We present a case of a 49-year-old Japanese woman with recalcitrant pemphigus vulgaris associated with multiple severe infections and very high anti-desmoglein 1 and 3 antibody titers. The lesions covered more than 70% of her body surface area. A combination of repeated steroid pulse therapy, plasmapheresis, and IVIG could not control the disease. However, a single cycle of rituximab therapy led to complete remission for 18 months, during her steroid dose was tapered. No adverse effects have been observed till date. Rituximab is a chimeric murine?human monoclonal antibody that targets the CD20 antigen found on B cells andcauses rapid depletion of this cell population. It has been approved for the treatment of non-Hodgkin B-cell lymphoma and rheumatoid arthritis in the United States. In addition, rituximab has been shown to be effective for the treatment of other autoimmune diseases such as systemic lupus erythematosus and pemphigus. This treatment should be considered for recalcitrant cases with persistent high titers of autoimmune antibodies despite combination therapy with corticosteroids, immunosuppressive agents, plasmapheresis, and IVIG. It can also be considered for cases of recurrence due to tapering of steroids and immunosuppressive agents. However, the adverse effects of immunosuppression by rituximab should be carefully considered for each individual patient.
A 67-year-old woman was referred to us with symmetrically distributed keratotic eruptions on the ulnar aspects of her thumbs and the radial aspects of her index and middle fingers. Histopathologically, we observed parakeratosis, loss of granular layer and liquefaction degeneration of basal layer around the top of the papillary dermis; in contrast, compact orthokeratosis and intact dermo-epidermal junction were observed at the epidermis above the crista profunda limitans. There were vertical columns of parakeratosis and orthokeratosis arranged in an alternate arrangement pattern. Dermoscopy showed a whitish, lace-like, double-line pattern along each side of the furrows. This dermoscopic pattern seems to correspond histopathologically to the regular distribution of parakeratosis above the papillary dermis and compact orthkeratosis above the crista profunda limitans. Although a clinical differential diagnosis of mechanic’s hands from other dermatoses may be difficult, dermoscopy may be a useful tool for distinguishing them.
We examined the clinical characteristics of 23 patients with mixed connective tissue disease, (MCTD) mainly focusing on the cutaneous manifestations seen in our department. The mean age at onset was 29±9.9 years, and the female to male ratio was approximately 10 : 1 (21 : 2). Raynaud’s phenomenon (22 out of 23 patients, 95.6%) and puffy fingers (18 out of 23 patients, 78.2%) were the common clinical manifestations in MCTD. Acrocyanosis (52.1%), livedo reticularis (34.7%), and macular telangiectasis (30.4%) were also common findings. In this study, the initial manifestations of 6 cases of MCTD were self-limiting fever associated with lymph node swellings and cutaneous symptoms such as exudative erythema and urticarial erythema. Histological examinations revealed a resemblance to interstitial granulomatous dermatitis (Ackerman syndrome). From our disease findings, we concluded that this symptom is one of the clinical characteristics associated with MCTD. We examined the association between cutaneous manifestations and complications; we observed an association between macular telangiectasis and interstitial pneumonia, so we speculated that this correlation reflected a symptom of the systemic scleroderma.