Pulmonary manifestations of pyoderma gangrenosum: 2 cases and a review of the literature

Respir Med. 2015 Apr;109(4):443-50. doi: 10.1016/j.rmed.2014.12.016. Epub 2015 Jan 14.

Abstract

Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatologic disease that occasionally is accompanied by extracutaneous manifestations, amongst these is pulmonary involvement. The etiology is unknown. More than 50% of PG cases are associated with an underlying systemic disease such as inflammatory bowel disease, rheumatoid arthritis, hematological disorder or malignancy. Extracutaneous manifestations are rare and only 29 cases of pulmonary involvement have been reported previously in the literature. Pyoderma gangrenosum is usually diagnosed in the third to sixth decade, but early debut in childhood is also described. Skin manifestations are usually evident before pulmonary involvement, although primary lung affection is seen. Pulmonary involvement is diagnosed simultaneously or from a few weeks up to several years after the diagnosis of cutaneous PG. The most important differential diagnoses are lung cancer, lung abscess and Wegener's granulomatosis. Histological specimens will exclude these diagnoses. The treatment of PG is immune modulation, but due to the rarity of the disease, only one randomized treatment trials exists [1] and the long term course of PG with pulmonary involvement is unknown. We present two cases of pulmonary manifestations of pyoderma gangrenosum and a review of the literature.

Keywords: Cavitating; Lung; Pyoderma gangrenosum.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anti-Infective Agents / administration & dosage
  • Anti-Infective Agents / adverse effects
  • Biopsy / methods
  • Dapsone* / administration & dosage
  • Dapsone* / adverse effects
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / adverse effects
  • Immunosuppressive Agents / classification
  • Infliximab* / administration & dosage
  • Infliximab* / adverse effects
  • Lung / pathology
  • Male
  • Middle Aged
  • Multiple Pulmonary Nodules* / diagnosis
  • Multiple Pulmonary Nodules* / drug therapy
  • Multiple Pulmonary Nodules* / etiology
  • Multiple Pulmonary Nodules* / physiopathology
  • Prednisolone* / administration & dosage
  • Prednisolone* / adverse effects
  • Pyoderma Gangrenosum* / complications
  • Pyoderma Gangrenosum* / diagnosis
  • Pyoderma Gangrenosum* / drug therapy
  • Respiratory Function Tests
  • Skin / pathology
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Anti-Infective Agents
  • Immunosuppressive Agents
  • Dapsone
  • Prednisolone
  • Infliximab