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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 2016 June;151(3):296-9

Copyright © 2016 EDIZIONI MINERVA MEDICA

language: English

Purely cutaneous sclerosing IgG4-related disease of the cephalic region: case report and a mini-review of the clinical and pathological aspects

Luca MUSCARDIN 1, Giovanni PAOLINO 2, Chiara PANETTA 1, Pietro DONATI 1

1 Dermatopathological Laboratory, Institute “San Gallicano Institute of Rome”, Rome, Italy; 2 Dermatological Clinic Department of Internal Medicine and Medical Specialties, Sapienza University, Rome, Italy


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IgG4-related disease is a recently defined emerging entity. Many different organs may be affected by this disease: pancreas, salivary and lacrimal glands, liver, peritoneum and lung. Also the skin may be affected, as secondary localization, while as primary cutaneous localization it has been rarely described. A male patient presented at our Institute with a two-year history of sclerosing erythematous nodules of the scalp. Histological examination showed a T-lymphocyte (CD3+) infiltrate with interspersed plasmacytoid cells and the interposition of a fibrosclerotic tissue. We found numerous IgG4+ cells at the periphery of the nodular structures, while the serum levels of IgG4 and the remaining blood chemistry analysis were normal. Only a few cases of primitive cutaneous pseudo-lymphomatous IgG4-related disease have been described in the literature. Our case showed the same clinical and histologic features of those previously described; the etiology of IgG4-related diseases remains to be elucidated.

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paolgio@libero.it