Complex Regional Pain Syndrome Associated with Henoch-Schonlein Purpura

Abstract

Introduction: Henochschonlein purpura (HSP) is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis, most commonly affecting children. It is a small-vessel vasculitis which is characterized by a tetrad of palpable purpura (without thrombocytopenia), abdominal pain, arthritis, and renal impairment. Although most commonly affecting the skin, joints, gastrointestinal tract, and kidneys, other organs may also be affected. Association of HSP with complex regional pain syndrome (CRPS) has not been reported in the literature. In the present case report, we are describing the successful management of CRPS of the upper limb in a diagnosed case of HSP. Case Presentation: A 14-year-old female patient, who was a diagnosed case of HSP, presented with features characteristic of CRPS in the right hand and forearm, based on the Budapest Diagnostic Criteria. Bone scintigraphy showed increased tracer uptake in joints of the right hand, also suggestive of CRPS. The pain was severe visual analog scale-90/100 and not controlled with conservative management. Patient was administered a series of 3 stellate ganglion minimally invasive pain and spine interventions (MIPSI) over the course of 2 weeks, which led to the resolution of her symptoms. Conclusion: HSP is a small-vessel vasculitis and may lead to peripheral nerve vasculitis. As reported in other cases, small-vessel vasculitis has led to the development of CRPS and thus may influence the development of CRPS in patients with HSP. CRPS as a complication of HSP has not been reported in the literature to date. Thus in a patient with HSP, CRPS could present as a rare complication and early intervention with the MIPSI of the affected region may lead to resolution of symptoms.