A case of rapidly progressive, fatal mycosis fungoides presenting as a haematoma-like lesion.

Mycosis fungoides usually follows an indolent clinical course. We report here a rapidly progressive case of mycosis fungoides with peculiar clinical and histological features, presenting as a haematoma-like mass on the thigh accompanied by multiple reddish brown erythematous lesions on the trunk and extremities. Histopathologically, the erythematous lesions showed epidermotropism of atypical T lymphocytes expressing CD4 and prominent syringotropism without syringometaplasia. The haematoma-like lesion consisted of diffuse and dense infiltrates of medium-to-large-sized pleomorphic lymphocytic cells expressing CD30, suggesting that CD30+ large-cell transformation had occurred. Mycosis fungoides presenting as a haematoma-like lesion is rare and may be a poor prognostic sign.

COVID-19 mRNA Vaccine-induced Pneumonitis.

A 65-year-old man experienced cough and shortness of breath 3 days after receiving the first dose of the Pfizer-BioNTech coronavirus disease 2019 (COVID-19) vaccine. Chest X-ray revealed bilateral infiltrates, and the desaturation deteriorated rapidly. The symptoms and radiographic abnormalities rapidly improved after the initiation of corticosteroid therapy. Intradermal testing of the Pfizer-BioNTech COVID-19 vaccine showed a delayed positive reaction. Based on these findings, the patient was diagnosed with COVID-19 vaccine-induced pneumonitis. The timing of the onset of pneumonitis after vaccination and the results of intradermal testing suggest that Type IV hypersensitivity against COVID-19 vaccine may have been responsible for this clinical condition.

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.

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

BACKGROUND: Patients with steroid-resistant bullous pemphigoid (BP) require an appropriate treatment option. OBJECTIVE: A 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. METHODS: We 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. RESULTS: We 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. CONCLUSION: IVIG provides a beneficial therapeutic outcome for patients with BP who are resistant to steroid therapy.

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.

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., 62.5-125 mg daily. The mean duration from the diagnosis of SSc to the initiation of bosentan was 9.5 years, and the observation period after bosentan administration was from 7 months to 4.5 years. In case 1, neither new digital ulcers nor Raynaud's phenomenon developed for 4.5 years. 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.

Isolated cutaneous manifestation of IgG4-related disease.

AIMS: The cutaneous manifestation of IgG(4)-related disease has rarely been reported. The aim of this study is to identify and describe the cutaneous manifestations associated with IgG(4)-positive plasma cell infiltration in the skin. METHODS: The authors investigated two cases of IgG(4)-related disease with solitary skin lesions and compared the immunohistochemical characteristics of infiltrating cells among IgG(4)-related disease, Kimura's disease and cutaneous pseudolymphoma. RESULTS: IgG(4)-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 IgG(4)-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-IgG(4) antibodies, and the ratio of IgG(4)+/IgG+ cells was more than 50%. Serum levels of IgG and IgG(4) were not elevated and no lesions were found in other organs. Skin samples taken from Kimura's disease showed histopathological features similar to those of IgG(4)-related disease. The proportion of IgG(4)+/IgG+ was high in Kimura's disease, but not in cutaneous pseudolymphoma. CONCLUSIONS: The solitary skin lesions of IgG(4)-related disease were similar histologically and immunohistochemically to the skin lesions of Kimura's disease. The concept of IgG(4)-related disease may help clarify the pathomechanism of diseases of unknown aetiology that possess features of IgG(4)-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 3-22 years 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.