Skin manifestations and vascular endothelial growth factor levels in POEMS syndrome: impact of autologous hematopoietic stem cell transplantation.

OBJECTIVES

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.

DESIGN

Case series from January 1993 through June 2007.

SETTING

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

PATIENTS

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

MAIN OUTCOME MEASURES

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.