By Lawrence S Chan
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Additional resources for Blistering Skin Diseases
For PNP associated with B-cell neoplasm, the anti-B-cell (CD20) 46 Indirect immunofluorescence microscopy using serum from a patient with paraneoplastic pemphigus, on rat bladder transitional epithelium substrate, reveals circulating IgG autoantibodies binding to the epithelial cell surfaces located on the bladder lumen. humanized mouse antibody, rituximab, seems to be the right choice and indeed has induced clinical resolution in two such PNP patients but not in a third . The mucosal lesions are particularly resistant to therapy, even if the primary neoplasm is removed.
In some patients, lesions on the extremities are targetoid configuration with central blister formation, resembling that of bullous pemphigoid or erythema multiforme. In addition, erythema on the V-shaped area of the upper chest is commonly observed. Some lesions are in lichenoid pattern (41), which can be the initial or sole clinical presentation [109; 115]. The occurrence of lichenoid lesions on palms and soles is a unique finding, distinct from the classic pemphigus vulgaris. In some patients, painful paronychial ulcerative lesions are observed (42).
Western blot analysis detects circulating IgG autoantibodies from patients’ sera reacting to a new epitope (MCW-4) within the NC16A domain of BP180 . Therapeutic strategy In patients with mild disease, a potent topical steroid could be used as the initial treatment option. Alternatively, tetracycline in combination with nicotinamide could be tried. For patients with moderate to severe disease, low-dose systemic steroid (prednisone, ≤40 mg/day), with or without immunosuppressive (azathioprine 100 mg/day) therapy, could be used.
Blistering Skin Diseases by Lawrence S Chan