Therapeutic challenge in a patient with pemphigus foliaceus variety similar to “El Bagre”

Authors

  • Juan Villlalobos José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic
  • Ana Laura Costa José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic
  • Sabrina Merenzon José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic
  • Mónica Di Milia José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic
  • Marcelo Label José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic

DOI:

https://doi.org/10.47196/gd19g221

Keywords:

pemphigus foliaceus, rituximab, gamma globulin, El Bagre

Abstract

Pemphigus is a severe, chronic autoimmune bullous disease (ABD) that affects the skin, mucous membranes, and cutaneous adnexa. It is associated with both genetic and environmental factors, including emotional stress, drugs, certain foods, viruses, and surgical interventions. Pemphigus has several clinical forms and is classified based on the level of blister formation: superficial (subcorneal), deep (suprabasal), and special/rare variants, which are less frequently described. First-line treatment involves systemic corticosteroids and rituximab, along with corticosteroid-sparing agents (such as azathioprine, mycophenolate mofetil, cyclophosphamide, and methotrexate).

We present a clinical case of a pemphigus variant not previously described in our country. This specific disease is more commonly reported in Colombia and Brazil. Furthermore, the patient's management presented a therapeutic challenge due to the complications encountered during treatment.

Author Biographies

  • Juan Villlalobos, José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic

    Dermatologist and Allergist, Dermatology Department

  • Ana Laura Costa, José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic

    Dermatologist, Dermatology Department

  • Sabrina Merenzon, José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic

    Dermatologist, Chief Resident, Dermatology Department

  • Mónica Di Milia, José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic

    Dermatologist, Autoimmune Bullous Diseases Section, Department of Dermatology

  • Marcelo Label, José María Ramos Mejía General Acute Care Hospital, City of Buenos Aires, Argentine Republic

    Dermatologist, Autoimmune Bullous Diseases and Mycology Section, Faculty Member at the University of Buenos Aires (UBA), Dermatology Department

References

I. Grupo de Trabajo de Enfermedades Ampollares Autoinmunes. Guías de manejo de los pénfigos 2020 [Internet]. Sociedad Argentina de Dermatología; 2020 [citado febrero 2025]. Disponible en: https://sad.org.ar/wp-content/uploads/2020/06/Guias-de-manejo-de-penfigo-2020.-.pdf.

II. Abreu-Vélez A, Howard SM, Lambraño H, Tobón-Arroyave S. Compromiso oral subclínico en pacientes con pénfigo foliáceo endémico. Concepto Dermatol Pract 2018; 8:2.

III. Abreu-Velez A, Upegui-Zapata Y, Valencia-Yepes C, Upegui-Quiceño E, et al. Patterns of antinuclear antibodies in a new variant of endemic pemphigus in El Bagre, Colombia, colocalizing with antigens against MIZAP, ARVCF, p0071, and desmoplakins I and II. J Appl Lab Med. 2022;7:1366-1378.

IV. Abreu-Velez AM, Howard MS, Hashimoto T, Grossniklaus HE. Human eyelid meibomian glands and tarsal muscle are recognized by autoantibodies from patients affected by a new variant of endemic pemphigus foliaceus in El-Bagre, Colombia, South America. J Am Acad Dermatol. 2010;62:437-447.

V. Abreu-Vélez AM, Upegui-Zapata Y, Valencia-Yepes C, Upegui-Quiceno, et al. Afectación de las áreas compuestas del corazón en el pénfigo foliáceo endémico. Concepto de Dermatol Pract. 2019;9:181-186.

VI. Hoffmann JHO, Enk AH. High-dose intravenous immunoglobulin in skin autoimmune disease. Front Immunol. 2019;10.

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Published

2026-07-16

Issue

Vol. 32 No. 2 (2026): May–August

Section

Clinical Cases

License

Copyright (c) 2026 on behalf of the authors. Reproduction rights: Argentine Society of Dermatology

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