Tumor necrosis factor-alpha (TNF-alpha) antagonists are effective for inflammatory diseases, such as Crohn's disease, rheumatoid arthritis (RA) and psoriasis. Although TNF-alpha antagonists are also useful for sarcoidosis, paradoxical occurrence of sarcoidosis or sarcoidal reaction may be observed. We report a Crohn's disease patient, who developed sarcoidosis during infliximab therapy. A 35-year-old man had been receiving infliximab for 7 months for Crohn's disease. He developed cough and fever, accompanied by an infiltrated erythematous plaque on his right knee. The chest radiography, skin biopsy and laboratory findings were all consistent with sarcoidosis.
Pemphigus vulgaris (PV) is an autoimmune bullous disease characterized by autoantibodies against desmogleins. We report a case of recalcitrant PV, which progressed from the mucosal to the mucocutaneous type, with a corresponding increase in anti-desmoglein (Dsg)1 and decrease in anti-Dsg3 antibody titres. Thus, the clinical features seemed to correlate with the ratio of anti-Dsg1 and 3. The patient also had anti-Dsg4 antibodies, which might be related to the nonscarring diffuse hair loss and marked facial involvement she also had. The patient did not respond to treatment with systemic steroid, ciclosporin, azathioprine, cyclophosphamide or double filtration plasmapheresis, and eventually died from fulminant thrombotic thrombocytopenic purpura of unknown cause.
S100-negative, CD1a-positive histiocytosis is a rare histiocytic disorder characterized by proliferation of histiocytic cells possessing a phenotype of epidermal Langerhans cells except for the lack of S100 expression and Birbeck granules. We report the case of a Japanese man suffering from S100-negative, CD1a-positive histiocytosis. The patient showed numerous smooth erythematous 5-10-mm papules/nodules on most of his body. The key histopathological feature was the presence of dermal infiltrates of non-epidermotropic S100-negative CD1a-positive mononuclear cells. No systemic involvement was detected. Initially bath-psoralen plus ultraviolet A therapy was effective, but the lesions became recalcitrant to this treatment. Methylprednisolone pulse therapy followed by low-dose methotrexate (up to 30 mg/week) in combination with prednisolone (15 mg/day) effectively controlled the skin lesions.
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