Diffuse Lung Disease |

Pulmonary Pyoderma Gangrenosum Without Cutaneous Manifestations FREE TO VIEW

Kenneth Sakata, MD; Sudheer Penupolu, MD; Lewis Wesselius, MD; Thomas Colby, MD
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Mayo Clinic Arizona, Scottsdale, AZ

Chest. 2014;146(4_MeetingAbstracts):416A. doi:10.1378/chest.1990595
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SESSION TITLE: Miscellaneous Cases I

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Sunday, October 26, 2014 at 10:45 AM - 12:00 PM

INTRODUCTION: Pyoderma gangrenosum (PG) is a chronic inflammatory, nonneoplastic, sterile skin disorder of unknown etiology. Extracutaneous manifestations of PG are rare and occur commonly in the lung. Pulmonary lesions are seen either concurrently or after the cutaneous manifestations of PG. To our knowledge, there have been only 3 adult cases where pulmonary findings preceded cutaneous disease. We report the first case of pulmonary PG that manifested without cutaneous lesions.

CASE PRESENTATION: A 19 y.o. male presented with complaints of 2 weeks of dyspnea, hemoptysis, and diarrhea. His past medical history is significant for smoking, marijuana use and chronic sinusitis. He was febrile, tachycardic, tachypneic, and hypoxic. Crackles were heard throughout all lung fields. His skin exam was normal. His lab exam was only remarkable for leukocytosis. A CXR showed new bilateral infiltrates. He was started on empiric antibiotics, however, he deteriorated. A chest CT revealed multifocal areas of consolidation, cavitation, and bilateral pleural effusions. Autoimmune panel, coccidioidomycosis serologies, HBV, HCV, HIV, CT sinuses, a left middle turbinate biopsy, EGD, and colonoscopy, were all unremarkable. Bronchoalveolar lavage fluid was bloody, but cultures were negative. A right thoracostomy with wedge biopsies of the right lung was consistent with PG. Cultures of the lung tissue were negative. Oral prednisone 60 mg daily was initiated and he rapidly improved.

DISCUSSION: PG is associated with systemic diseases in 50% of cases. It is commonly associated with inflammatory bowel disease, seronegative rheumatoid arthritis, or immunologic and hematologic diseases. An extensive battery of tests looking for associated systemic conditions and infections were negative. Lesions are often limited to the skin, however, extracutaneous manifestations of PG have been reported in other organs, with the lungs being the most common site. We report the first case of pulmonary PG without cutaneous findings.

CONCLUSIONS: We report the first case of pulmonary PG without an associated underlying systemic disorder and without cutaneous manifestations.

Reference #1: Gibson et al. Neutrophilic dermatoses and myeloproliferative disease: Report of two cases. Mayo Clin Proc 1985;60:735-740.

Reference #2: McCulloch et al. Severe steroid-responsive pneumonitis associated with pyoderma gangrenosum and ulcerative colitis. Thorax 1985;40.

Reference #3: Kitagawa et al. Primary pyoderma gangrenosum of the lungs. J Am Acad Dermatol 2008;59:S114-116.

DISCLOSURE: The following authors have nothing to disclose: Kenneth Sakata, Sudheer Penupolu, Lewis Wesselius, Thomas Colby

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