C. Rivollier et al., Notalgia paresthetica associated with multiple endocrine neoplasia type IIA (Sipple's Syndrome): A report of 3 cases, ANN DER VEN, 126(6-7), 1999, pp. 522-524
Introduction. Notalgia paresthetica is an isolated sensory mononeuropathy P
atients have a pruritus in the mid-upper back. Its association with multipl
e endocrine neoplasia type IIA has been reported in a few cases. We report
three cases of this association.
Case reports. Case n degrees 1: A 45 year-old woman had multiple endocrine
neoplasia type IIA with a medullary thyroid carcinoma and a primary hyperpa
rathyroidism; she had a mid-upper back pigmented lesion. Histological exami
nation showed dermal melanosis and deposits of amyloid in the dermis. Case
n degrees 2: A woman had a multiple endocrine neoplasia type IIA which was
diagnosed at the age of 60; she had a surgical treatment for a pheochromocy
toma, a medullary thyroid carcinoma, and a primary hyperparathyroidism; she
had dermatological examination for a pruriginous lesion of the mid-upper b
ack. Case n degrees 3: The daughter of the patient n degrees 2. had had a s
urgical cure for a medullary thyroid carcinoma and a pheochromocytoma at th
e age of 31; she had a papulous and-pruriginous lesion in the left scapular
area. Her daughter and her sister had a multiple endocrine neoplasia type
IIA, without notalgia paresthetica.
Discussion. Notalgia paresthetica is a benign cutaneous disorder which can
be associated with multiple endocrine neoplasia type IIA, it can be conside
red that notalgia paresthetica is an early clinical marker of multiple endo
crine neoplasia type IIA,. Patients with a familial history of notalgia par
esthetica or with an onset of notalgia paresthetica in childhood should be
screened for multiple endocrine neoplasia type IIA,. Patients with multiple
endocrine neoplasia must also been screened for notalgia paresthetica beca
use its finding is an argument for a familial form of multiple endocrine ne
oplasia type IIA,. Dermatologists should be aware of this association.