A Case of Primary Cutaneous Marginal Zone Lymphoma in a 12-year-old with
Mast Cell Activating Syndrome
Abstract
Primary cutaneous marginal zone lymphoma (PCMZL) is reported to
primarily affect non-Hispanic Caucasian males over 50 years old. We
present a case of a 16-year-old female with mast cell activation
syndrome (MCAS) who developed PCMZL of the skin of both cheeks, left
forearm and right arm. The patient was treated initially with
intralesional steroids which successfully shrunk lesions on her face and
left forearm. Rituximab and doxycycline were attempted but stopped due
to severe allergic reactions and anaphylaxis. Ultimately, the remaining
nodules on her right arm were surgically excised.