Pleural effusion in Sweet's syndrome

[Shell567]

[CASE-STUDY] A 68-Year-Old Man With Skin Rash and a Pleural Effusion.

- 68-year-old man with an itchy red rash on right thigh which later spread to extremities and trunk.

- Fevers of > 38.3°C, night sweats, fatigue, shortness of breath, and a dry cough.

- Prescribed triamcinolone 0.1% cream (steroid cream) for his rash and azithromycin (antibiotic) for PRESUMED PNEUMONIA, with no improvement in symptoms.

- History of relapsing polychondritis (rare disease which causes inflammation of the cartilage, and can affect lungs) for which he was prescribed infliximab (immunosuppressant) and low-dose prednisone (steroid).

- Blood tests showed raised ESR and CRP, indicating increased levels of inflammation in the body

- Chest imaging revealed a pleural effusion (collection of fluid next to the LUNG). A sample of fluid from the lung showed a mixed lymphocytic and eosinophilic exudate (white blood cells - 875 total cells, 2% polymorphonuclear neutrophils, 45% lymphocytes, and 30% eosinophils).

- Skin biopsy showed SWEET'S SYNDROME (SS).

- Prednisone (main treatment for SS) was increased from 7.5 mg/d to 20 mg/d, and leflunomide (immunosuppressant) was started, with rapid improvement of both the respiratory and skin symptoms.

Multiorgan involvement in SS can include: encephalitis, myocarditis, alveolitis, pleural effusions, hepatitis, small and large bowel inflammation, and glomerulonephritis.

Patients with pulmonary SS (affecting lungs) have reported nonproductive cough and shortness of breath, "and chest imaging has revealed parenchymal infiltrates, nodules, and pleural effusions. When performed, results of lung biopsy have shown neutrophilic alveolar infiltrates."

"Pleural effusions associated with SS are rare...thus, it is difficult to identify a predominant cellular pattern that is seen with the syndrome."

Stiller, R. H., Gadzhiev, M., Schachtel, A. K., Chang, O.H., Bastawrous, S., Shantz, H. H., Matute-Bello, G. and Albert, T. J. (2020) A 68-Year-Old Man With Skin Rash and a Pleural Effusion. CHEST JOURNAL, JUL;158(1):e33-e36.

journal.chestnet.org/articl...

[Shell567 Administrator Sweet's Syndrome UK]

Pulmonary SS (SS affecting the lungs) is often misdiagnosed as bacterial pneumonia.