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Ped Rheum Boards

Scleroderma-like Disorders

QuestionAnswer
scleredema B-hemolytic Strep infection
scleredema - Edematous induration - Begins on face, neck, and shoulders - Progresses to thorax and proximal extremities - May have peau d’orange appearance - Spares hands
scleredema resolves in 6-12 months
nephrogenic systemic fibrosis gadolinium exposure in patients with renal failure
nephrogenic systemic fibrosis - Edematous induration leading to progressive fibrosis - Begins on distal hands and feet - Progresses to proximal extremities - May have peau d’orange appearance - Trunk involvement is rare - Spares face and head
nephrogenic systemic fibrosis Chronic and progressive (unless patient recovers renal function)
eosinophilic fasciitis Excessive physical exercise or trauma
eosinophilic fasciitis - Symmetric - Painful - Edematous induration - Begins in extremities, neck, and trunk - May have peau d’orange appearance - Spares hands and feet
eosinophilic fasciitis Acute onset. May be long lasting and treatment resistant
Diabetic cheiroarthropathy diabetes mellitus
Diabetic cheiroarthropathy - Progressive tightening of the skin and soft tissues - Results in contractures of the finger joints in as many as 29% of children with juvenile diabetes mellitus
Diabetic cheiroarthropathy Generally irreversible, but passive occupational therapy may improve function
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