We describe the clinical features and post mortem findings in a patient with POEMS syndrome (P — polyneuropathy, O — organomegaly, E — endocrinopathy, M — M protein, S — skin changes), with particular emphasis on the evolution of the endocrinopathy over a three year period. In addition to gynaecomastia this case exhibited primary hypothyroidism and primary and secondary adrenal and gonadal failure. The widespread nature of the endocrine changes suggests that a circulating substance interferes with the action of trophic hormones, possibly by inhibiting second messenger production.
ShimpoS.Solitary plasmacytoma with polyneuritis and endocrine disturbances. Jap J Clin Med1988; 26: 2444–2456.
2.
CrowRS. Peripheral neuritis in myelomatosis. Br Med J1956; 2: 802–804.
3.
IwashitaH, InoueN, NagamatsuK.Polyneuropathy, pigmentation, diabetes mellitus and monoclonal gammopathy; report of an autopsy case (Japan). Rinsho Shinkeigaku1971; 11: 492–499.
4.
BardwickPA, ZvaiflerNJ, GillNM, NewmanD, GreenwayGD, ResmickKL. Plasma cell neoplasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes; the POEMS syndrome — report on two cases and a review of the literature. Medicine (Baltimore)1980; 59: 311–322.
5.
NakanishiT, SobueI, ToyokuraYThe Crow Fukase syndrome: A study of 102 cases in Japan. Neurology (Cleveland)1984; 34: 712–720.
6.
SolomonREB, GibbsDD. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes: The POEMS syndrome. J R Soc Med1982; 75: 553–555.
7.
PuigL, MorenoA, DomingoPCutaneous angiomas in POEMS syndrome. J Am Acad Dermatol1985; 12: 961–964.
8.
BurtonJL. Peripheral neuropathy associated with dysproteinaemia, skin changes and endocrinopathy. Br Med J1986; 292: 1415–1416.
9.
MatsumineH.Accelerated conversion of androgen to oestrogen in plasma cell dyscrasia associated with polyneuropathy, anasarca and skin pigmentation. New Engl J Med1985; 313: 1025–1026.