Download Treatment of Pain in Dercum`s Disease with Lidoderm® (Lidocaine 5

PAIN MEDICINE
Volume 9 • Number 8 • 2008
CASE REPORT
Treatment of Pain in Dercum’s Disease with Lidoderm®
(Lidocaine 5% Patch): A Case Report
Mehul J. Desai, MD, MPH,* Radhika Siriki, MD,† and Dajie Wang, MD*
*Department of Anesthesiology, Jefferson Pain Center, Thomas Jefferson University Hospital, Philadelphia,
Pennsylvania, USA; †Department of Internal Medicine, Englewood Hospital, Englewood, New Jersey, USA
ABSTRACT
Case. A patient with Dercum’s disease successfully treated with transdermal lidocaine 5% patches.
The patient’s pain was initially rated as an 8/10. At follow-up examination after 1 month, the patient
rated her pain as 3/10—a >60% reduction in pain; this pain reduction persisted at subsequent
1-month follow-up intervals.
Conclusion. Current therapeutic options in the treatment of Dercum’s disease have proven either
ineffective or cumbersome. The use of transdermal lidocaine is a safe and non-invasive treatment
modality that has been efficacious in alternate forms. The use of this medication might prove
preferable to more invasive or risky treatment and warrants further investigation.
Key Words. Lidocaine; Pain Management; Obesity
Introduction
D
ercum’s disease was first described by neurologist Francis Xavier Dercum in 1892 [1].
Also known as adiposis dolorosa, lipomatosis
dolorosa, Ander’s Syndrome, or Dercum–Vitaut
syndrome, it is a rare disorder characterized
by multiple painful subcutaneous lipomas on the
trunk and extremities. Dercum’s disease most
commonly occurs in obese, postmenopausal
women (female-to-male ratio approximately 30:1)
[2]. There is suspicion of an autosomal dominant
genetic predisposition to the development of
Dercum’s disease [3,4]. Most cases, however, tend
to occur sporadically. Although the exact patho-
Reprint requests to: Mehul J. Desai, MD, MPH, Jefferson
Pain Center, 834 Chestnut Street, Suite T150, Philadelphia, PA 19107, USA. Tel: 215-955-7246; Fax: 215-9235086; E-mail: [email protected].
physiology is unknown, it is postulated that the
etiology of the disease is related to abnormal blood
flow or circulatory dysfunction [5]. Treatment has
been challenging due to the rarity of this condition. Here we present a case of a patient successfully treated with lidocaine 5% transdermal
patches and briefly review the treatment literature.
Case Report
A 61-year-old female with medical history of
Dercum’s disease, paroxysmal supraventricular
tachycardia, rheumatoid arthritis, depression, and
migraine headaches presented to our tertiary pain
clinic for evaluation. Her medications at presentation included verapamil, nortriptyline, sumatriptan, and gabapentin three times daily, all stable for
greater than 6 months. The patient’s complaints
included severe pain in the right medial and
© American Academy of Pain Medicine 1526-2375/08/$15.00/1224 1224–1226
doi:10.1111/j.1526-4637.2008.00417.x
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Introduction. Dercum’s disease is a rare disorder characterized by multiple painful subcutaneous
lipomas on the trunk and extremities. It most commonly occurs in obese, postmenopausal women.
The pain associated with this condition is postulated to arise from enlarging lipomas producing
pressure on peripheral nerves, thereby initiating pain and sometimes paresthesias. Treatment has
been challenging due to the rarity of this condition.
Treatment of Pain in Dercum’s Disease
posterior thigh and right anterior chest wall. This
pain was characterized as constant burning and
shooting. The pain was initially rated as an 8/10.
Physical examination revealed discrete lipomas at
the right anterior chest wall and right posterior
and medial thigh. Lidocaine 5% transdermal
patches were prescribed and placed over the
two painful sites. At follow-up examination after 1
month, the patient rated her pain as 3/10—a >60%
reduction in pain; this pain reduction persisted at
subsequent 1-month follow-up intervals.
Discussion
The majority of treatment studies reported in
the literature are uncontrolled case reports focusing on medical and surgical interventions. Surgical
therapies in the literature include lipoma resection
and liposuction. Surgery has demonstrated only
partial relief of the pain, with the advent of new
painful lesions at nearby locations [5,13].
Medical therapy has consisted of a variety
of agents, including analgesics, membranestabilizing agents such as lidocaine and mexiletine,
and corticosteroids. Recently, the use of interferon
alfa-2b therapy in patients with concurrent hepatitis C was reported to result in long-term pain
relief [14]. Systemic steroids and pituitary supplementation have not proven effective. Palmer
reported some reduction of pain with different
doses of prednisone in a single patient [8]. Reggiani et al. reported successful pain relief with the
use of EMLA, a eutectic mixture of lidocaine 2.5%
and prilocaine 2.5%, in a patient who previously
failed to respond to intravenous lidocaine, oral
mexiletine, and intramuscular methylprednisolone therapy [15]. Mexiletine is an analogue of
lidocaine that is not metabolized as rapidly as
lidocaine and is available in an oral form. Longstanding pain relief after oral treatment with
mexiletine has been reported in patients with
Dercum’s disease who were previously responsive
to intravenous lidocaine therapy [16,17].
Currently, case reports are the largest segment
of the literature describing some efficacy with
intravenous lidocaine. Lidocaine theoretically
blocks impulse conduction in peripheral nerves
and depresses cerebral activity, therefore promoting pain relief. One such report stated that
1,300 mg of lidocaine was given daily for 4 days to
achieve pain relief for a patient with Dercum’s
disease; however, this dosage prompted warnings
regarding serious side effects such as cardiac toxicity and seizure activity [18]. Iwane et al. reported
pain relief lasting up to 10 hours with intravenous
lidocaine doses of 200–400 mg on every-other-day
dosing; however, the patient reported euphoria
and numbness during infusion, and flushing and
altered mental status was noted following treatment [7]. Petersen and Kastrup reported two
patients with Dercum’s disease treated with doses
from 200–600 mg of lidocaine resulting in 1 week
to 25 days of total pain relief over a 2-year period
[17]. Atkinson reported 2–12 months of pain relief
with lidocaine infusions over a 2-year period [19].
Finally, Devillers and Oranje reported pain-free
periods of up to 4 months with lidocaine infusions
every 6 months [16]. Unfortunately, this therapy
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There are three common subtypes of Dercum’s
disease: type I: juxta-articular type, with painful
folds of fat on the inside of the knees and/or on the
hips, in rare cases only evident in upper-arm fat;
type II: diffuse, generalized type, where widespread pain from fatty tissue is found, apart from
that of type I, also often in the dorsal upper-arm
fat, in the axillary and gluteal fat, in the stomach
wall, in dorsal fat folds, and on the soles of the feet;
and type III: lipomatosis, nodular type, with
intense pain in and around multiple lipomas,
sometimes in the absence of general obesity;
lipomas are approximately 0.5–4 cm, soft, and
attached to the surrounding tissue [6].
This syndrome consists of four cardinal symptoms: 1) multiple, painful, fatty masses; 2) generalized obesity; 3) weakness and fatigability; and 4)
mental disturbances, including emotional instability, depression, epilepsy, confusion, and dementia.
The typical patient is an obese postmenopausal
woman between 40 and 60 years of age with pain
that appears out of proportion to physical finding.
The pain can last for hours, can be paroxysmal or
continuous, and worsens with movement. The
condition can also be associated with early congestive heart failure, myxedema, joint pain, paroxysmal flushing episodes, tremors, cyanosis,
hypertension, headaches, and epistaxis [1,7–9].
The pain associated with this condition is postulated to arise from enlarging lipomas. These
lipomas are thought to produce pressure on peripheral nerves, thereby initiating pain and sometimes
paresthesias, although this hypothesis is not universally accepted [10,11]. Other theories include
defective lipid metabolism and endocrine abnormalities [10,12]. The neuropathic pain resulting
from this condition remains a challenge due to the
rarity of this condition and its unclear pathoetiology. Current therapy focuses on symptomatic
control and can be either medical or surgical.
1225
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Conclusions
Current therapeutic options in the treatment of
Dercum’s have proven cumbersome or invasive.
The use of transdermal lidocaine is a safe and
non-invasive treatment modality that has been
efficacious in alternate forms. Although the existing data suggest a systemic lidocaine effect, low
plasma levels of lidocaine administered by transdermal delivery systems likely necessitate that they
be utilized directly at the lesion site. The use of
this medication might prove to be useful in a treatment algorithm of Dercum’s disease, particularly
early in disease progression. Its use as a first-line
agent might prove preferable to more invasive or
risky treatments and warrants further investigation, although controlled trials might be challenging given the rarity of this condition.
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has multiple potential side effects and is best
administered at a tertiary care facility with significant experience in this modality. Furthermore,
repeated treatments with intravenous lidocaine are
cumbersome and expensive.
Transdermal lidocaine patches have been efficacious in the treatment of neuropathic pain
originating from complex regional pain syndrome,
painful diabetic neuropathy, and postherpetic neuralagia [20,21]. Systemic effects are very unlikely
due to the fact that plasma concentrations of
topical lidocaine are well below the concentrations
that might cause cardiac anti-arrhythmic activity
(1.5 mg/mL) and lidocaine toxicity (5 mg/mL) [22].
Even with administration of a lidocaine patch for
12 hours followed by removal of the patch for 12
hours, the maximal concentration is 0.13 mg/mL,
which is well below toxic levels [22].
Desai et al.