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.

Pemphigus with features of both vulgaris and foliaceus variants localized to the nose / Mandel, Victor Desmond; Farnetani, Francesca; Vaschieri, Cristina; Manfredini, Marco; Cesinaro, Anna Maria; Giusti, Francesca; Pellacani, Giovanni. - In: THE JOURNAL OF DERMATOLOGY. - ISSN 1346-8138. - 43:8(2016), pp. 940-943. [10.1111/1346-8138.13314]

Pemphigus with features of both vulgaris and foliaceus variants localized to the nose

PELLACANI, Giovanni
2016

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.
2016
Autoimmune disease; Optical coherence tomography; Pemphigus foliaceus; Pemphigus vulgaris; Reflectance confocal microscopy.
01 Pubblicazione su rivista::01a Articolo in rivista
Pemphigus with features of both vulgaris and foliaceus variants localized to the nose / Mandel, Victor Desmond; Farnetani, Francesca; Vaschieri, Cristina; Manfredini, Marco; Cesinaro, Anna Maria; Giusti, Francesca; Pellacani, Giovanni. - In: THE JOURNAL OF DERMATOLOGY. - ISSN 1346-8138. - 43:8(2016), pp. 940-943. [10.1111/1346-8138.13314]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1483641
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