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Successful use of rituximab, an anti‐CD20 monoclonal antibody, to treat IgA nephropathy in a patient with recessive dystrophic epidermolysis bullosa

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lesions are caused by alterations of the immune system caused by the systemic diseases with which the disorder is associated. These alterations trigger an excessive inflammatory reaction in response to… Click to show full abstract

lesions are caused by alterations of the immune system caused by the systemic diseases with which the disorder is associated. These alterations trigger an excessive inflammatory reaction in response to bacterial infections or other external triggers, although cases in healthy individuals have also been reported. The differential diagnoses should include pemphigus vegetans. A positive immunofluorescence result was previously considered to be diagnostic of pemphigus vegetans; however, cases of PSV-PDV with positive immunofluorescence, characterized by more frequent IgA granular deposits, have been published recently, similar to our first patient. For both of our patients, the diagnosis of pemphigus vegetans was ruled out by the absence of IgG and C3 deposits in the basal membrane. To our knowledge, this is the first report of PSV-PDV associated with recreational drugs; however, we have identified two previous clinical cases, both of which were diagnosed as pemphigus vegetans with DIF results consisting of intercellular deposits of IgG and/or C3, but both had similar clinical findings to those of our patients. As both of these cases were reported many years ago and immunohistochemical techniques have since improved, it is possible that those patients may also have had PSVPDV, as DIF can sometimes be positive in that setting. Treatment of PSV-PDV includes control of any concomitant systemic disease if present, and cessation of drug use, which has given good results in cases of pemphigus associated with substance abuse. Administration of systemic corticosteroids and antibiotics has also been shown to be effective. We treated our patients with a 2-month course of combination therapy of doxycycline and rifampicin, along with corticosteroids at a dosage of 0.5 mg/ kg/day for 2 weeks, tapering off over the following 2 months, which resulted in improvement of the lesions. Both patients were also offered support by the addiction treatment unit, but were lost to follow-up.

Keywords: pemphigus vegetans; psv pdv; patient; use rituximab; successful use

Journal Title: Clinical and Experimental Dermatology
Year Published: 2022

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