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Pulmonary Pyoderma Gangrenosum: A Rare Cause of Multiple Pulmonary Masses FREE TO VIEW

Sarah Kilbourne, MD; Manojkumar Patel, MBBS; Mary Baker, BS; Elliot Smith, MD; Lauren Murphy, BS
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University of Virginia, Charlottesville, VA

Chest. 2014;146(4_MeetingAbstracts):323A. doi:10.1378/chest.1992260
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SESSION TITLE: Miscellaneous Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare, neutrophilic, ulcerative, non-infectious dermatosis. Extracutaneous manifestations are rare, but pulmonary involvement has been reported. Described here is a patient who presented with a history of cutaneous PG and rapidly enlarging pulmonary masses.

CASE PRESENTATION: A 52-year-old man presented with a ten day history of fever, chills, malaise, and non-productive cough. Past medical history: PG of the lower extremities and 3 year history of pulmonary nodules of unknown etiology. On admission, he was febrile, tachycardic, had a leukocytosis, and ulcers with heaped violaceous borders on his lower extremities. CT chest revealed enlargement of multiple pulmonary masses, including a large left apical mass (12.4 x 7.3 x 11cm) and right lower lobe mass with cavitation (7.2 x 9.2 cm). Core biopsy of the left mass revealed dense neutrophilic infiltration with granulomatous inflammation, without evidence of vasculitis or malignancy. Bacterial, fungal, and acid-fast stains and cultures were negative. With no rhinosinus or renal involvement and a negative cANCA, granulomatosis with polyangitiis was unlikely. With biopsy findings of neutrophilic inflammation without infection, malignancy, or vasculitis, pulmonary PG was diagnosed and treatment with prednisone was initiated. With symptomatic improvement within 24 hours, the patient was continued on prednisone as an outpatient. Chest CT after 6 months of steroid therapy revealed near resolution of the pulmonary masses.

DISCUSSION: The classic presentation of PG is a erythematous-violaceous nodular cutaneous lesion that rapidly progresses to a painful ulcer. PG has an association with ulcerative colitis, monoclonal gammopathy, hematologic disorder, malignancy, and hepatitis C. Histology reveals non-specific necrotizing inflammatory granulomas with a neutrophilic infiltrate. The most frequent pulmonary manifestations are nodules with or without cavitation. The differential diagnosis of pulmonary PG includes infection, malignancy, vasculitis. In pulmonary PG, there is an associated increase in hematologic tumors, namely myelodysplastic syndrome or monoclonal gammopathy. This patient was unusual compared to previously reported cases given the extraordinary size of the pulmonary masses.

CONCLUSIONS: Though a rare manifestation of a rare disease, pulmonary PG should be considered in the appropriate patient with pulmonary abnormalities.

Reference #1: Actas Dermosifiliogr. 2011;102:373-7.

Reference #2: J Am Acad Dermatol. 2000;43:108-12.

Reference #3: Chest. 2001;119:977-8.

DISCLOSURE: The following authors have nothing to disclose: Sarah Kilbourne, Manojkumar Patel, Mary Baker, Elliot Smith, Lauren Murphy

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