Abstract:

Sweets syndrome is a reactive inflammatory disorder of unknown etiology that can mimic other clinical conditions. We present a case of Sweets syndrome with neurologic manifestations clinically and histologically mimicking systemic cryptococcosis. The patient was a 63-year-old man presenting with fever, altered mental status and rapidly progressing disseminated papules and plaques. Systemic antibiotics for community acquired pneumonia were initiated. Physical examination revealed vesicles and umbilicated edematous papules and plaques on the scalp, face, trunk and upper extremities. Due to abrupt clinical deterioration in spite of broad antibiotic coverage, acyclovir and amphotericin B were added for possible herpes encephalitis and systemic mycosis. Blood, urine cultures and CSF analysis were negative for infectious etiologies and tests for HIV, HSV, VZV, and CSF and serum cryptococcal antigen were negative. Brain MRI was negative for acute intracranial pathology. Skin biopsy revealed papillary dermal edema and a dense dermal neutrophilic predominant infiltrate with basophilic bodies surrounded by capsule-like vacuolated spaces resembling Cryptococcus. GMS and PAS staining and all tissue cultures were negative. Additional staining with MPO and CD15 highlighted the infiltrate which was predominantly neutrophilic. The patient was diagnosed with cryptococcoid Sweets syndrome and started on systemic steroids with immediate improvement of both neurologic manifestations and cutaneous lesions. While respiratory tract infection is a possible trigger, work up is still ongoing to identify other potential risk factors. This constellation of findings has rarely been described in the literature and highlights the importance of recognizing atypical presentations of Sweets syndrome to prevent unnecessary delay in diagnosis and ensure timely medical therapy.

Financial Disclosure:
No current or relevant financial relationships exist.

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Hesham Alshaikh, MD