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Pyoderma gangrenosum (PG) is a chronic inflammatory disease of unknown cause that presents as an inflammatory and ulcerative disorder of the skin. PG is often associated with an underlying systemic disease. However, the frequencies of the underlying diseases are unclear in Japanese patients. In this retrospective, observational study, all patients diagnosed with PG who visited dermatology departments of nine regional hospitals in and around Ibaraki Prefecture were collected from 1982 to 2011 or 2014. The diagnoses of PG were based on the characteristic clinical and histological appearances and ruling out of infection. Sixty-two PG patients, including 29 males and 33 females, were identified. The ages of onset were 16-89 years, and the mean age was 50.2 years. Fifty (80%) of the 62 patients presented with an ulcerative PG, and the lower leg was the most common site (74%). Forty-six (74%) PG patients had underlying diseases. The most frequent was ulcerative colitis (32%), followed by myelodysplastic syndrome (11%), rheumatoid arthritis (6%) and aortitis syndrome (5%). For treatment, 54 cases (87%) received systemic corticosteroids and 10 received additional treatment with cyclosporin. There was no significant correlation between underlying diseases and response to the initial treatment. Multivariate analysis revealed that the number of affected sites negatively correlated with successful initial treatment. Fifteen (24%) of the 62 cases relapsed. In conclusion, ulcerative colitis and hematological disorders were frequently associated with PG while approximately a quarter of the cases were idiopathic.

Mucous membrane pemphigoid (MMP) is an autoimmune bullous disease of the mucous membranes, including the eye, nose, oral cavity, pharynx, larynx and oesophagus, with occasional skin lesions (1). We report here a case of a patient with MMP who demonstrated concomitant tracheal and bronchial lesions, in addition to oral, ocular and cutaneous lesions, in whom autoantibodies to β3 subunit of laminin-332 were detected by immunoblot analysis. CASE REPORTA 76-year-old Japanese woman visited Tsuchiura Kyodo General Hospital for pain in the mouth and hoarseness, which had been present for 5 months. She had no remarkable past or family history.Physical examination revealed conjunctival inflammation, bullae on the lip, ulcers and erosions in the oral cavity, and erosions on the abdomen (Fig. 1a-d).Laboratory data showed elevated C-reactive protein (4.18 mg/ dl; normal values, < 0.3 mg/dl) and white blood cell (9,600/μl; normal ranges, 4,000-8,000 μl). Circulating autoantibodies to bullous pemphigoid (BP) 180 (normal values, < 9), BP230 (normal values, < 9), desmoglein (Dsg) 1 (normal values, < 5), and Dsg3 (normal values, < 5) were not detected by enzyme-linked immunosorbent assay (ELISA). The BP180 ELISA index was 0.40 and the BP230 ELISA index was 0.70. The Dsg1 and Dsg3 ELISA indexes were less than 5. Endoscopy revealed ulcers in the pharynx, larynx and oesophagus. Because of the pain in the patient's throat, bronchoscopy could not be performed before treatment. Gallium scintigraphy and computed tomography did not detect any malignant tumours.Histopathological examination of biopsies taken from the lesions on the lower abdomen and the lower lip showed subepithelial cleft, lymphocytic infiltration and increased fibroblasts in the connective tissues. Direct immunofluorescence showed linear deposition of IgG and C3 at the basement membrane zone (BMZ). Indirect immunofluorescence of 1M NaCl-split skin revealed circulating IgG anti-BMZ autoantibodies bound to the dermal side of the split at 1:40. Immunoblot analyses of normal human epidermal and dermal extracts, recombinant proteins of BP180 NC16a and C-terminal domains and concentrated HaCaT cell culture supernatant showed no positive reactivity, while the 140 kDa β3 subunit was detected in purified human laminin-332 (Fig. 1e). A diagnosis of MMP with autoantibodies to β3 subunit of laminin-332 was made.Eight weeks after combination treatment with systemic pred nisolone 50 mg daily and cyclophosphamide pulse (500 mg every 4 weeks), the inflammatory cutaneous and mucosal lesions cleared, but conjunctival adhesion and stenosis of the

Sphingobacterium species rarely cause human infection. We herein report two cases of cellulitis complicated with bacteremia due to this genus. The patients, both in their 70s and receiving corticosteroid therapy for their underlying diseases, had antecedent skin injuries in their affected limbs. The patients' symptoms improved promptly and completely with the administration of cefazolin, which did not inhibit the growth of isolated organisms at a concentration of 4 μg/mL.

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A 47-yr-old woman who had previously received methimazole (MMI) treatment for hyperthyroidism was found to have glucagon binding autoantibodies in plasma. She had never received glucagon. The binding substances were detected in plasma at the time of a glucagon RIA. [125I]Glucagon binding was inhibited only by porcine glucagon and porcine glicentin, and dissociated at acid pH. The substances proved to be glucagon binding antibodies (immunoglobulin G, L-chain K-type), as determined by ammonium sulfate and radioprecipitation. There were no clinical manifestations related the presence of these autoantibodies. In a survey of 91 patients with thyroid disease, 3 patients whose plasma bound [125I]glucagon were identified among 41 with hyperthyroidism who were receiving MMI treatment. Such binding was not found in plasma from untreated hyperthyroid patients, those receiving propylthiouracil or those with chronic thyroiditis. These findings suggest that the development of glucagon antibodies in hyperthyroidism may be associated with MMI treatment.

In this study, we report a case of a patient on dialysis who presented necrotic lesions on the legs and penile ulceration 7 years after a mechanical aortic valve replacement. The diagnosis of calciphylaxis was not confirmed even after skin biopsy, and multidisciplinary management was not initiated until the patient was admitted with septic shock. Cardiovascular surgeons should be aware of warfarin-induced calciphylaxis, whose pathophysiology differs from that of atherosclerosis. Considering poor long-term survival of dialysis patients, mechanical valves should be reserved only for those patients whose estimated survival is longer than the time taken for a biological valve to deteriorate.