[Systemic mast cell disease associated with cutaneous xanthomas and markedly elevated serum IgE].

@article{Hino1990SystemicMC,
  title={[Systemic mast cell disease associated with cutaneous xanthomas and markedly elevated serum IgE].},
  author={Norihiko Hino and Tooru Yamabe and Takayuki Ono and Naomi Sasaki and Koji Nanba},
  journal={[Rinsho ketsueki] The Japanese journal of clinical hematology},
  year={1990},
  volume={31 9},
  pages={1478-82}
}
Systemic mast cell disease (SMCD) is a rare disease often associated with symptoms of general malaise, pruritus, diarrhea, vomiting, fever, urticaria pigmentosa, hepatosplenomegaly and lymphadenopathy. We reported a case of SMCD associated with cutaneous xanthoma and serum hyper IgE. Skin biopsy revealed xanthomas and diffuse infiltration of mast cells in the dermis. The association of SMCD with xanthoma was reported in the literature for only one case. The hyper IgE could be due to the defect… CONTINUE READING