title: Cutaneous manifestations of familial amyloidotic polyneuropathy.
abstract: Familial amyloidotic polyneuropathy is an autosomal dominant amyloidosis, characterized by the systemic deposition of amyloid with a particular involvement of the peripheral nerves. The disease generally manifests as a severe sensory, motor and autonomic neuropathy. Cardiomyopathy, nephropathy, vitreous opacities and carpal tunnel syndrome may occur in a variable association with the neuropathy. Trophic dermatological lesions are frequent in the more advanced stages of the disease. We examined the skin of 142 patients. The cutaneous manifestations more frequently observed were: xerosis (81.6%), seborrheic dermatitis (21.8%), traumatic and burn lesions (19.7%), acne (18.3%), neurotrophic ulcers (14%) and onychomycosis (10.5%). Among the hepatic transplanted patients (31%), seborrheic dermatitis and acne were the most frequent diagnoses.
authors.affiliation: Dermatology, Hospital Geral st. Antonio, sa, Porto--Portugal. derma@chvng.min_saude.pt
authors.forename: N
authors.initials: N
authors.lastname: Rocha
authors.forename: G
authors.initials: G
authors.lastname: Velho
authors.forename: M
authors.initials: M
authors.lastname: Horta
authors.forename: A
authors.initials: A
authors.lastname: Martins
authors.forename: A
authors.initials: A
authors.lastname: Massa
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011115:Polyneuropathies / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D011174:Portugal / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D012871:Skin Diseases / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy