TY - JOUR
T1 - Scleromyxoedema
T2 - Treatment of cutaneous and systemic manifestations with high-dose intravenous immunoglobulin
AU - Kulczycki, A.
AU - Nelson, M.
AU - Eisen, A.
AU - Heffernan, M.
PY - 2003/12
Y1 - 2003/12
N2 - Scleromyxoedema is a rare disease characterized by cutaneous sclerosis, mucin deposition and paraproteinaemia. Internal disease is common, particularly musculoskeletal, gastrointestinal and central nervous system involvement. We report a series of three consecutive patients with scleromyxoedema treated with high-dose intravenous immunoglobulin (hdIVIg). Each of the three patients had relatively low levels of a highly basic IgG-λ paraprotein, and each has demonstrated a sustained response of both their cutaneous and extracutaneous disease to hdIVIg. As all patients had perioral skin involvement and microstomia, one measure of cutaneous improvement was the increase in intraincisor distance. Extracutaneous manifestations of scleromyxoedema that improved included ureteral stricture, vocal strength and dysphagia.
AB - Scleromyxoedema is a rare disease characterized by cutaneous sclerosis, mucin deposition and paraproteinaemia. Internal disease is common, particularly musculoskeletal, gastrointestinal and central nervous system involvement. We report a series of three consecutive patients with scleromyxoedema treated with high-dose intravenous immunoglobulin (hdIVIg). Each of the three patients had relatively low levels of a highly basic IgG-λ paraprotein, and each has demonstrated a sustained response of both their cutaneous and extracutaneous disease to hdIVIg. As all patients had perioral skin involvement and microstomia, one measure of cutaneous improvement was the increase in intraincisor distance. Extracutaneous manifestations of scleromyxoedema that improved included ureteral stricture, vocal strength and dysphagia.
KW - Case series
KW - Intravenous immunoglobulin
KW - Paraprotein
KW - Scleromyxoedema
UR - http://www.scopus.com/inward/record.url?scp=0346725885&partnerID=8YFLogxK
U2 - 10.1111/j.1365-2133.2003.05682.x
DO - 10.1111/j.1365-2133.2003.05682.x
M3 - Article
C2 - 14674909
AN - SCOPUS:0346725885
SN - 0007-0963
VL - 149
SP - 1276
EP - 1281
JO - British Journal of Dermatology
JF - British Journal of Dermatology
IS - 6
ER -