case challenges •
case challenges: radiology • case challenges
An 11-year-old Girl with
Burning Sensation in Her Hands
Fatih Firinci, MD; Salih Kavukçu, MD; Sebnem Aktan, MD; Alper Soylu, MD;
Atilla Mehmet Türkmen, MD; and Özden Anal, MD
A
n 11-year-old girl presented
with burning sensation, high
temperature, and erythema
involving both hands, especially the
right hand, along with the right arm (see
Figure). These complaints had been
present for the past 4 months, getting
more severe in hot weather and with
exercise, while showing some relief
with cold application and with rest.
Fatih Firinci, MD, is Pediatrician, Department of Pediatrics, Dokuz Eylul
University Hospital, Izmir, Turkey. Salih
Kavukçu, MD; Alper Soylu, MD; Atilla
Mehmet Türkmen, MD; and Özden Anal,
MD, are Professors of Pediatrics, Dokuz
Eylul University Hospital. Sebnem Aktan,
is Professor of Dermatology, Dokuz Eylul
University Hospital.
Address correspondence to: Salih
Kavukcu, MD, Mithatpasa cad 665/4
Kucukyali 35280, Izmi, Turkey; or e-mail
s.kavukcu@deu.edu.tr.
Dr. Firinci; Dr. Kavukçu; Dr. Aktan; Dr. Soylu; Dr. Türkmen; and Dr. Anal have disclosed
no relevant financial relationships.
doi: 10.3928/0090.4481-20101024-05
Figure. An 11-year-old girl presented with burning sensation, high temperature, and erythema involving both hands, especially the right hand, along with the right arm.
Systemic questioning did not reveal
morning stiffness, rash, alopecia, dryness in eyes or mouth, or bloody diarrhea. Only occasional pain in joints of
hands and feet were described. Family
history was uneventful.
In physical examination, vital
signs were within normal ranges.
Widespread erythema was present
on both hands, with a more reticular
pattern on the dorsal part of the right
forearm. Fingers were edematous,
and periungual erythema was present on the fingers of right hand.
Persistent symptoms of burning
sensation and temperature eleva-
For diagnosis, see page 699.
Editor’s note: Each month, this department features a discussion of an unusual diagnosis in genetics, radiology,
or dermatology. A description and images are presented, followed by the diagnosis and an explanation of how the
diagnosis was determined. As always, your comments are welcome via e-mail at pedann@slackinc.com.
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case challenges
tion, along with local vasodilation
and hyperemia, which are activated
by environmental heat and exercise
and are relieved by cold application
and rest, suggested erythromelalgia.
Treatment with misoprostol was begun, considering the low rate of adverse effect of the drug and to avoid
masking any probable underlying
collagen disorder.
Laboratory evaluation for differential diagnosis and classification
of erythromelalgia yielded the following results:
• Hb 13.3 g/dL;
• leukocyte 5000/µL;
• platelet 134,000/µL;
• urine specific gravity 1010;
• pH 6.0;
• protein (1+);
• blood (-);
• 2-3 WBC/hpf;
• glucose 82 mg/dL;
• BUN 21 mg/dL;
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•
•
•
creatinine 0.55 mg/dL;
albumin 4.6 g/dL;
LDL 160 mg/dL;
On the seventh day of
prednisolone treatment,
erythema, pain, and
edema in the right forearm
decreased significantly,
while ESR regressed to
normal values.
HDL 77 mg/dL;
triglycerides 68 mg/dL.
Electromyography and upper
extremity and renal Doppler ultrasonography were normal. Serologic tests revealed hypocomple•
•
mentemia as C3 74 mg/dL (82-173
mg/dL), C4 9.6 mg/dL (13-46 mg/
dL), and positivity in ANA (1/160);
anti-dsDNA (1/20), pANCA and
anticardiolipin IgM (40 MPL/mL),
and IgG (24 GPL/mL) antibodies. Other laboratory results were
erythrocyte sedimentation rate
(ESR): 42 mm/h, CRP: 1.2 mg/dL
(0.1-8.2 mg/dL), rheumatoid factor: negative, extractable nuclear
antigen panel: negative.
Steroid treatment with 2 mg/kg/
day prednisolone was started, and
misoprostol was stopped. On the
seventh day of prednisolone treatment, erythema, pain, and edema
in the right forearm decreased significantly, while ESR regressed to
normal values. Prednisolone was
decreased to alternate day doses
at the end of 4-week treatment
course, and acetyl salicylic acid
(100 mg/d) was added.
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case challenges
D I A G N O S I S
Secondary Erythromelalgia
due to Systemic Lupus
Erytematosus (SLE)
DISCUSSION
A patient with complaints of
burning sensation, erythema, and
an increase in temperature of the
extremities, relieved by cold application and rest, should raise the
suspicion of erythromelalgia. A
careful evaluation in such a case
is needed for differential diagnosis
of some clinical conditions, such
as Raynaud’s syndrome, burning
feet syndrome, reflex sympathetic
dystrophy, angiodyskinesis, acrocyanosis, lipodermatosclerosis,
and erysipelas.1,2
Five criteria suggested by
Thompson et al. are used in the diagnosis of erythromelalgia:3 burning pain in the extremities; increase
in pain in exposure to high environmental temperature; decrease in
pain by cold application; erythema
in the affected body area; and increase in temperature in the affected skin region. All of these were
observed in this child, and she was
diagnosed with erythromelalgia.
Erythromelalgia can be primary
but also may appear secondary to
another disease. Therefore, other
underlying clinical conditions that
might be the cause of erythromelalgia should be properly excluded,
such as drugs (verapamile, nicardipine, bromocriptine, pergolide,
mercury); vasculitis syndromes;
cardiovascular diseases (hypertension, venous insufficiency); hematologic disorders (pernicious ane-
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mia, thrombocytemia, thrombotic
thrombocytopenic purpura, polycytemia, hereditary spherocytosis,
leukemias); neuropathies (polyneuropathy, hereditary sensory neuropathy, central nervous system lesions); infections (syphilis, AIDS);
autoimmune diseases; musculoskeletal disorders; cholesterol crystal
embolism syndrome; diabetes mellitus; hypercholesterolemia; gout
and familial nephritis; conversion
disorder, and malignancies.1-6
In this patient with erythromelalgia, history of arthralgia, hypocomplementemia, high ESR, along with
low CRP, ANA and anti dsDNA
Erythromelalgia can be
primary but also may
appear secondary to
another disease.
positivity, and complaints that affect
the life quality, there were suggestions of erythromelalgia secondary
to SLE, although the lupus criteria
were not completely fulfilled. However, the diagnosis of SLE is a dynamic process. Thus, a patient may
fulfill the SLE criteria in time.
The dramatic improvement of the
patient in response to steroid treatment
provided further support for SLE. It is
probable that, when the dose of steroid
is decreased or stopped completely, the
patient will develop other SLE manifestations. Thus, the patient should be
in close follow-up for other clinical
findings and complications of SLE.
Primary erythromelalgia can
be quite resistant to treatment, although the symptoms in the secondary type usually decrease with
the treatment of the underlying
condition.2 In primary erythromelalgia, various treatment modalities have been tried, such as:
• non-steroidal anti-inflammatory drugs (NSAIDs; aspirin,
indomethacin);
• beta-blockers;
• antihistamines; physical methods;
• vasodilators (nitroprusside);
• anticonvulsants (gabapentin,
carbamazepine);
• antidepressants (amytriptylline);
• antimitotics (carmustine);
• alpha-blockers (prazoscine,
doxazoscine);
• immunosuppressants;
• migraine medications;
• dapsone;
• prostaglandin E1 analogues;
• topical nitroglycerine;
• pentoxyphylline;
• dipyridamole;
• mexiletine HCl;
• quinine;
• muscle relaxants;
• surgical treatments, such as
sympathectomy.
The effectiveness of these medications is variable.1,4,7,8 However,
improvement in secondary erythromelalgia is usually observed with the
treatment of the underlying cause.2
In this patient, prostaglandin
E1 analogue, misoprostol, was
administered initially. However,
because secondary erythromelalgia in SLE has been reported to be
a cutaneous vasculitis responding
to oral prednisolone,9 the treatment was changed to prednisolone when SLE was considered
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case challenges
as the primary clinical condition.
Although steroids also have been
mentioned to be effective in idiopathic erythromelalgia, the rapid
response of the patient to steroid
treatment favored the diagnosis of
SLE associated erythromelalgia.
CONCLUSION
The presentation of this case demonstrates that when erythromelalgia is
considered in a pediatric patient, underlying clinical conditions should be
carefully sought before it is diagnosed
as idiopathic, and proper treatment
should be instituted accordingly.
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REFERENCES
1. Davis MD, O’Fallon WM, Rogers
RS, Rooke TW. Natural history of
erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol.
2000;136(3):330-336.
2. Mork C, Kvernebo K. Erythromelalgia
— a mysterious condition? Arch Dermatol. 2000;136(3):406-409.
3. Thompson GH, Hahn G, Rang M. Erythromelalgia. Clin Orthop Relat Res.
1979;144:249-254
4. Cohen JS. Erythromelalgia: new theories
and new therapies. J Am Acad Dermatol.
2000;43:841-847.
5. Herskovitz S, Loh F, Berger AR, Kucherov M. Erythromelalgia: association
with hereditary sensory neuropathy and
response to amitriptyline. Neurology.
1993;43(5 Pt 1):621-622.
6. Odom RB, James WD, Berger TG: An-
drew’s diseases of the skin. In: Clinical
Dermatology. 9th ed. Philadelphia: W.B.
Saunders; 2000:1012-1103.
7. Mork C, Salerud EG, Asker CL, Kvernebo K. The prostaglandin E1 analog
misoprostol reduces symptoms and microvascular arteriovenous shunting in
erythromelalgia-a double-blind, crossover, placebo-compared study. J Invest
Dermatol. 2004;122(3):587-593.
8. Suh DH, Kim SD, Ahn JS, et al. A case
of erythromelalgia successfully controlled by systemic steroids and pentazocine--is it related to a unique subtype of
neutrophilic dermatosis? J Dermatol.
2000;27(3):204-210.
9. Drenth JP, Michiels JJ, van Joost
T, Vuzevski VD. Secondary erythermalgia in systemic lupus erythematosus: comment. J Rheumatol.
1993;20(1):144-146.
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