Radiation-Associated Pemphigus Vulgaris in a Patient With Preceding Malignancy: Treatment With Rituximab as a Valuable Option

Abstract

Pemphigus is a chronic autoimmune blistering disorder, characterized by (muco-)cutaneous erosions due to autoantibodies against desmoglein 3 and/or 1. Pemphigus induction might be associated with drugs, malignancy or radiation therapy (RT); the latter being only rarely described. A rigorous literature review revealed around 30 cases of RT-associated pemphigus, which had been primarily treated with topical and/or systemic steroids, in some cases also dapsone or few other immunosuppressive agents were given. The most common underlying cancer type was breast cancer. We here present a 63-year-old male patient, who was pre-treated with adjuvant RT for larynx carcinoma 3 months before admission. He developed extensive cutaneous, ocular, and oral erosions. Despite the clinical picture comparable to a paraneoplastic pemphigus, the diagnosis of pemphigus vulgaris of mucocutaneous type was established based on the direct immunofluorescence, showing positive cell surface IgG and discrete C3 deposits, with matching cell surface IgG pattern on monkey esophagus. Serum autoantibodies to desmoglein 1 and 3 were highly positive. No further autoantibodies were found, thus paraneoplastic pemphigus was excluded. The patient was treated with high dose prednisolone, partially given intravenously up to 2 mg/kg per day, as well as topical disinfectants and class IV steroid cream. To stabilize the disease rituximab 2 × 1,000 mg was given, leading to clinical and serological remission for up to 2 years now. We show that rituximab represents a good treatment option for the frequently treatment-refractory RT-associated pemphigus, a clinically and immunologically specific RT-induced skin disorder, resulting in long-term clinical, and serological remission.

Localized pemphigus exacerbation associated with underlying breast cancer

“…In the literature, pemphigus occurrence in patients with underlying breast cancer has been reported as a particularly rare association, with most of the published cases occurring after radiation therapy. 4 Here, we describe a patient with a history of oral pemphigus vulgaris (PV) experiencing a severe disease flare predominantly affecting her right breast skin in the setting of an underlying ductal carcinoma.…”

Gefitinib induced Pemphigus Vulgaris with PRIDE complex

“…11,12 In the case of our patient, a histopathological examination and direct immunofluorescence test were done, suggestive of drug-induced papulopustular dermatoses and pemphigus Vulgaris.Our patient showed a significant extent of severity of pemphigus (total activity score: 24) according to the PDAI scoring pattern as total activity score depends on the sum of total skin plus total scalp plus total mucosa (Table 1). 13 Our case was assessed as probable case using Naranjo adverse drug reaction probability scale which classify adverse drug reaction as definite (≥9), probable (5)(6)(7)(8), possible (1)(2)(3)(4) and doubtful (0) case. 14 PRIDE complex can be managed symptomatically.…”

“…2 About 5-11% of Pemphigus is related to malignancy. 4 Pemphigus Vulgaris is a rare mucocutaneous life-threatening disease usually characterized by painful blisters that initially starts from the mouth/oral cavity later involve other parts of the body and are not associated with itching and scar recovery. 1,2 Pemphigus Vulgaris has a higher incidence rate among the drug-induced Pemphigus, i.e., 0.1-0.5/100000 people/ year.…”

Recurrence of Pemphigus Vulgaris after Bilateral Breast Irradiation: A Case Report and Review of the Literature

“…In the light of these cases, we highlight a tendency toward the severity of these lesions with resistance to oral and general corticosteroid treatments in more than half of the cases (56%) as well as a tendency for the lesion progression in nonirradiated areas. Schauer et al [ 10 ] had indeed shown that for one-third of documented cases of radio-induced pemphigus, it is necessary to add an immunosuppressant to control the disease. In 3 cases, rituximab, an anti-CD20 monoclonal antibody, had been used for rapid and complete lesion remission.…”