title: Pemphigus with features of both vulgaris and foliaceus variants localized to the nose.
abstract: We report the case of a 74-year-old man affected by an unusual variant of pemphigus. He presented with a crusty and scaly lesion of the nose. We performed reflectance confocal microscopy and optical coherence tomography on the lesion, which suggested an unexpected diagnosis of pemphigus. Therefore, to confirm our diagnostic suspicions, we executed indirect immunofluorescence and two biopsies, one for histopathological examination and one for direct immunofluorescence. Histopathological evaluation showed acantholysis with formation of clefts in the granular and spinous layers of the epidermis. Direct immunofluorescence revealed immunoglobulin G and C3 deposit to the full thickness of the epidermis. Indirect immunofluorescence showed intercellular antibodies at a titer of 1:40 in the suprabasal epidermis. The immunoblot analysis using epidermal extract revealed the presence of circulating antibodies directed to 130- and 160-kDa antigens in the patient's serum. These two antigens were evidenced from nitrocellulose membrane with colorimetric AP systems, which highlighted the presence of autoantibodies against desmoglein (Dsg)1 and Dsg3 (sodium dodecylsulfate polyacrylamide gel electrophoresis). We also performed an enzyme-linked immunoassay. All these findings suggested that this patient's pemphigus had features of both vulgaris and foliaceus variants.
authors.affiliation: Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Victor Desmond
authors.initials: VD
authors.lastname: Mandel
authors.affiliation: Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Farnetani
authors.affiliation: Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Vaschieri
authors.affiliation: Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Manfredini
authors.affiliation: Department of Anatomic Pathology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Cesinaro
authors.affiliation: Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Giusti
authors.affiliation: Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Pellacani
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood; D051183:Desmoglein 1 / Q000276:immunology; D051184:Desmoglein 3 / Q000276:immunology; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008297:Male; D018613:Microscopy, Confocal; D009666:Nose / Q000473:pathology; D010392:Pemphigus / Q000000981:diagnostic imaging* / Q000276:immunology / Q000473:pathology; D041623:Tomography, Optical Coherence
chemical_list: D001323:Autoantibodies; C495297:DSG1 protein, human; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3
keywords: autoimmune disease; optical coherence tomography; pemphigus foliaceus; pemphigus vulgaris; reflectance confocal microscopy