We report a case of a 42-year-old female with past medical history significant for cutaneous mastocytosis who presented with complains of progressive dry eyes, and dry mouth for the past few weeks. Her vital signs were remarkable for blood pressure of 106/71 mmHg, pulse of 86 beats per minute, temperature of 36.7°C (98.1°F) (oral), and respiratory rate of 16 breaths per minute. Physical exam was significant for bilateral dryness in eyes, cracks at the corners of the mouth, dry and smooth tongue. Lab work revealed a benign blood count and chemistry. Sedimentation rate was elevated at 25 mm/hr, CRP was also elevated at 11.1 mg/L, and ANA with speckled pattern was elevated at 1250 1/dil. Speckled pattern was elevated at 1250 1/dil. Hence patient was managed with muscarinic agonists such as cevimeline and pilocarpine, which improved her symptoms. We report a rare case of a patient with maculopapular cutaneous mastocytosis who develops Sjogren’s syndrome. Literature review revealed that in the past there have been case reports which linked systemic mastocytosis to Sjogren’s syndrome, however there are no case reports of maculopapular cutaneous mastocytosis linking to Sjogren’s syndrome. Clinicians need to be aware that even the milder form of mastocytosis, cutaneous type, can also be associated with Sjogren’s syndrome. Absence of well defined histopathological features and lack of clinical awareness can delay its diagnosis and treatment.
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