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SHORT COMMUNICATION
Extensive Nasal Plaque: An Unusual Presentation of IgG4-related Disease
Camila CORTÉS 1 , Silvia BIELSA 2 , Josep Manel FERNÁNDEZ 1 , Felip VILARDELL 3 , Blanca GONZALEZ-FARRÉ 4 and Josep
Manel CASANOVA 1
Departments of 1 Dermatology, 2 Internal Medicine and 3 Pathology, University Hospital Arnau de Vilanova, ES-25198 Lleida, and 4 Department
of Pathology, Hospital Clinic, Barcelona, Spain. E-mail: [email protected]
Accepted Mar 1, 2018; Epub ahead of print Mar 2, 2018
IgG4-related disease (IgG4-RD) is a fibro-inflammatory
condition characterized mainly by tissue infiltration by
IgG4 + plasma cells with a tendency to mass forming.
IgG4-RD can involve any part of the body, including the
skin. We report here a rare case of cutaneous IgG4-RD pre-
senting with a nasal infiltrative lesion. To our knowledge,
this is the first report of this rare clinical presentation.
CASE REPORT
A 67-year-old man with no relevant clinical history presented with
an erythematous asymptomatic plaque in the nasal ala, which had
appeared 6 months earlier. The lesion spread progressively to the
entire nasal pyramid and left infraorbital region (Fig. 1a). He had
no systemic symptoms. Blood count, biochemical profile and
haemostasis were normal, and bacterial, fungal and mycobacterial
cultures were unremarkable. Computed tomography (CT) scan
and magnetic resonance imaging (MRI) showed a large infiltrative
lesion affecting cutaneous and subcutaneous tissue in both nasal
alae (Fig. S1a 1 ). Positron emission tomography (PET) scanning
confirmed the findings (Fig. S1b 1 ). Thoraco-abdominal CT scan
was performed and no relevant anomalies were detected. Serum
protein analysis showed increased levels of gamma-globulins with
total IgG levels of 19.2 g/l (normal 7.5–16 g/l) and IgG4 3.8 g/l
(0.08–1.4 g/l). Serological tests for human immunodeficiency virus
(HIV), hepatitis B virus (HBV), hepatitis C virus (HCV), and anti-
neutrophil cytoplasmic antibodies (ANCA) were negative. Biopsy
of the lesion revealed a dense dermic lymphoplasmacytic infiltrate
with a background of neutrophils, eosinophils and histiocytes with
prominent storiform fibrotic stroma (Fig. 2a). Focal images of
lymphocytic venulitis without necrosis were also observed (Fig.
2b). Immunohistochemical analysis highlighted
a large number of IgG4 + plasma cells (Fig. 2c).
IgG4 + plasma cells were present at approximately
60–140 per high-power field (Fig. 2d). The ratio of
IgG4 + /IgG plasma cells ranged from 30% to 70%.
Epstein-Barr virus hybridization was negative and
clonal rearrangements in the T-cell receptor were
not detected. Based on these findin