( 11.0 mg/dl). The patient demonstrated auricular chondritis, ocular inflammation, hearing loss and seronegative inflammatory arthritis, whieh fulfilled the diagnostic criteria of RP according to Michet et al. (5). An enzyme-linked immunosorbent assay (ELISA) was performed as described using type II collagens as substrates (6). Results ofthe ELISA showed a high titre of antitype II collagen antibodies in the patient's serum: titres in sera from the patient and from normal individuals were 2.3 and 0.06 ±0.03 (n= 10) for antitype II collagen antibodies. Thrombocytopaenia and leukocytosis were deteeted. A bone marrow aspiration revealed MDS. Thirty mg/day of prednisolone was successful in suppressing the external ear inflammation and other organ symptoms, including eye, inner ear and joint symptoms, had rapidly subsided. Three months after his initial presentation to our institution, the patient often experienced coughing, hoarseness, aphonia, dyspnoea, wheezing, or tenderness over the trachea. Chest radiographs and computed tomography scans ofthe chest showed a left lower lobe infiltrate with pleural effusion. Exhaustive analysis of sputum, blood, urine, pleural fluid, and broncho-alveolar lavage fluid were negative for any infectious organism. Bronehoseopy with transbronehial biopsy for persistent left lower lobe infiltrate revealed bronchiolitis obliterans organizing pneumonia (BOOP). Eight months after admission at our institution, the patient presented with high-grade fever and painful cutaneous lesions on his neck, chest, back and extremities. Physical examination revealed multiple, erythematous, pseudovesicular plaq...