Complex Regional Pain Syndrome: What the Dermatologist Should Know
May 2018 | Volume 17 | Issue 5 | Original Article | 532 | Copyright © 2018
Azam A. Qureshi BA and Adam J. Friedman MD
George Washington Medical Faculty Associates, Washington, DC
Reflex sympathetic dystrophy is a subtype of complex regional pain syndrome, a condition characterized by persistent post-injury extremity pain. Temperature and sweating changes, edema, mobility changes, and a variety of hair, nail, and skin sequelae have been described. Only 23 articles published since 1990 describe dermatologic changes in CRPS. Given this paucity of literature, we present a case to further elucidate cutaneous manifestations of CRPS. Our patient is a 52-year-old Caucasian woman with a 19-year history of reflex sympathetic dystrophy who has presented with several dermatologic complaints. She was first referred to the clinic due to episodic, mildly tender, clustered, non-blanching, and non-palpable petechiae on the legs bilaterally, which was histologically consistent with leukocytoclastic vasculitis. No systemic involvement was identified and symptoms resolved with topical steroids. The patient was also noted to have severe ten toenail dystrophy without any evidence of onychomycosis based on multiple cultures and PAS staining of clipped nails. Avulsion of the great toenail was performed to provide symptomatic relief. Incidentally, the patient was found to have lower extremity and facial hypotrichosis on physical exam. Further, she required increased lidocaine administration in addition to pre and post-procedure lidocaine and prilocaine 5% emulsion cream for various office procedures, suggestive of lidocaine insensitivity. This case captures previously described cutaneous manifestations of CRPS such as vasculitis, nail dystrophy, extremity hypotrichosis, and telangiectasia, along with newly described potential manifestations about which dermatologists should be aware, including facial hypotrichosis and lidocaine insensitivity. J Drugs Dermatol. 2018;17(5):532-536.
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Reflex sympathetic dystrophy (RSD), is a subcategory of complex regional pain syndrome (CRPS), a term which encompasses many entities identified in the literature such as causalgia and algodystrophy.1,2 A hallmark of CRPS is localized post-injury extremity pain that is out of proportion to what would be expected after a similar trauma. Other manifestations of CPRS include autonomic abnormalities, motor dysfunction, and increased bone resorption.3 Notably, a variety of hair, nail, and skin changes have been described to occur in association with CPRS.4-6 Dermatologic findings can be non-specific, and therefore are difficult to associate definitively with CRPS in individual patients.5 Because the cutaneous signs are often misdiagnosed as other conditions with similar skin findings, it is imperative for dermatologists to understand the diversity of cutaneous manifestations of CRPS.5,7 We present a case of a patient with long-standing RSD in order to provide a guided review of previously described dermatologic manifestations of RSD while detailing newly described potential features of the disease.
Our patient is a 52-year-old Caucasian female with a 19-year history of RSD developing after a non-displaced left tibia fracture suffered while hiking. Persistent pain and swelling despite bracing and physical therapy led to a formal diagnosis of RSD six months following the onset of symptoms. She initiated care with a pain specialist and embarked on a lengthy treatment course involving systemic steroids, nerve block treatments, epidural ropivacaine 0.1% infusion treatments, intravenous ketamine infusions, phenoxybenzamine, nifedipine, gabapentin, pregabalin, transdermal lidocaine patches, and transdermal clonidine patches. In addition to hallmark CRPS pain symptoms, she experienced secondary Raynaud’s symptoms in her feet bilaterally, which improved with diosmiplex. Furthermore, the patient has experienced a variety of dermatologic manifestations including vasculitis, nail changes, lidocaine insensitivity, localized hypotrichosis, and facial telangiectasia.
Our patient has reported intermittent episodes of a mildly tender rash appearing as multiple clustered non-blanching and non-palpable petechiae on the lower legs bilaterally. At times, the rash was pruritic. A punch biopsy of a characteristic lesion (Figure 1A) revealed a mild superficial perivascular and interstitial neutrophilic infiltrate with leukocytoclasia, rare eosinophils, sparse superficial perivascular lymphocytes, and mild erythrocyte extravasation. No systemic involvement was identified and symptoms resolved with topical steroids.Vasculitis has been previously described in patients with RSD.8-10 Webster et al presented a case series including two RSD