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Pulmonology Procedures |

Pulmonary Involvement in Sweet's Syndrome

Jey Chung*, MD; Jonathan Chen, MD; Jean Oak, MD; Tushar Desai, MD
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Stanford University, Stanford, CA


Chest. 2012;142(4_MeetingAbstracts):878A. doi:10.1378/chest.1390908
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Abstract

SESSION TYPE: Bronchology Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Sweet's syndrome is a rare neutrophilic dermatosis manifesting as erythematous skin plaques, fever, and neutrophilia. It is typically associated with an underlying systemic process, commonly a hematologic malignancy. We report the case of a patient presenting with a rapidly progressive dermatosis and respiratory failure, found to have myelodysplastic syndrome with Sweet’s Sydrome and airway involvement.

CASE PRESENTATION: A 66 year-old previously healthy gentleman presents with fevers, chills, and rhinorrhea, along with a rash on his leg. On evaluation he is noted to have a significant fever, an erythematous, purpuric rash on his lower extremity, as well as leukocytosis, bandemia, anemia, thrombocytopenia, elevated inflammatory markers, and coagulopathy. Empiric, broad-spectrum antibiotics are initiated; however, overnight he decompensates, developing hypoxic respiratory failure and an eruption of patchy, purpuric lesions over his face and body. Bone marrow biopsy reveals myelodysplastic syndrome, and skin biopsy demonstrates a neutrophilic dermatitis consistent with Sweet’s Syndrome. Bronchoscopy reveals areas of small, pearly-white nodules overlying patchy mucosal erythema, presumed to represent extracutaneous Sweet’s Syndrome involving the bronchial mucosa. Despite high-dose corticosteroid therapy and maximal supportive care, the patient progresses to multi-organ failure and dies within two weeks of his initial presentation.

DISCUSSION: Reported is a case of fulminant Sweet's syndrome with involvement of the tracheobronchial airways. Pulmonary involvement in Sweet’s Syndrome is rare, with only a few dozen case reports in literature. Pulmonary manifestations include interstitial infiltrates, nodules, effusions, and bronchial mucosal lesions, as seen here. Pulmonary manifestations often coincide temporally with cutaneous lesions. Additionally, pulmonary involvement in Sweet’s Syndrome appears to be frequently associated with the presence of an underlying hematologic dyscrasia. Prompt biopsy of lesions is essential for diagnosis, allowing for rapid initiation of immunosuppressive agents which may improve the course of disease. Unfortunately, despite prompt diagnosis and appropriate therapy in this patient with Sweet’s Syndrome, myelodysplastic syndrome, and associated pulmonary involvement, the disease process ultimately proves fatal.

CONCLUSIONS: Although rare, pulmonary involvement can be observed in Sweet’s Syndrome and often correlates temporally with skin findings. Additionally, underlying hematologic disorders are frequently associated with pulmonary involvement. Ultimately, this constellation of findings seemingly portends a poor prognosis.

1) Astudillo L. Pulmonary involvement in Sweet’s syndrome: a case report and review of the literature. Int Dermatol 2006;45:677-680.

2) Ravaglia C. Sweet's syndrome associated with myelogenous leukemia and pulmonary involvement. Monaldi Arch Chest. 2011;75(2):149-50.

DISCLOSURE: The following authors have nothing to disclose: Jey Chung, Jonathan Chen, Jean Oak, Tushar Desai

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