Blame it on the Pill

A second skin biopsy at our institute revealed mixed dermatitis pattern with features of granuloma annulare and neutrophilic dermatosis consistent with Sweet's syndrome. Work-up for systemic malignancy was negative. The rash improved with a course of prednisone but led to steroid induced diabetes. He developed hoarseness of voice and dyspnea. Laryngoscopy revealed laryngitis and an ulcer on the epiglottis. He had an elevated ESR (114), CRP (8), ANA (1:320), and myeloperoxidase antibody (MPO)(21). High-resolution chest CT showed interstitial lung disease. Pulmonary function testing revealed FVC of 85% and FEV1/FVC of 92%. Right lobe wedge biopsy of the lung revealed obliterative fibrosis and smooth muscle metaplasia in areas of prior bronchioles, typical for chronic obliterative bronchiolitis usually associated with collagen vascular disease. The constellation of findings of positive ANA, lymphopenia (at presentation), neutrophilic dermatosis, interstitial lung disease (BOOP- bronchiolitis obliterans with organizing pneumonia) and positive MPO was highly suggestive of systemic lupus erythematosus (SLE). He was started on azathioprine and prednisone was tapered. This resulted in resolution of disc edema in the right eye and improvement in the left. A follow up contrast enhanced MRI of brain and orbits was normal. A follow up lumbar puncture (for headache) showed normal opening pressure (11 cm H2O), and normal CSF contents. Sweet's syndrome (SS) is an acute neutrophilic dermatosis characterized by erythematous plaques and papules that presents with fever and leukocytosis, usually seen in association with autoimmune disease, malignancy or medication use. Neuro-sweet is believed to be a distinct entity with meningoencephalitis as the cause of raised CSF pressure leading to papilledema.