title: Calciphylaxis in a patient with POEMS syndrome without renal failure and/or hyperparathyroidism. A case report.
abstract: POEMS (Crow-Fukase) syndrome is a rare plasma cell lymphoproliferative disorder associated with polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal (M) gammopathy and skin (S) abnormalities. The latter are usually not specific and include hyperpigmentation, hypertrichosis, cutaneous angioma and skin-thickening. A 45-year-old Italian woman was admitted to hospital because of muscle weakness, marked fatigue and paresthesia of the upper and lower extremities. Two and a half years earlier, a POEMS syndrome had been diagnosed on the basis of a history of organomegaly and mild lymphadenopathy, IgA-lambda monoclonal gammopathy, hypothyroidism, severe lower and upper limb sensory-motor peripheral neuropathy and a single osteosclerotic lesion in the left humerus. Eight weeks later, she developed skin lesions bioptically shown to be due to calciphylaxis-induced cutaneous vasculitis. To our knowledge, this is the first case of POEMS syndrome with this peculiar type of vasculitis. The absence of predisposing conditions, namely renal failure, hyperparathyroidism or clotting disorders renders the pathogenetic mechanism(s) of this severe type of vasculitis more intriguing.
authors.affiliation: Dipartimento di Scienze Biomediche e Oncologia Umana, Università degli Studi di Bari.
authors.forename: Ileana
authors.initials: I
authors.lastname: De Roma
authors.forename: Raffaele
authors.initials: R
authors.lastname: Filotico
authors.forename: Michele
authors.initials: M
authors.lastname: Cea
authors.forename: Pasquale
authors.initials: P
authors.lastname: Procaccio
authors.forename: Federico
authors.initials: F
authors.lastname: Perosa
mesh_terms: D001777:Blood Coagulation; D002115:Calciphylaxis / Q000097:blood* / Q000209:etiology* / Q000473:pathology; D002130:Calcium Phosphates / Q000097:blood; D005221:Fatigue / Q000209:etiology; D005260:Female; D006728:Hormones / Q000097:blood; D006801:Humans; D006961:Hyperparathyroidism / Q000150:complications; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology; D016878:POEMS Syndrome / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D010292:Paresthesia / Q000209:etiology; D051437:Renal Insufficiency / Q000150:complications; D014657:Vasculitis / Q000209:etiology
chemical_list: D002130:Calcium Phosphates; D006728:Hormones; C020243:calcium phosphate