Cutaneous lesion associated with multiple endocrine neoplasia type 2A: lichen amyloidosis or notalgia paresthetica?

摘要: Three patients of a French family demonstrated an association multiple endocrine neoplasia type 2A (MEN 2A) with pruritic scapular skin lesion. The lesions are similar to those described as familial cutaneous lichen amyloidosis in unrelated MEN and medullary thyroid carcinoma families, but histological, immunohistochemical, ultrastructural analysis biopsies from each patient the did not show amyloid deposition. topography lesion follows dermatomes C8-D3. report only pruritus also paresthesia hyperalgesia, one showed touch hypoesthesia pain hyperesthesia area Such neurological features suggests notalgia paresthetica (NP), neuropathy posterior dorsal rami nerves. We thus suggest that associated might be secondary pathology neural crest-derived sensory amyloid, when present, would scratching. propose presenting NP suspect for 2A.