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Neurocutaneous spectrum of multiple endocrine neoplasia-1
Shireen Furtado1, Nandita Ghosal, Sunil V Furtado
1Consultant Dermatologist, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bangalore-560 066, Karnataka, India.
Abstract:
Multiple endocrine neoplasia type I or Wermer syndrome is characterized by primary hyperparathyroidism, enteropancreatic endocrine tumor, and a pituitary pathology. A 35-year-old male presented with visual field defects, hyperprolactinemia, and hypogonadism. He also had multiple infraumbilical skin-colored nodules. A syndromal association of Wermer syndrome was derived using the dermal, pituitary, parathyroid, and gastrointestinal hormonal manifestations of the tumor. The radiological and histological findings of lesion which underwent biopsy are discussed. The presence of collagenomas, lipomas, and hypopigmented macules in a patient with neuroendocrine symptoms should raise the suspicion of an underlying multiple endocrine neoplasia.
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