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Complex regional pain syndrome
Emily S. Carr, BS, Ashley De La Cerda, MD, and Katherine Fiala, MD
Complex regional pain syndrome (CRPS) is a neurologic disorder that
often results in debilitating chronic pain, but the diagnosis may elude
providers as it is one of exclusion. A history of trauma may be elucidated.
We report a case of CRPS and review the clinical findings, appropriate
workup, and treatment options for the patient. The patient we describe
went through an extensive workup before receiving the correct diagnosis.
Delay in diagnosis leads to prolonged suffering for the patient and, at
times, unnecessary invasive debridement procedures. Raising awareness
of this entity may help physicians make the correct diagnosis early, as
well as initiate a collaborative effort between neurology, anesthesiology,
and dermatology to provide the patient the most favorable outcome.
W
e present a case of complex regional pain syndrome
(CRPS) in a 41-year-old man to highlight the importance of early recognition and diagnosis to reduce
the significant morbidity associated with this disease.
CASE DESCRIPTION
A 41-year-old white man presented to the emergency department with a severely painful, nonhealing ulceration on his
left index finger after cutting his finger on bailing wire. He
was evaluated in an emergency department and discharged.
He returned several days later with increasing redness and pain,
received a dose of intravenous vancomycin, and was discharged
with oral trimethoprim/sulfamethoxazole. Several days later,
the worsening pain was so severe that he requested amputation
of his finger. Upon admission, his wound was debrided in the
operating room. After several days of intravenous vancomycin,
he was discharged with oral minocycline. All wound cultures
performed over the course of his hospitalizations were negative
for pathogens. Upon his fourth presentation, still in excruciating pain, the dermatology service was consulted for body
tissue culture. Examination of his left index finger revealed a
dry, heme-crusted ulceration with surrounding erythema and
violaceous edema (Figure). He was otherwise healthy, but did
suffer from depression and multiple suicide attempts in the past.
He reported no drug allergies.
The biopsy for tissue culture was negative for fungus, bacteria, and acid-fast bacilli. A plain radiograph displayed soft tissue
swelling. The diagnosis of CRPS following trauma was made.
Proc (Bayl Univ Med Cent) 2016;29(3):333–334
Figure. A painful, nonhealing, dry ulceration on the left index finger with surrounding
violaceous and erythematous edema.
The patient did not follow up in the dermatology department,
and attempts to contact him were unsuccessful. Chart review
showed that he attempted suicide several weeks later.
DISCUSSION
CRPS is a condition that is aptly named, as it is often a
complex entity to diagnose and manage. The disorder results
from a neurologic dysfunction that produces severe and often
debilitating pain. It most often affects extremities and may result from trauma or a vascular event. The condition has many
pseudonyms, including reflex sympathetic dystrophy, algodystrophy, causalgia, Sudeck’s atrophy, transient osteoporosis, and
acute atrophy of bone, which adds to the confusion. In 1993, a
consensus group settled on CRPS as an umbrella term.
The diagnosis of CRPS requires the presence of pain and
sensory changes in a specific region following a noxious event.
The pain is out of proportion to the inciting stimulus and can
be associated with erythema, swelling, temperature changes,
From the Texas A&M Health Science Center College of Medicine (Carr, De La
Cerda, Fiala) and the Department of Dermatology, Baylor Scott & White Health
(De La Cerda, Fiala), Temple, Texas.
Corresponding author: Katherine Fiala, MD, Department of Dermatology, Baylor
Scott & White Health, 2401 S. 31st Street, Temple, TX 76508 (e-mail: Katherine.
[email protected]).
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and abnormal pseudomotor activity (1). There are two types:
type I has no apparent nerve injury (90%) and type II has an
identifiable nerve injury.
The reported skin changes are nonspecific and require awareness of this entity for it to be included in the differential diagnosis. Sundaram et al reported that the most common skin-related
changes include edema (58%), erythema (54%), dermatitis
(35%), erythematous papules (23%), atrophy (23%), ulceration
(13%), and bullae (13%) (2). Our patient presented with edema, erythema, and a nonhealing ulceration in addition to severe
pain. Other disorders often considered first in the differential
are infection, peripheral vascular disease, peripheral neuropathy,
deep venous thrombosis, scleroderma, thoracic outlet syndrome,
rheumatoid arthritis, and perhaps even a conversion or factitious
disorder. Infection was initially considered the likely diagnosis
for our patient, resulting in a debridement procedure.
There are three stages of CRPS. In stage 1, patients may feel
burning pain and develop cutaneous signs of edema, erythema,
or dermatitis but lack underlying bony involvement. During
stage 2, there can be worsening edema of the soft tissues, skin
thickening, and muscle wasting. In stage 3, or chronic CRPS,
there is decreased range of motion, contractures, atrophy of the
skin, and significant demineralization of the bone. However,
one study found no evidence of three consecutive phases of
the disease (3). Patients diagnosed in stage 3 portend a worse
prognosis and should be treated aggressively. Early manifestations are often more consistent with an inflammatory reaction
than a disturbance of the nervous system, which may lead to a
delay in diagnosis (3).
CRPS is a clinical diagnosis of exclusion, but studies that
may aid in making the diagnosis early in the disease are autonomic function testing, bone scintigraphy, plain radiographs,
and magnetic resonance imaging (MRI). One study found bone
scans superior to plain radiographs and MRI for ruling out
CRPS (4).
Treatment should be instituted immediately upon diagnosis
to alleviate the debilitating pain patients suffer with this disorder.
Conservative measures include nonsteroidal antiinflammatory
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drugs, tricyclic antidepressants, gabapentin, topical capsaicin,
bisphosphonates, and low-dose oral glucocorticoids (5–8). More
aggressive therapies for refractory cases include nerve and spinal
cord stimulation, regional nerve blocks, and sympathectomy
(9). Smoking cessation can result in improvement (10). No
matter the stage, physical and occupational therapy should be
initiated upon diagnosis (11). We hope to close this practice gap
and bring more awareness to this painful condition.
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Baylor University Medical Center Proceedings
Volume 29, Number 3