screening and early detection which helps informed decision making, potentially reducing healthcare burden within a family. Hyperparathyroidism has been reported in one other case of PGL-4 syndrome
and could be an exceedingly rare manifestation potentially leading to earlier diagnosis. PBRT has been reported to have excellent tumor control and can be used when surgical resection is not viable.
THE MANY HEADS OF HYDRALAZINE: A RARE CASE OF HYDRALAZINE INDUCED LUPUS ERYTHEMATOSUS AND HYDRALAZINE INDUCED P-ANCA POSITIVE BULLOUS HEMORRHAGIC SWEET SYNDROME Kashish Shah, Parinistha Mohan, Shriya Vudari, Prajwal Kumsi Sreedhar, Sarah Baker; Willis-Knighton Health, Shreveport, LA.
Introduction: Overlap between Lupus and Sweet syndrome is an infrequently reported but important adverse effect of hydralazine. We discuss an exceedingly rare presentation of hydralazine induced lupus erythematosus and p-ANCA positive Cryptococcoid Sweet Syndrome presenting with bullous hemorrhagic lesions and encephalopathy. Case description: A 70-year-old female with stage III chronic kidney disease, hypertension and anemia presented with weakness, unintentional weight loss, failure to thrive, skin lesions, and progressively worsening confusion. She had recently been evaluated by rheumatology for her anemia, acute kidney injury, positive ANA, and low complement levels. Examination was notable for hemorrhagic papules and vesicles over bilateral eyelids and ulcerations over labial mucosa. Vital signs were stable except for a fever of 100.1 oF. Suspecting herpes zoster, acyclovir was started. Routine investigations showed low hemoglobin, elevated creatinine, and elevated CRP. Urinalysis showed microscopic hematuria and proteinuria. A full body CT scan revealed tree-in-bud pattern infiltrates in the bilateral lungs. Over the course of her admission, her confusion worsened and she developed hemorrhagic plaques over bilateral eyelids, bilateral ears, nostrils, oral mucosa, and scalp, as well as hemorrhagic bullae
over bilateral fingertips and legs. A MRI and CT of the head, cerebrospinal studies, and infectious workup were unremarkable. An autoimmune evaluation was positive for anti-ds DNA antibodies, anti-histone antibodies, anti-myeloperoxidase antibodies, and p-ANCA. Suspecting vasculitis, a punch biopsy was obtained, high dose methylprednisolone was started, and hydralazine was discontinued. Histopathology revealed dermal infiltration with cryptococcoid neutrophils without any features of vasculitis, consistent with Sweet Syndrome. Her hemoglobin dropped again, and she was found to have subcapsular splenic hematoma for which she underwent splenic artery embolization. Her skin and mucosal lesions showed some improvement, but her confusion persisted. She was discharged to a skilled nursing facility with stable hemoglobin but was re- admitted due to melanotic stool and rapidly dropping hemoglobin. She continued to deteriorate clinically and eventually passed of cardiopulmonary arrest. Discussion: Although rare, hydralazine induced Sweet syndrome can present with concomitant lupus. As our case exemplifies, bullous hemorrhagic Cryptococcoid Sweet Syndrome can have refractory neurological manifestations even in the absence of definitive radiological findings, posing a challenge in identification and treatment of this entity.
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