Publication

Nonbullous pemphigoid: Insights in clinical and diagnostic findings, treatment responses, and prognosis

Lamberts, A., Meijer, J. M., Pas, H. H., Diercks, G. F. H., Horvath, B. & Jonkman, M. F., Aug-2019, In : Journal of the American Academy of Dermatology. 81, 2, p. 355-363 9 p.

Research output: Contribution to journalArticleAcademicpeer-review

APA

Lamberts, A., Meijer, J. M., Pas, H. H., Diercks, G. F. H., Horvath, B., & Jonkman, M. F. (2019). Nonbullous pemphigoid: Insights in clinical and diagnostic findings, treatment responses, and prognosis. Journal of the American Academy of Dermatology, 81(2), 355-363. https://doi.org/10.1016/j.jaad.2019.04.029

Author

Lamberts, Aniek ; Meijer, Joost M. ; Pas, Hendri H. ; Diercks, Gilles F. H. ; Horvath, Barbara ; Jonkman, Marcel F. / Nonbullous pemphigoid : Insights in clinical and diagnostic findings, treatment responses, and prognosis. In: Journal of the American Academy of Dermatology. 2019 ; Vol. 81, No. 2. pp. 355-363.

Harvard

Lamberts, A, Meijer, JM, Pas, HH, Diercks, GFH, Horvath, B & Jonkman, MF 2019, 'Nonbullous pemphigoid: Insights in clinical and diagnostic findings, treatment responses, and prognosis', Journal of the American Academy of Dermatology, vol. 81, no. 2, pp. 355-363. https://doi.org/10.1016/j.jaad.2019.04.029

Standard

Nonbullous pemphigoid : Insights in clinical and diagnostic findings, treatment responses, and prognosis. / Lamberts, Aniek; Meijer, Joost M.; Pas, Hendri H.; Diercks, Gilles F. H.; Horvath, Barbara; Jonkman, Marcel F.

In: Journal of the American Academy of Dermatology, Vol. 81, No. 2, 08.2019, p. 355-363.

Research output: Contribution to journalArticleAcademicpeer-review

Vancouver

Lamberts A, Meijer JM, Pas HH, Diercks GFH, Horvath B, Jonkman MF. Nonbullous pemphigoid: Insights in clinical and diagnostic findings, treatment responses, and prognosis. Journal of the American Academy of Dermatology. 2019 Aug;81(2):355-363. https://doi.org/10.1016/j.jaad.2019.04.029


BibTeX

@article{c84a30f8125842cc82e70b2faa939cd8,
title = "Nonbullous pemphigoid: Insights in clinical and diagnostic findings, treatment responses, and prognosis",
abstract = "Background: Nonbullous pemphigoid is an under-recognized phenotype of the autoimmune bullous disease pemphigoid, characterized by the absence of blisters. Several disease aspects have not been studied previously.Objective: To describe the characteristics of nonbullous pemphigoid.Methods: A retrospective review study of medical records. The diagnosis of pemphigoid was based on meeting 2 of the following 3 criteria: (1) pruritus, (2) positive direct immunofluorescence microscopy, or (3) positive indirect immunofluorescence microscopy on salt-split skin.Results: The review included 69 patients. The mean delay in diagnosis was 29 months. Skin examination most often showed pruritic papules/nodules (37{\%}) or pruritus without primary skin lesions (22{\%}). Histopathologic findings were mainly nonspecific. Results of direct and indirect immunofluorescence microscopy were positive in 60{\%} and 69{\%}, respectively. During follow-up, blisters formed in 17{\%}, which was associated with a positive indirect immunofluorescence microscopy (P = .014) and a positive BP180 immunoblot result (P = .032). The Kaplan-Meier estimates of mortality at 1, 2, and 3 years were 14{\%}, 34{\%}, and 46{\%}, respectively, with an 8.6-fold increased all-cause mortality risk.Limitations: The retrospective study design.Conclusions: Nonbullous pemphigoid presented with heterogeneous pruritic skin lesions, resulting in delayed diagnosis. Direct and indirect immunofluorescence microscopy are essential to diagnose nonbullous pemphigoid, in contrast to histopathology, mainly showing nonspecific findings. An increased all-cause mortality risk was observed during follow-up.",
keywords = "autoimmune blistering disease, autoimmune bullous disease, case series, clinical characteristics, mortality, nonbullous pemphigoid, pemphigoid, prognosis, treatment, INDIRECT IMMUNOFLUORESCENCE, ANTIBODIES, MORTALITY, PROFILES, PRURITUS, EPITOPES, BP230, 1ST",
author = "Aniek Lamberts and Meijer, {Joost M.} and Pas, {Hendri H.} and Diercks, {Gilles F. H.} and Barbara Horvath and Jonkman, {Marcel F.}",
year = "2019",
month = "8",
doi = "10.1016/j.jaad.2019.04.029",
language = "English",
volume = "81",
pages = "355--363",
journal = "Journal of the American Academy of Dermatology",
issn = "0190-9622",
publisher = "MOSBY-ELSEVIER",
number = "2",

}

RIS

TY - JOUR

T1 - Nonbullous pemphigoid

T2 - Insights in clinical and diagnostic findings, treatment responses, and prognosis

AU - Lamberts, Aniek

AU - Meijer, Joost M.

AU - Pas, Hendri H.

AU - Diercks, Gilles F. H.

AU - Horvath, Barbara

AU - Jonkman, Marcel F.

PY - 2019/8

Y1 - 2019/8

N2 - Background: Nonbullous pemphigoid is an under-recognized phenotype of the autoimmune bullous disease pemphigoid, characterized by the absence of blisters. Several disease aspects have not been studied previously.Objective: To describe the characteristics of nonbullous pemphigoid.Methods: A retrospective review study of medical records. The diagnosis of pemphigoid was based on meeting 2 of the following 3 criteria: (1) pruritus, (2) positive direct immunofluorescence microscopy, or (3) positive indirect immunofluorescence microscopy on salt-split skin.Results: The review included 69 patients. The mean delay in diagnosis was 29 months. Skin examination most often showed pruritic papules/nodules (37%) or pruritus without primary skin lesions (22%). Histopathologic findings were mainly nonspecific. Results of direct and indirect immunofluorescence microscopy were positive in 60% and 69%, respectively. During follow-up, blisters formed in 17%, which was associated with a positive indirect immunofluorescence microscopy (P = .014) and a positive BP180 immunoblot result (P = .032). The Kaplan-Meier estimates of mortality at 1, 2, and 3 years were 14%, 34%, and 46%, respectively, with an 8.6-fold increased all-cause mortality risk.Limitations: The retrospective study design.Conclusions: Nonbullous pemphigoid presented with heterogeneous pruritic skin lesions, resulting in delayed diagnosis. Direct and indirect immunofluorescence microscopy are essential to diagnose nonbullous pemphigoid, in contrast to histopathology, mainly showing nonspecific findings. An increased all-cause mortality risk was observed during follow-up.

AB - Background: Nonbullous pemphigoid is an under-recognized phenotype of the autoimmune bullous disease pemphigoid, characterized by the absence of blisters. Several disease aspects have not been studied previously.Objective: To describe the characteristics of nonbullous pemphigoid.Methods: A retrospective review study of medical records. The diagnosis of pemphigoid was based on meeting 2 of the following 3 criteria: (1) pruritus, (2) positive direct immunofluorescence microscopy, or (3) positive indirect immunofluorescence microscopy on salt-split skin.Results: The review included 69 patients. The mean delay in diagnosis was 29 months. Skin examination most often showed pruritic papules/nodules (37%) or pruritus without primary skin lesions (22%). Histopathologic findings were mainly nonspecific. Results of direct and indirect immunofluorescence microscopy were positive in 60% and 69%, respectively. During follow-up, blisters formed in 17%, which was associated with a positive indirect immunofluorescence microscopy (P = .014) and a positive BP180 immunoblot result (P = .032). The Kaplan-Meier estimates of mortality at 1, 2, and 3 years were 14%, 34%, and 46%, respectively, with an 8.6-fold increased all-cause mortality risk.Limitations: The retrospective study design.Conclusions: Nonbullous pemphigoid presented with heterogeneous pruritic skin lesions, resulting in delayed diagnosis. Direct and indirect immunofluorescence microscopy are essential to diagnose nonbullous pemphigoid, in contrast to histopathology, mainly showing nonspecific findings. An increased all-cause mortality risk was observed during follow-up.

KW - autoimmune blistering disease

KW - autoimmune bullous disease

KW - case series

KW - clinical characteristics

KW - mortality

KW - nonbullous pemphigoid

KW - pemphigoid

KW - prognosis

KW - treatment

KW - INDIRECT IMMUNOFLUORESCENCE

KW - ANTIBODIES

KW - MORTALITY

KW - PROFILES

KW - PRURITUS

KW - EPITOPES

KW - BP230

KW - 1ST

U2 - 10.1016/j.jaad.2019.04.029

DO - 10.1016/j.jaad.2019.04.029

M3 - Article

VL - 81

SP - 355

EP - 363

JO - Journal of the American Academy of Dermatology

JF - Journal of the American Academy of Dermatology

SN - 0190-9622

IS - 2

ER -

ID: 92915811