title: Pulmonary manifestations of pyoderma gangrenosum: 2 cases and a review of the literature.
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.
authors.affiliation: Department of Respiratory Medicine and Allergology, Aarhus University Hospital, Denmark. Electronic address: Melina.gade@midt.rm.dk.
authors.forename: Melina
authors.initials: M
authors.lastname: Gade
authors.affiliation: Department of Dermatology, Aarhus University Hospital, Denmark.
authors.forename: Frej
authors.initials: F
authors.lastname: Studstrup
authors.affiliation: Department of Respiratory Medicine and Allergology, Aarhus University Hospital, Denmark.
authors.forename: Anne Kathrine
authors.initials: AK
authors.lastname: Andersen
authors.affiliation: Department of Respiratory Medicine and Allergology, Aarhus University Hospital, Denmark.
authors.forename: Ole
authors.initials: O
authors.lastname: Hilberg
authors.affiliation: Department of Dermatology, Aarhus University Hospital, Denmark.
authors.forename: Carsten
authors.initials: C
authors.lastname: Fogh
authors.affiliation: Department of Respiratory Medicine and Allergology, Aarhus University Hospital, Denmark.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bendstrup
mesh_terms: D000890:Anti-Infective Agents / Q000008:administration & dosage / Q000009:adverse effects; D001706:Biopsy / Q000379:methods; D003622:Dapsone / Q000008:administration & dosage / Q000009:adverse effects; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000145:classification; D000069285:Infliximab / Q000008:administration & dosage / Q000009:adverse effects; D008168:Lung / Q000473:pathology; D008297:Male; D008875:Middle Aged; D055613:Multiple Pulmonary Nodules / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D017511:Pyoderma Gangrenosum / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D012129:Respiratory Function Tests; D012867:Skin / Q000473:pathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000890:Anti-Infective Agents; D007166:Immunosuppressive Agents; D003622:Dapsone; D011239:Prednisolone; D000069285:Infliximab
keywords: Cavitating; Lung; Pyoderma gangrenosum