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Painful swollen and erythematous hands and feet.

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Vol. 39, No. 10, October 1996, pp 1761-1762
8 1996, American College of Rheumatology
Figure 1. Erythema of the patient’s extremities.
Case presentation
A 27-year-old woman presented with a history of
intermittent swelling, erythema, and burning in her
hands and feet, which had begun during pregnancy 4
years previously. During these symptomatic episodes,
John J. Hart, MD, University of Kansas School of Medicine-
the fingertips, palms, toes, and soles of the feet would
become red and swollen (Figure l), and she would
experience warmth and pain. She had been diagnosed as
having recurrent viral exanthemas. The symptoms were
brought on if she became too warm in general, if her feet
or hands specifically became too warm, or with excessive
use of her feet or hands. The episodes lasted from
minutes to hours. The feet were less frequently involved
than the hands, but occasionally there would be joint
stiffness in the feet, which did not occur in the hands.
Relief was obtained by elevation of the extremities and
by ice water immersion. Aspirin at doses of almost 4 gm
per day occasionally gave partial relief.
The patient’s earlier medical history was significant for mitral valve prolapse, paroxysmal atrial tachycardia (PAT), and hypertension. She took oral verapamil daily to control the PAT and hypertension. A
cesarean section with the pregnancy 4 years before,
when her symptoms had begun, was the only surgery she
had undergone. She consumed alcohol socially and
smoked 1 pack of cigarettes per day.
Results of a complete blood cell count, platelet
count, urinalysis, and immunologic survey were normal.
A skin punch biopsy revealed arteriolar intimal fibromuscular proliferation.
Atenolol was prescribed in place of the verapamil, with good blood pressure control and decreased
PAT symptoms. The recurrent dermatologic condition
gradually responded to withdrawal of verapamil treatment. The patient had been switched to atenolol, which
resulted in good blood pressure control and decreased
symptoms of PAT. Complete resolution of symptoms
was eventually achieved.
What is your diagnosis?
(a) Erythromelalgia
(b) Primary erythermalgia
(c) Secondary erythermalgia
Diagnosis: Secondary erythermalgia
Address reprint requests to John J. Hart, MD, University of
Kansas School of Medicine-Wichita, Department of Family and
Community Medicine, 855 North Hillside, Wichita, KS 67214.
Submitted for publication August 30, 1995; accepted in
revised form April 15, 1996.
Erythromelalgia is diagnosed on the basis of 3
features: 1) platelet counts exceed 400 x 1og/liter; 2)
Table 1. Causes of secondary erythermalgia
Polyarteritis nodosa
Thromboangiitis obliterans
Rheumatoid arthritis
Hereditary sensory neuropathy
Cutaneous vasculitis
Systemic lupus erythematosus
Diabetes mellitus
Pernicious anemia
Lichen sclerosus et atrophicus
Upper respiratory tract viral infections (poxviruses)
Venous insuffiency
Spinal cord disease
Multiple sclerosis
Hereditary nephritis
Various drugs (verapamil, nifedipine, bromocriptine, nicardipine,
low-dose aspirin gives rapid relief of symptoms which
lasts for days; and 3) there is histopathologic evidence of
arterioles with fibromuscular proliferation and occasional thrombotic occlusion (1). The symptoms are often
distributed asymmetrically and unilaterally. There is
preferential involvement of the fingertips, 1 or more
toes, or the sole of the forefoot. The symptoms of
erythremia, warmth, congestion, and pain may progress
to ischemic acrocyanosis and, in the most severe cases, to
frank gangrene. The response to low-dose aspirin is
rapid (hours) and long-lasting (days), and the occurrence of this response has been considered pathognomonic for erythromelalgia (2).
Erythromelalgia is associated with polycythemia
Vera and variants of chronic myeloproliferative disorders. Evidence of a myeloproliferative disorder only
rarely is found before symptoms of erythromelalgia
occur; the symptoms of erythromelalgia typically antedate the onset of myeloproliferative disease by a median
of 2.5 years (2). Ongoing screening in patients with
erythromelalgia symptoms, therefore, is recommended.
The response of erythromelalgia to treatment of an
underlying myeloproliferative disorder is variable.
Primary erythermalgia requires 6 criteria for
diagnosis (3): 1) hands or feet are erythematous, warm,
swollen, and painful during attacks; 2) attacks are bilateral or symmetric in the hands or feet; 3) attacks are
brought on or exacerbated by exercise, standing, or
exposure to warmth; 4) elevation and exposure to cold
give relief; 5) attacks are refractory to medical treatment;
and 6) there is no associated disease state or precipitating
medication (3). Primary erythermalgia has no associated
disorder, specific histopathologic findings, or treatment.
It is an autosomal dominant trait that typically manifests
at a young age, often by puberty (4). In comparison with
erythromelalgia, the symptoms may extend to the lower
legs, but show relative sparing of the toes. There is no
progression to peripheral ischemia or gangrene.
Secondary erythermalgia presents with the same
clinical picture as primary erythermalgia, but develops
secondary to one of many drugs or disorders (Table 1).
Withdrawal of the precipitating medication or treatment
of the underlying disorder usually causes the symptoms
to resolve, although this may take some time. Skin
biopsy findings are not specific, although erythermalgia
secondary to systemic lupus erythematosus has been
associated with cutaneous vasculitis (5).
The patient described herein had 3 features
known to be associated with secondary erythermalgia: 1)
onset during pregnancy, 2) hypertension, and 3) verapamil treatment (6). Unusual was the fact that while the
patient's condition did not respond to aspirin consistently or completely, the skin biopsy did show arteriolar
fibromuscular intimal proliferation, which is usually seen
with erythromelalgia. Also unusual was the recurrence
of symptoms with a second pregnancy 2 years after the
presentation reported herein. The syndrome resolved
completely within 1 year of delivery. The platelet count
was monitored for 4 years after the initial resolution of
symptoms (which included the second pregnancy and its
associated recurrence of symptoms) and was never
higher than 350 X lO'/liter.
The literature is confusing in mixing of terminology
concerning these 3 clinically similar syndromes. The classifications as outlined herein are the emerging standards. A
working knowledge of the definitions of these 3 syndromes
is critical for appropriate diagnosis and treatment.
1. Kurzrock R, Cohen PR: Erythromelalgia: review of clinical characteristics and pathophysiology. Am J Med 91:416-422, 1991
2. Kurzrock R, Cohen PR: Erythromelalgia and myeloproliferative
disorders. Arch Intern Med 149:105-109, 1989
3. Drenth JP, Michiels JJ: Three types of erythromelalgia. BMJ
301:454-455, 1990
4. Finley WH, Lindsey JR, Fine JD, Dixon GA, Burbank MK. Autosoma1 dominant erythromelalgia. Am J Med Genet 42:310-315, 1992
5. Drenth JP, Michiels JJ, van Joost T, Vuzevski VD: Secondary
erythermalgia in systemic lupus erythematosus. J Rheumatol 20:
144146, 1993
6. Drenth JP, Michiels JJ, van Joost T, Vuzevski VD: Verapamilinduced secondary erytherrnalgia. Br J Dermatol 127:292-294,1992
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painful, feet, swollen, hands, erythematous
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