Introduction: Reflex sympathetic dystrophy (Complex Regional Pain Syndrome type 1) is little known by dermatologists. We report a pediatric case of reflex sympathetic dystrophy with predominant cutaneous involvement.
Case report: A 10 year-old girl presented a warm, painful and relapsing right hand edema for seven months (three outbreaks). The hand was cyanotic, pigmented and painful. Routine blood tests were normal. Radiography and radionuclide bone scan were consistent with stage 1 reflex sympathetic dystrophy. Physiotherapy led to dramatic improvement.
Discussion: Reflex sympathetic dystrophy is known since the XVIIIth century. In the last decade, progress in radiology and bone scan have provided elements for understanding the physiopathology of the disease. Microvascular abnormalities under the control of sympathetic nervous system are characteristic of different stages of reflex sympathetic dystrophy. Recently, neurovascular system experiments showed that sympathetic reflex tonus changes may be controlled by the central nervous system. Dermatologic changes of reflex sympathetic dystrophy are well known: edema and erythema in first stage, cyanosis in second stage, sclerosis and atrophia in third stage, but pediatric cases are rarely reported.
Conclusion: Reflex sympathetic dystrophy is a complex disease, however its physiopathology is now understood. The clinical presentation can be atypical and the dermatologist may be the first to be consulted.