A recent case report described an inflammatory variant of EBA with lesions on the trunk, the hands and the oral mucosa. The patient received four courses of rituximab
Fig. 8.5. Clinical response in a patient with mucosal pemphigus vulgaris (PV) to treatment with rituximab. The PV patient with extensive oral erosions shown here was refractory to immunosuppressive treatment including mycopheno-late mofetil (3 g/day) and azathioprine (1.75 mg/kg/ day), respectively, in combination with methylpredniso-lone (0.5 -1.5 mg/kg/day) for
6 months (A, before rituximab treatment). Rituximab treatment led to a rapid clinical response (B, 2 months after rituximab treatment)
Fig. 8.6. Clinical response of mechanobullous epidermolysis bullosa acquisita to rituximab. Patient 1 showed blisters and erosions on his hands (a, left), feet and the oral mucosa before rituxi-mab treatment. Marked improvement of the lesions on the hands was noted 15 weeks after completion of therapy (a, right), while erosions of the oral mucosa remained largely unaffected. Patient 2 (b, left) showed extensive blisters and erosions of the trunk and hands. Nine months after completion of rituximab therapy, an almost complete clinical remission with post-inflammatory atrophic hyperpigmentations on the chest and a few crusty erosions was visible (b, right)
(i.v., 375 mg/m2) as an adjuvant treatment in weekly intervals. Seven weeks after the completion of rituximab therapy, the patient was in complete remission and received tapering doses of azathioprine (175 mg/ day), colchicine (160 mg/day) and prednisolone (80 mg/day). As an unusual adverse event a deep venous thrombosis of the lower leg developed between the first and second infusion of rituximab.
In contrast, administration of rituximab in patients with mechanobullous EBA shows a different clinical response. In our own experience, an EBA patient with erosive lesions of the oral mucosa, the esophagus and the nasopharynx and tense blisters on the hands, lower legs and feet showed only a partial clinical response to rituximab. Subsequently, he received adjuvant therapy with rituximab 375 mg/m2 x4 over a period of 4 weeks. Concomitant medication with mycophenolate mofetil 3 g/day was maintained during and after B-cell depletion therapy. Within 15 weeks gradual improvement of the lesions on the hands was perceived, whereas the oral lesions and the lesions on the soles showed only slight improvement (Fig. 8.6). A second patient with mechanobullous EBA refractory to treatment to a variety of systemic immunosuppressive drugs in the past, who had lesions that mainly affected the trunk and the hands, was treated in the same way as the previous patient with rituximab. Within 8 months his disease was well controlled on monotherapy with rituximab with only very few residual crusty erosions and atro-phic lesions. The excellent clinical response was accom panied by a decline in titres of circulating autoanti-bodies, which were undetectable > 9 months after completion of rituximab therapy. Complete B-cell depletion persisted for > 9 months. In both patients rituximab was well tolerated with no side effects.
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