Stiff skin syndrome and myeloma successfully treated with autologous haematopoietic stem cell transplantation (HSCT)

CER6270
2013 Vol.31, N°2 ,Suppl.76
PI 0181, PF 0183
Case Reports

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PMID: 23910622 [PubMed]

Received: 14/01/2013
Accepted : 08/04/2013
In Press: 23/07/2013
Published: 23/07/2013

Abstract

Stiff skin syndrome (SSS) is a rare scleroderma-like syndrome characterised by stone hard skin, joint limitation and progressive restriction of chest that may lead to death. We describe the efficacy of haematopoietic autologous stem cell transplantation (HSCT) in a case of SSS secondary to a smouldering myeloma (SM), with severe joint disability, lung interstitial disease and oesophageal dysfunction. The patient was evaluated at 1, 12 and 18 months after HSCT, clinically (joint motility, HAQ and NYHA for dyspnoea) and instrumentally (DLCO, chest HRCT, oesophagus x-ray). After 18 months since HSCT, we observed a high improvement, contemporaneously to SM remission, of HAQ, joint motility, lung (at DLCO and HRCT) and oesophageal abnormalities.