Immunohistochemical with type IV collagen. A diagnostic tool to differentiate anti-p200 pemphigoid from epidermolysis bullosa acquisita

Authors

  • Karen Ginzburg F. J. Muñiz Hospital, City of Buenos Aires, Argentina
  • Olga Forero F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina
  • María Emilia Candiz F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina
  • Esteban Maronna F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina
  • María Gracia Funes Ghigi F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina
  • Lara Marotte F. J. Muñiz Hospital, City of Buenos Aires, Argentina
  • Luciana Cabral Campana J. M. Ramos Mejía Hospital, Autonomous City of Buenos Aires, Argentina
  • Mónica Di Milia J. M. Ramos Mejía Hospital, Autonomous City of Buenos Aires, Argentina
  • Marcelo Label University of Buenos Aires, Autonomous City of Buenos Aires, Argentina
  • María Cecilia Madeo Dr. E. Tornú General Acute Hospital, Autonomous City of Buenos Aires, Argentina
  • Claudia Massone Dr. E. Tornú General Acute Hospital, Autonomous City of Buenos Aires, Argentina
  • Eliana Pavón Dr. E. Tornú General Acute Hospital, Autonomous City of Buenos Aires, Argentina
  • Graciela Rodríguez Costa Hospital de Clínicas José de San Martín, Ciudad Autónoma de Buenos Aires, Argentina
  • Nadia Rojano José de San Martin Clinical Hospital, Autonomous City of Buenos Aires, Argentina

DOI:

https://doi.org/10.47196/da.v30i2.2576

Keywords:

immunohistochemical collagen IV, anti-p200 pemphigoid, laminin gamma 1, acquired bullous epidermolysis, autoimmune bullous dermatoses

Abstract

Introduction: the diagnosis of autoimmune bullous diseases represents quite a challenge. When the immunofluorescence with salt-split technique has IgG and C3 at the floor of the membrane zone, several differential diagnoses are considered. Immunohistochemical with collagen IV is suggested as a complementary tool to differentiate between acquired bullous epidermolysis and anti-p200 pemphigoid.

Objectives: use immunohistochemical with collagen IV to differentiate anti-p200 pemphigoid from epidermolysis bullosa acquisita. Secondly, describe the epidemiological, clinical, and histopathological characteristics of these patients with these entities within our population.

Design: cross-sectional, retrospective, descriptive, observational, multicenter study.

Materials and methods: immunohistochemical with collagen IV was performed on biopsy specimens from patients whose direct immunofluorescence with salt-split technique showed IgG and C3 deposits on the floor of the membrane zone.

Results: 19 samples were analyzed, 12 (63.2%) testing positive for immunohistochemical with collagen IV, and 7 (36.8%) showing no labeling. Among the positive samples, 8 (42.1%) exhibited labeling on the floor of the membrane zone, interpreted as probable anti-p200 pemphigoid, and 4 (21.1%) on the roof, interpreted as acquired bullous epidermolysis.

Conclusions: immunohistochemical with collagen IV turned out to be an accessible and highly useful method that complements the diagnostic methods of autoimmune bullous dermatoses of the dermoepidermal junction. In this study, we observed a high predominance of cases with a probable diagnosis of anti-p200 pemphigoid, which reinforces the fact that this pathology is underdiagnosed due to the lack of accurate diagnostic methods.

Author Biographies

Karen Ginzburg, F. J. Muñiz Hospital, City of Buenos Aires, Argentina

Chief Resident, Dermatology Service

Olga Forero, F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina

Chief Resident, Dermatology Service

María Emilia Candiz, F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina

Staff Dermatologist, Dermatology Service

Esteban Maronna, F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina

Pathologist

María Gracia Funes Ghigi, F. J. Muñiz Hospital, Autonomous City of Buenos Aires, Argentina

4th year student, Dermatology Service

Lara Marotte, F. J. Muñiz Hospital, City of Buenos Aires, Argentina

3rd year resident, Dermatology Service

Luciana Cabral Campana, J. M. Ramos Mejía Hospital, Autonomous City of Buenos Aires, Argentina

Staff Physician, Dermatology Service

Mónica Di Milia, J. M. Ramos Mejía Hospital, Autonomous City of Buenos Aires, Argentina

Dermatologist, Autoimmune Blistering Diseases Sector

Marcelo Label, University of Buenos Aires, Autonomous City of Buenos Aires, Argentina

Dermatologist, Doctor of Medicine (UBA), president of the Argentine Society of Dermatology, Autonomous City of Buenos Aires, Argentina

María Cecilia Madeo, Dr. E. Tornú General Acute Hospital, Autonomous City of Buenos Aires, Argentina

Dermatologist, Head of the Dermatology Service

Claudia Massone, Dr. E. Tornú General Acute Hospital, Autonomous City of Buenos Aires, Argentina

Head of the Pathological Anatomy Unit

Eliana Pavón, Dr. E. Tornú General Acute Hospital, Autonomous City of Buenos Aires, Argentina

Assigned Dermatologist, Dermatology Service

Graciela Rodríguez Costa, Hospital de Clínicas José de San Martín, Ciudad Autónoma de Buenos Aires, Argentina

Dermatologist, former head of outpatient clinics, José de San Martín Clinical Hospital, Autonomous City of Buenos Aires, Argentina

Nadia Rojano, José de San Martin Clinical Hospital, Autonomous City of Buenos Aires, Argentina

Dermatologist, Dermatology Service

References

I. Maronna E. Histopatología. En: Forero O, Candiz M.E, Olivares L. Dermatosis ampollares autoinmunes. Haga su diagnóstico. Ed Journal. Buenos Aires 2021;10-28

II. Forero O, Roquel L. Inmunofluorescencia. En: Forero O, Candiz M.E, Olivares L. Dermatosis ampollares autoinmunes. Haga su diagnóstico. Ed Journal. Buenos Aires 2021;29-40.

III. Candiz ME. Serologías por ELISA. En: Forero O, Candiz M.E, Olivares L. Dermatosis ampollares autoinmunes. Haga su diagnóstico. Ed Journal. Buenos Aires 2021;41-50.

IV. García-Díez I, Martínez-Escala M.E, Ishii N, Hashimoto, et ál. Descripción de dos casos de penfigoide anti-p200. Utilidad de una técnica inmunohistoquímica sencilla en el diagnóstico diferencial con otras enfermedades ampollosas autoinmunes. Actas Dermosifilogr. 2017;108:e1-e5.

V. Sajeela-Rasheed V. Anti-p200 pemphigoid: a review. J Skin Sex Transm Dis. 2023;5:22-27.

VI. Luzar B, McGrath J. Inherited and autoimmune subepidermal blistering diseases. En: Calonje E, Brenn T, Lazar A, Billings S. McKee’s pathology of the skin with clinical correlations. Elsevier, Edinburgh, 2020:118-170.

VII. Meijer JM, Diercks GF, Schmidt E, Pas HH, et ál. Laboratory diagnosis and clinical profile of anti-p200 pemphigoid. JAMA Dermatol. 2016;152:897-904.

VIII. Ginzburg K, Forero O, Candiz ME, Maronna E, et ál. Penfigoide anti-p200: ¿enfermedad poco frecuente o subdiagnosticada? Dermatol Argent. 2022;28:25-29.

IX. Forero O, Candiz ME. Epidermólisis ampollar adquirida, variedad inflamatoria símil penfigoide de las mucosas. En: Forero O, Candiz M.E, Olivares L. Dermatosis ampollares autoinmunes. Haga su diagnóstico. Ed Journal. Buenos Aires 2021; 231-234.

X. Dainichi T, Koga H, Tsuji T, Ishii N, et ál. From anti-p200 pemphigoid to anti-laminin gamma1 pemphigoid. J Dermatol. 2010;37:231-238.

XI. Lau I, Goletz S, Holtsche MM, Zillikens D, et ál. Anti-p200 pemphigoid is the most common pemphigoid disease with serum antibodies against the dermal side by indirect immunofluorescence microscopy on human salt-split skin. J Am Acad Dermatol. 2019;81:1195-1197.

XII. Tamamura R Nagarsuka H Comparative analysis of basal lamina type IV collagen chains, matrix metalloproteinases-2 and -9 expressions in oral dysplasia and invasive carcinoma. Acta Histochem. 2012;115:113-119.

XIII. Urushiyama H, Terasaki Y, Nagasaka S, Terasaki M, et ál. Role of α1 and α2 chains of type IV collagen in early fibrotic lesions of idiopathic interstitial pneumonias and migration of lung fibroblasts. Lab Invest. 2015;95:872-85.

XIV. Goletz S, Hashimoto T, Zillikens D, Schmidt E. Anti-p200 pemphigoid. J Am Acad Dermatol. 2014;71:185-191.

XV. Gao Y, Qian H, Hashimoto T, Li X. Potential contribution of anti-p200 autoantibodies to mucosal lesions in anti-p200 pemphigoid. Front Immunol. 2023;14:1118846.

XVI. Kridin K, Ahmed AR. Anti-p200 pemphigoid: a systematic review. Front Immunol. 2019;10:2466.

XVII. Velásquez-Lopera MM, Vélez-López N, Álvarez-Acevedo LC, Ruiz-Restrepo JD. Epidermólisis ampollar adquirida, variedad inflamatoria símil penfigoide ampollar. En: Forero O, Candiz M.E, Olivares L. Dermatosis ampollares autoinmunes. Haga su diagnóstico. Ed Journal. Buenos Aires 2021;228-230.

XVIII. Kridin K, Kneiber D, Kowalski EH, Valdebran M, et ál. Epidermolysis bullosa acquisita: a comprehensive review. Autoimmun Rev. 2019;18:786-795.

Published

2024-05-16

Issue

Section

Original Articles