PMC6985440_01
Clinical pictures of the patient at initial presentation and after treatment. Extensive erosive skin detachment of the frontal trunk and neck (A), right axillary fold (B), and shoulders and neck (C). Three months after systemic treatment with rituximab and ongoing low dose glucocorticosteroids, intact skin with postinflammatory hyperpigmentations was observed (D).
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F1
We present a 63-year-old male patient, who was referred to our department due to extensive cutaneous (Figures 1A-C) and mucosal erosions (eyes, nose, lips, mouth, and esophagus). Currently, the follow-up-time is two and a half years (Figure 1D), showing that rituximab is a valuable treatment for this rare pemphigus subtype; it improved the patient's quality of life tremendously.
Immunological characterization of the patient. (A) The direct immunofluorescence (DIF) shows cell surface IgG deposition on the keratinocytes of the entire epidermis. (B) Indirect immunofluorescence (IIF) with monkey esophagus (ME) shows circulating cell surface IgG autoantibodies. (C) Immunoblotting with normal human epidermal extracts shows IgG antibodies reactive with 130-kDa Dsg3 (lane 4).
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F2
IgG (Figure 2A) and C3 in the entire epidermis. IIF with normal human skin and monkey esophagus (Figure 2B) revealed circulating cell surface IgG autoantibodies at a titer of 1:160. Immunoblotting of normal human epidermal extracts demonstrated IgG autoantibodies against the 130-kDa Dsg3 (Figure 2C, lane 4), but no reactivity with Dsg1, envoplakin, periplakin, BP230, BP180, LAD-1 antigen, collagen VII, or laminin-332.