Localized pemphigus exacerbation associated with underlying
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Pemphigus is a rare, autoantibody-mediated, mucocutaneous disease characterized by loss of the adhesion between keratinocytes and intraepidermal blistering.

A 54-year-old woman presented with a 3-month history of a skin rash localized to her right breast. Six months before presentation, she was diagnosed with oral pemphigus vulgaris (PV). An enzyme- linked immunosorbent assay at this time point showed elevation of both anti-Desmoglein(Dsg)3 (150 UI/mL) and Dsg1 (100 UI/mL) IgG antibodies. The disease was managed with a short-course of oral and topical corticosteroids, with complete remission on low dose systemic corticosteroids (prednisone 7.5 mg/day) without the need of other immunosuppressive medications.

She was suffering from a major depressive disorder, for which she was on treatment with trazodone, sertraline, lamotrigine, and duloxetine. Physical examination showed a significant retraction of the right breast and nipple; initial hardening and retraction of her right breast had appeared since about 18 months, but the patient did not consult her physician until the manifestation of the skin rash. The skin overlying her right breast was covered with multiple confluent erosions, hyperkeratotic scales and crusts. The morphological anatomy and the skin of the contralateral breast appeared normal. Some erythematous-scaling plaques were also noted across the back.

Histopathology examination obtained from an erosion of the right breast’s skin showed suprabasal epidermal acantholysis. Direct immunofluorescence (IIF) from the perilesional skin showed intercellular deposition of IgG and C3 in the epidermis, while ELISA showed high level of IgG autoantibodies against Dsg1 (101.3 UI/mL) and Dsg3 (148.8 UI/mL). The above findings were consistent with a relapse of her PV.

A CT scan and a subsequent breast biopsy confirmed the presence of an invasive triple negative ductal carcinoma. Surgical removal of the tumor resulted in a marked improvement of the pemphigus flare, with complete resolution of the lesions on the breast skin and persistence of a few residual lesions on the trunk, which did not require an increase in her daily prednisone dose.

This case provides further evidence for the pathogenetic link between pemphigus and solid tumors. Clinicians should be aware about the possibility of underlying malignancies in
pemphigus patients experiencing localized flares.

Source: https://www.jaadcasereports.org/article/S2352-5126(20)30752-9/fulltext?rss=yes
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