Osamu Tago

A randomized double-blind trial of intravenous immunoglobulin for bullous pemphigoid.

BACKGROUNDnPatients with steroid-resistant bullous pemphigoid (BP) require an appropriate treatment option.nnnOBJECTIVEnA multicenter, randomized, placebo-controlled, double-blind trial was conducted to investigate the therapeutic effect of high-dose intravenous immunoglobulin (IVIG; 400mg/kg/day for 5days) in BP patients who showed no symptomatic improvement with prednisolone (≥0.4mg/kg/day) administered.nnnMETHODSnWe evaluated the efficacy using the disease activity score on day15 (DAS15) as a primary endpoint, and changes in the DAS over time, the anti-BP180 antibody titer, and safety for a period of 57days as secondary endpoints.nnnRESULTSnWe enrolled 56 patients in this study. The DAS15 was 12.5 points lower in the IVIG group than in the placebo group (p=0.089). The mean DAS of the IVIG group was constantly lower than that of the placebo group throughout the course of observation, and a post hoc analysis of covariance revealed a significant difference (p=0.041). Furthermore, when analyzed only in severe cases (DAS≥40), the DAS15 differed significantly (p=0.046). The anti-BP180 antibody titers showed no difference between the two groups.nnnCONCLUSIONnIVIG provides a beneficial therapeutic outcome for patients with BP who are resistant to steroid therapy.

Isolated cutaneous manifestation of IgG4-related disease

Aims The cutaneous manifestation of IgG4-related disease has rarely been reported. The aim of this study is to identify and describe the cutaneous manifestations associated with IgG4-positive plasma cell infiltration in the skin. Methods The authors investigated two cases of IgG4-related disease with solitary skin lesions and compared the immunohistochemical characteristics of infiltrating cells among IgG4-related disease, Kimuras disease and cutaneous pseudolymphoma. Results IgG4-related disease manifested as an indurated plaque on the anterior chest in one case and a nodule on the toe in the other case. Histopathologically, skin lesions of IgG4-related disease showed a dense, mixed-cell infiltrate containing lymphocytes, plasma cells and eosinophils along with fibrosis. Plasma cells stained positively with anti-IgG and anti-IgG4 antibodies, and the ratio of IgG4+/IgG+ cells was more than 50%. Serum levels of IgG and IgG4 were not elevated and no lesions were found in other organs. Skin samples taken from Kimuras disease showed histopathological features similar to those of IgG4-related disease. The proportion of IgG4+/IgG+ was high in Kimuras disease, but not in cutaneous pseudolymphoma. Conclusions The solitary skin lesions of IgG4-related disease were similar histologically and immunohistochemically to the skin lesions of Kimuras disease. The concept of IgG4-related disease may help clarify the pathomechanism of diseases of unknown aetiology that possess features of IgG4-related disease.

Clinical follow‐up study of adult‐onset Still’s disease

Eighteen patients with adult‐onset Still’s disease have been followed up for 3–22u2003years in our department. Initial manifestations were fever with skin rash in 14 patients, fever, skin rash and sore throat in two, skin rash in one and arthralgia in one. During the follow‐up period, typical skin rash was seen in all patients, of them five patients (29%) revealed atypical skin rash simultaneously. Atypical rash included persistent erythema with pigmentation in two, persistent plaques and papules with linear erythema in two and edema of the eyelids mimicking dermatomyositis in one. Persistent papules and plaques revealed histologically characteristic features, such as dyskeratotic keratinocyte and liquefaction degeneration as well as a sparse superficial dermal infiltrate containing scattered neutrophils. In patients of chronic articular type and polycyclic systemic type, atypical skin rash, lymphadenopathy and hyperferritinemia were noted to be significantly higher than those of monocyclic type. These factors might be prognostic factors of adult‐onset Still’s disease in our study.

Digital Gangrene in Systemic Lupus Erythematosus

Digital ulcers and gangrene are common skin manifestations of connective tissue diseases, especially systemic sclerosis, although they are relatively rare in systemic lupus erythematosus. We describe here three patients with digital gangrene and systemic lupus erythematosus. None of the patients showed high disease activity of systemic lupus erythematosus at the time the digital gangrene developed. Two patients were positive for anti-RNP antibodies; however, no symptoms of other collagen diseases were present. One patient had anti-phosphatidylserine/prothrombin complex antibodies, and the other had anti-cardiolipin beta2 glycoprotein I antibodies and lupus anticoagulant at low titre. All patients showed narrowing or occlusion of radial and/or ulnar arteries in addition to digital arteries. Although a complication of anti-phospholipid syndrome is considered to be a possible cause, there may be unidentified causes other than thrombosis, atherosclerosis, overlap syndrome and vasculitis.

Bosentan for digital ulcers in patients with systemic sclerosis

Recurrent digital ulcers are manifestations of vascular disease in patients with systemic sclerosis (SSc). We report six patients with severe digital ulcers who were treated with bosentan administered p.o., 62.5–125u2003mg daily. The mean duration from the diagnosis of SSc to the initiation of bosentan was 9.5u2003years, and the observation period after bosentan administration was from 7u2003months to 4.5u2003years. In case 1, neither new digital ulcers nor Raynaud’s phenomenon developed for 4.5u2003years. In case 2, digital ulcers recurred after the discontinuation of bosentan; however, re‐administration of bosentan lead to the improvement. In cases 3–5 with recurrent digital ulcers, no new lesions have developed. In these five patients, pain evaluated by visual analog scale was significantly reduced. In three patients, bosentan was discontinued because of severe liver dysfunction. These results suggest that bosentan is an effective treatment for refractory digital ulcers associated with SSc; however, liver function should be carefully monitored. Compared to the doses of bosentan used to treat pulmonary hypertension, relatively lower doses may effectively control painful digital ulcer/gangrene in patients with SSc.

Clinical analysis of leg ulcers and gangrene in rheumatoid arthritis.

Leg ulcers are often complicated in patients with rheumatoid arthritis (RA), however, the etiology is multifactorial. We examined the cases of leg ulceration or gangrene in seven RA patients who were hospitalized over the past 3 years. One patient was diagnosed as having pyoderma gangrenosum. Although vasculitis was suspected in three patients, no histological evidence was obtained from the skin specimens. In these patients, angiography revealed the stenosis or occlusion of digital arteries. In the remaining three patients, leg ulcers were considered to be due to venous insufficiency. Treatment should be chosen depending on the causes of leg ulcers.

A case of acne fulminans successfully treated with cyclosporin a and prednisolone.

Acne fulminans (AF) is a rare condition and the most severe form of acne. It is characterized by the sudden onset of painful and ulcerative pustules and systemic symptoms including high fever and polyarthralgia. Response to ordinary antibiotic agents is poor, and the treatment most often recommended is systemic corticosteroids. We report here a case of a young Japanese man with acne fulminans presenting with severe pustules and pyoderma gangrenosum (PG)-like ulcerations.

Elevated serum levels of TARC/CCL17, eotaxin-3/CCL26 and vascular endothelial growth factor in a patient with non-episodic angioedema associated with eosinophilia and granulomatous cutaneous reaction.

Episodic angioedema associated with eosinophilia (EAE) is characterized by recurrent episodes of edema, eosinophilia in peripheral blood, urticaria, fever, and weight gain [1]. Non-episodic angioedema associated with eosinophilia (NEAE) has been reported as a single episode of angioedema that was responsive to treatment of low-dose corticosteroids or antihistamines [2].A 41-year-old Japanese woman noted urticaria and swelling in both lower legs in September 2011. The swelling extended to hands and [...]

Systemic sclerosis associated with silicone breast implantation

ejd.2013.2167 Auteur(s) : Sei-ichiro Motegi smotegi@gunma-u.ac.jp, Tomoyasu Hattori, Osamu Tago, Akira Shimizu, Osamu Ishikawa Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan We observed 5 systemic sclerosis (SSc) patients with silicone breast implantation (SBI) (2.1%) (mean age, 56.8) and 232 SSc patients without SBI (mean age, 65.3) from 2006 to 2012. Characteristics of the SSc patients with or without SBI are summarized [...]

An unusual case of erythema elevatum diutinum with penile and laryngeal manifestations

Erythema elevatum diutinum (EED) is a rare disorder of chronic cutaneous vasculitis. Skin lesions consist of red or brownish plaques and nodules with a symmetrical distribution on the extensor surfaces of the extremities [1]. However, atypical locations have been reported, such as the penis [2], oral mucosa [3, 4], and cornea [5]. In this study, we report an EED patient with classic cutaneous lesions and their unusual distribution on the larynx and penile shaft.A 64-year-old man with recurrent painful [...]