Skin manifestations and vascular endothelial growth factor levels in POEMS syndrome: impact of autologous hematopoietic stem cell transplantation.
OBJECTIVE
To investigate skin manifestations of the polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and their correlation with serum vascular endothelial growth factor (s-VEGF-A) levels and to describe the impact of autologous peripheral blood stem cell transplantation (aPBSCT) on these manifestations and the correlation with s-VEGF-A levels.


METHODS
Case series from January 1993 through June 2007.


METHODS
Hospitalized care in Assistance Publique-Hôpitaux de Paris in Pitié-Salpêtrière and Tenon hospitals.


METHODS
Twenty-three patients with POEMS syndrome, 10 of whom were clinically followed up after aPBSCT.


METHODS
Description and distribution of clinical lesions at POEMS syndrome diagnosis, skin evaluation after aPBSCT, and s-VEGF-A levels measured at POEMS syndrome diagnosis and after aPBSCT.


RESULTS
In 21 patients with skin manifestations at POEMS syndrome diagnosis, the most common skin manifestations were hemangiomas (18 patients [86%]), hyperpigmentation (16 [76%]), skin thickening (12 [57%]), acrocyanosis (12 [57%]), hypertrichosis (11 [52%]), acquired facial lipoatrophy (11 [52%]), and white nails (8 [38%]). The median s-VEGF-A level was not different between patients with and without skin manifestations except in those with hypertrichosis (P = .04). After aPBSCT, no significant correlation was observed between s-VEGF-A level decreases and response of skin manifestations, again except for hypertrichosis (P = .007).


CONCLUSIONS
Acquired facial lipoatrophy and livedo should be added to the skin manifestations of POEMS syndrome. Despite a role of s-VEGF-A in various skin manifestations, the impact of s-VEGF-A level decreases on skin outcomes is weak after aPBSCT, mostly resulting in clinical stabilization.
Barete
Stéphane
S
Department of Dermatology, University , 75020 Paris, France. stephane.barete@psl.aphp.fr
Mouawad
Roger
R
Choquet
Sylvain
S
Viala
Karine
K
Leblond
Véronique
V
Musset
Lucile
L
Amoura
Zahir
Z
Khayat
David
D
Francès
Camille
C
D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000097:blood / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D012189:Retrospective Studies; D012867:Skin / Q000378:metabolism / Q000473:pathology*; D014182:Transplantation, Autologous; D042461:Vascular Endothelial Growth Factor A / Q000097:blood*
C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A