Pyoderma Gangrenosum associated with Paroxysmal Nocturnal Haemoglobinuria

  • Francisco Ronaldo Moura Filho Médico Residente/Resident of Dermatology, Serviço de Dermatologia do Hospital Universitário Getúlio Vargas (HUGV), Universidade Federal do Amazonas (UFAM), Manaus, Brasil
  • Aline Guimarães Grana Médico Residente/Resident of Dermatology, Serviço de Dermatologia do Hospital Universitário Getúlio Vargas (HUGV), Universidade Federal do Amazonas (UFAM), Manaus, Brasil
  • Suzi Marla Carvalho Maron Médico Residente/Resident of Dermatology, Serviço de Dermatologia do Hospital Universitário Getúlio Vargas (HUGV), Universidade Federal do Amazonas (UFAM), Manaus, Brasil
  • Raquel Rodrigues Ferreira Rocha de Alencar Acadêmico de Medicina/Medical Academic, da Universidade Federal do Amazonas, Manaus, Brasil
  • Bruno Vianei Real Antonio Acadêmico de Medicina/Medical Academic, da Universidade Federal do Amazonas, Manaus, Brasil
  • Patricia Chicre Bandeira de Melo Dermatologista Especialista pela Sociedade Brasileira de Dermatologia/Dermatologist, Mestre em Medicina Tropical pela Universidade do Estado do Amazonas, Manaus, Brasil
Keywords: Hemoglobinuria, Paroxysmal, Pyoderma Gangrenosum

Abstract

Pyoderma gangrenosum is a chronic neutrophilic dermatosis with no established etiology. It is often associated with systemic diseases such as inflammatory rheumatic diseases, inflammatory bowel diseases, malignant neoplasms and haematological diseases including nocturnal paroxysmal hemoglobinuria. It presents as ulcerated and painful skin lesions with rapid and progressive evolution, mainly in the lower limbs. Ulcerations may arise spontaneously or induced by trauma (pathergy phenomenon). The period between the onset of the lesions and the diagnosis is usually prolonged. The diagnosis is based on clinical data and confirmed after the exclusion of other causes of cutaneous ulcerations. We report a case of Pyoderma gangrenosum in the left upper limb with onset after a traumatic event in a patient with nocturnal paroxysmal hemoglobinuria.

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Published
2017-07-20
How to Cite
Moura Filho, F. R., Guimarães Grana, A., Carvalho Maron, S. M., Rodrigues Ferreira Rocha de Alencar, R., Vianei Real Antonio, B., & Chicre Bandeira de Melo, P. (2017). Pyoderma Gangrenosum associated with Paroxysmal Nocturnal Haemoglobinuria. Journal of the Portuguese Society of Dermatology and Venereology, 75(2), 173-177. https://doi.org/10.29021/spdv.75.2.773
Section
Case Reports