Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus

Siavash Toosi, Jeffrey W. Collins, Christine M. Lohse, Michael M. Wolz, Carilyn N. Wieland, Michael J. Camilleri, Alison Bruce, Marian T. Mcevoy, Julia Lehman

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Abstract

Background: The pemphigus group is characterized by the presence of circulating immunoglobulins against desmosomes. IgG/IgA pemphigus is defined by the presence of IgG and IgA cell surface deposits upon direct immunofluorescence (DIF) and/or circulating IgG and IgA autoantibodies upon indirect immunofluorescence. Previous reports of patients with IgG/IgA pemphigus are sparse. Whether IgG/IgA pemphigus is best classified as a subtype of IgG (classic) pemphigus or IgA pemphigus, or as a distinct entity, has yet to be determined. Objectives: We compared the features of patients with IgG/IgA pemphigus to those of IgG pemphigus and IgA pemphigus. Methods: Retrospective clinicopathologic study of patients with IgG, IgG/IgA, and IgA pemphigus evaluated at our clinic (1993-2013). Results: We included 26, 13, and seven patients with IgG, IgG/IgA, and IgA pemphigus, respectively. Patients with IgG/IgA pemphigus did not differ significantly from patients with IgG pemphigus in terms of clinical and microscopic features, DIF findings, anti-desmoglein antibody values, and treatments required. However, patients with IgG/IgA pemphigus were significantly different from patients with IgA pemphigus regarding intertriginous distribution (P = 0.038) and pustular lesions (P < 0.001), acantholysis (P = 0.043), and presence of intercellular C3 deposits on DIF (P < 0.001). Conclusion: Comparative clinicopathologic data imply that IgG/IgA pemphigus may best be regarded as a variant of IgG pemphigus and distinct from IgA pemphigus.

Original languageEnglish (US)
Pages (from-to)e184-e190
JournalInternational Journal of Dermatology
Volume55
Issue number4
DOIs
StatePublished - Apr 1 2016

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Pemphigus
Immunoglobulin A
Immunoglobulin G
Direct Fluorescent Antibody Technique
Desmogleins
Acantholysis
Desmosomes

ASJC Scopus subject areas

  • Dermatology

Cite this

Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus. / Toosi, Siavash; Collins, Jeffrey W.; Lohse, Christine M.; Wolz, Michael M.; Wieland, Carilyn N.; Camilleri, Michael J.; Bruce, Alison; Mcevoy, Marian T.; Lehman, Julia.

In: International Journal of Dermatology, Vol. 55, No. 4, 01.04.2016, p. e184-e190.

Research output: Contribution to journalArticle

Toosi, S, Collins, JW, Lohse, CM, Wolz, MM, Wieland, CN, Camilleri, MJ, Bruce, A, Mcevoy, MT & Lehman, J 2016, 'Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus', International Journal of Dermatology, vol. 55, no. 4, pp. e184-e190. https://doi.org/10.1111/ijd.13025
Toosi, Siavash ; Collins, Jeffrey W. ; Lohse, Christine M. ; Wolz, Michael M. ; Wieland, Carilyn N. ; Camilleri, Michael J. ; Bruce, Alison ; Mcevoy, Marian T. ; Lehman, Julia. / Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus. In: International Journal of Dermatology. 2016 ; Vol. 55, No. 4. pp. e184-e190.
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abstract = "Background: The pemphigus group is characterized by the presence of circulating immunoglobulins against desmosomes. IgG/IgA pemphigus is defined by the presence of IgG and IgA cell surface deposits upon direct immunofluorescence (DIF) and/or circulating IgG and IgA autoantibodies upon indirect immunofluorescence. Previous reports of patients with IgG/IgA pemphigus are sparse. Whether IgG/IgA pemphigus is best classified as a subtype of IgG (classic) pemphigus or IgA pemphigus, or as a distinct entity, has yet to be determined. Objectives: We compared the features of patients with IgG/IgA pemphigus to those of IgG pemphigus and IgA pemphigus. Methods: Retrospective clinicopathologic study of patients with IgG, IgG/IgA, and IgA pemphigus evaluated at our clinic (1993-2013). Results: We included 26, 13, and seven patients with IgG, IgG/IgA, and IgA pemphigus, respectively. Patients with IgG/IgA pemphigus did not differ significantly from patients with IgG pemphigus in terms of clinical and microscopic features, DIF findings, anti-desmoglein antibody values, and treatments required. However, patients with IgG/IgA pemphigus were significantly different from patients with IgA pemphigus regarding intertriginous distribution (P = 0.038) and pustular lesions (P < 0.001), acantholysis (P = 0.043), and presence of intercellular C3 deposits on DIF (P < 0.001). Conclusion: Comparative clinicopathologic data imply that IgG/IgA pemphigus may best be regarded as a variant of IgG pemphigus and distinct from IgA pemphigus.",
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T1 - Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus

AU - Toosi, Siavash

AU - Collins, Jeffrey W.

AU - Lohse, Christine M.

AU - Wolz, Michael M.

AU - Wieland, Carilyn N.

AU - Camilleri, Michael J.

AU - Bruce, Alison

AU - Mcevoy, Marian T.

AU - Lehman, Julia

PY - 2016/4/1

Y1 - 2016/4/1

N2 - Background: The pemphigus group is characterized by the presence of circulating immunoglobulins against desmosomes. IgG/IgA pemphigus is defined by the presence of IgG and IgA cell surface deposits upon direct immunofluorescence (DIF) and/or circulating IgG and IgA autoantibodies upon indirect immunofluorescence. Previous reports of patients with IgG/IgA pemphigus are sparse. Whether IgG/IgA pemphigus is best classified as a subtype of IgG (classic) pemphigus or IgA pemphigus, or as a distinct entity, has yet to be determined. Objectives: We compared the features of patients with IgG/IgA pemphigus to those of IgG pemphigus and IgA pemphigus. Methods: Retrospective clinicopathologic study of patients with IgG, IgG/IgA, and IgA pemphigus evaluated at our clinic (1993-2013). Results: We included 26, 13, and seven patients with IgG, IgG/IgA, and IgA pemphigus, respectively. Patients with IgG/IgA pemphigus did not differ significantly from patients with IgG pemphigus in terms of clinical and microscopic features, DIF findings, anti-desmoglein antibody values, and treatments required. However, patients with IgG/IgA pemphigus were significantly different from patients with IgA pemphigus regarding intertriginous distribution (P = 0.038) and pustular lesions (P < 0.001), acantholysis (P = 0.043), and presence of intercellular C3 deposits on DIF (P < 0.001). Conclusion: Comparative clinicopathologic data imply that IgG/IgA pemphigus may best be regarded as a variant of IgG pemphigus and distinct from IgA pemphigus.

AB - Background: The pemphigus group is characterized by the presence of circulating immunoglobulins against desmosomes. IgG/IgA pemphigus is defined by the presence of IgG and IgA cell surface deposits upon direct immunofluorescence (DIF) and/or circulating IgG and IgA autoantibodies upon indirect immunofluorescence. Previous reports of patients with IgG/IgA pemphigus are sparse. Whether IgG/IgA pemphigus is best classified as a subtype of IgG (classic) pemphigus or IgA pemphigus, or as a distinct entity, has yet to be determined. Objectives: We compared the features of patients with IgG/IgA pemphigus to those of IgG pemphigus and IgA pemphigus. Methods: Retrospective clinicopathologic study of patients with IgG, IgG/IgA, and IgA pemphigus evaluated at our clinic (1993-2013). Results: We included 26, 13, and seven patients with IgG, IgG/IgA, and IgA pemphigus, respectively. Patients with IgG/IgA pemphigus did not differ significantly from patients with IgG pemphigus in terms of clinical and microscopic features, DIF findings, anti-desmoglein antibody values, and treatments required. However, patients with IgG/IgA pemphigus were significantly different from patients with IgA pemphigus regarding intertriginous distribution (P = 0.038) and pustular lesions (P < 0.001), acantholysis (P = 0.043), and presence of intercellular C3 deposits on DIF (P < 0.001). Conclusion: Comparative clinicopathologic data imply that IgG/IgA pemphigus may best be regarded as a variant of IgG pemphigus and distinct from IgA pemphigus.

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