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. 2021 Aug 5;15:129–132. doi: 10.1016/j.jdcr.2021.07.026

Table I.

Previously reported cases of pemphigus primarily localized to the nasal region

Reference Age (y), sex Pemphigus type Remained localized Antibodies Treatment
Paramsothy15 34, M PF No DIF: IgG deposition between epidermal cells
IIF: Positive
Oral prednisone
Paramsothy15 65, M PF No DIF: IgG between epidermal cells and IgM in basement membrane
IIIF: Positive
Topical clobetasol propionate
Newton et al13 27, M PF Yes DIF: Intercellular IgG and C3 Topical clobetasol propionate
Newton et al13 62, F PF No DIF: Intercellular IgG Oral cyclophosphamide
Newton et al13 43, M PF Yes DIF: None Oral prednisolone, 20 mg/d
Kishibe16 63, M PF Yes DIF: IgG deposition Oral prednisolone 40 mg/d
Ghoneim and Zaiac11 37, M PF Yes DIF: Intercellular IgG and C3 throughout epidermis Triamcinolone ointment and one-time intralesional injection of triamcinolone 2.5 mg/mL
Fleming17
51, M PF Yes DIF: Intercellular IgG and C3 Topical steroids (unspecified) and oral prednisolone
Mandel et al9 74, M PF/PV Yes Histopathology: Acantholysis with formation of clefts in the granular and spinous layers of the epidermis
DIF: IgG and C3 throughout epidermis
IIF: Intracellular antibodies (titer, 1:40) in suprabasal epidermis
Oral prednisone 25 mg/d for 1 mo
Aizawa18 M PF/PV Unknown Histopathology: Subcorneal acantholysis
DIF: None
IIF: IgG deposits on keratinocyte cell surface
CLIA: Anti-DSG-1 antibodies (391 U/mL); anti-DSG-3 antibodies (23.5 U/mL; normal level, <20 U/mL for both)
ELISA: Negative result for desmocollins 1-3
Topical corticosteroids (unspecified)
Zhang19 36, M PV Yes DIF: IgG and C3 in a patchy net-like intercellular pattern in the epidermis
IIF: Monkey esophagus, negative
ELISA: Anti-DSG-3 antibodies (89.6 U/mL, normal < 20); negative result for anti-DSG-1 antibody
Mometasone furoate cream 1%
Sigmund20 43, F PV, vegetans type Yes Histopathology: Mixed inflammatory cell infiltrate that included a large number of eosinophils and plasma cells; pseudoepitheliomatous hyperplasia; intraepidermoid microabscesses; eosinophilic spongiosis Prednisone 60 mg daily for 2 wk and maintained on prednisone 30 mg daily
Taylor21 65, M PV No Topical and intralesional steroids

CLIA, Chemiluminescent immunoassay; DIF, direct immunofluorescence; DSG, desmoglein; ELISA, enzyme-linked immunosorbent assay; Ig, immunoglobulin; IIF, indirect immunofluorescence; PF, pemphigus foliaceus; PV, pemphigus vulgaris.