[Crow-Fukase syndrome (POEMS syndrome). The first Italian presentation of a case and review of the literature].
A 42-year-old Italian man affected with a multisystemic disease is presented. The main features were: polyneuropathy, organomegaly (liver, spleen and lymph nodes enlargement), endocrinopathy (loss of libido, low plasmatic levels of testosterone), monoclonal protein (k-light chains only in 100-fold-concentrated urine sample, without other signs of plasmocytic proliferation), skin changes. Clinical skin alterations were striking: diffuse thickening, hyperpigmentation, hyperhidrosis , hypertrichosis, while histo- and immunopathological examination of skin biopsies showed proliferation of dermal collagen fibers and deposition of melanin in the epidermis. These findings were suggestive for the diagnosis of Crow-Fukase (POEMS) syndrome, associated with peculiar angiofollicular lymph node hyperplasia (Castleman's disease). The patient was followed up for 15 months with steroids, systemic chemotherapy, plasma-exchange and immunomodulating drugs. A massive anasarca complicated the picture leading him to death. Actually the pathomechanisms of this rare disease have not been fully elucidated. The relations between this syndrome and some malignant lymphoproliferative diseases (i.e. osteosclerotic myeloma) are controversial; at least a part of its features could be either reactive or tissue-specific-antibody mediated. A genetic influence should be suggested from the Japanese reports.
Ospedale di Lecco, Divisione di Dermatologia.
A
A
Carcaterra
R
R
Santini
G
G
Sozzi
E
E
Zuccoli
D000328:Adult; D004700:Endocrine System Diseases / Q000175:diagnosis*; D006023:Glycoproteins / Q000652:urine*; D006529:Hepatomegaly / Q000175:diagnosis; D006801:Humans; D007558:Italy; D008206:Lymphatic Diseases / Q000175:diagnosis* / Q000473:pathology; D008297:Male; D009422:Nervous System Diseases / Q000175:diagnosis*; D012871:Skin Diseases / Q000175:diagnosis* / Q000473:pathology; D013163:Splenomegaly / Q000175:diagnosis; D013577:Syndrome
D006023:Glycoproteins; C023587:protein M (glycoprotein)
Sindrome di Crow-Fukase (s. POEMS). Prima descrizione italiana di un caso e revisione della letteratura.