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Case Report
Dermatology 1995;190:232-234
J P J l Drentlf
J J Michielsh
T. Van J o o s f
V. D. Vuzevskiil
Department o f Medicine, Division o f
General Internal Medicine, University
Hospital St. Radboud, Nijmegen, and
Departments o f
Hematology,
Derm atology and
Clinical Pathology, University Hospital,
Erasmus University, Rotterdam,
The Netherlands
Secondary Erythermalgia
Associated with an Autoimmune
Disorder of Undetermined
Significance
Key Words
Abstract
E rytherm algia
A u to im m u n ity
A dult
A 50-year-old female patient is described with an acquired, persisting and yet
incurable erytherm algia featured by sym m etric burning pain and red congestion
o f the extremities secondary to cutaneous vasculitis, A w eakly positive antinuclear antibody titer and high titers o f antibodies against gastric parietal m u c o sa
cells pointed to an underlying but unclassiliable au to im m u n e disorder. It is c o n
cluded that histopathology o f lesional skin contributes to the differential d ia g n o
sis of primary and secondary erytherm algia.
Vasculitis
Introduction
E rythrom elalgia and erytherm algia are two distinct clinical syndrom es o f burning painful and red congested
extrem ities [ 1 - 3 ] . The curable variant erythromelalgia is
causally related to throm bocythem ia and results from platelet-m ediated arteriolar inflammation and thrombosis. Aspirin com pletely alleviates all sym ptom s by irreversible inhibition of platelet cyclooxygenase activity and aggregation
[4-7].
T h e incurable variant of primary erytherm algia is a rare
congenital disorder which spontaneously arises in childhood or adolescence as bilateral sym m etrically burning
extrem ities in the absence of detectable disease [8, 9]. The
histopathology in prim ary erytherm algia is nonspecific and
m ay sh o w only slight perivascular infiltration with ly m p h o
cytes [9].
S eco n d ary erytherm algia usually arises at the adult age
either in association with cutaneous vasculitis or with the
use o f drugs. Secondary erytherm algia in these conditions
Received:
May 26, 1994
Accepted:
July i l , 1994
responds to treatm ent o f the underlying d iso rd er or d isc o n
tinuation o f the incrim inated drug 11, 8, 10, 111.
There also appears to be a persisting and incurable form
of erytherm algia arising at the adult age ( 12|. H ow ever, the
histopathology o f incurable erytherm algia at the adult age
has never been investigated. We have had the opportunity
to study an adult w o m an w ho developed incurable erythermalgia in the setting o f an unclassiliable a u to im m u n e disorder. E vidence is presented that the specific clinical m anifestations and the histopathological findings contribute to the
differential diagnosis o f prim ary and secondary erytherm algia.
Case Report
Since 1984, a 50-year-old woman has suffered from a unilateral
painful erythema on the left heel, progressively extending to the foot,
ankles and lower leg. In 1986, an erythema o f the right foot, ankle and
lower leg was noted. The condition progressed to bilateral burning
distress and red sw elling o f both feet, ankles and lower legs. Since
Dr. J.J, Michiels
■
Department ol‘ Hematology
University Hospital D ijkziyt
Dr. Molemvaterplein 40
N L -3 0 1 5 GD Rotterdam (The Netherlands)
S. Karyer AO, Basel
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legs became a symmetric feature. At this stage, the symptoms superficially resembled the features of primary erythermalgia [9], As in primary erythermalgia, the lesions had
a bilateral distribution, there was aggravation by excercise
and elevation of the ambient temperature worsened the
symptoms. Also, the slight relief by cold, rest and elevation
of the affected extremities and the resistance to treatment fit
with the diagnosis of primary erythermalgia. However, particular symptoms are against the diagnosis of primary erythermalgia and are more in agreement with a secondary
variant: the complaints and signs started as a unilateral process and spread subsequently to both feet and hands,
whereas in primary erythermalgia the discomfort always
starts bilaterally as a symmetric process. In contrast to a
typical fluctuating course with frank exacerbations and
symptom-free periods in primary erythermalgia, the symptoms in our patient became a constant and chronic clinical
feature without any fluctuations. Moreover in our patient
symptoms started at adult age unlike primary erythermalgia. Histopathology of erythermalgic skin showed perivascular inflammatory infiltrates of lymphocytes and plasma
cells consistent with (peri)vasculitis, being different iron
fibromuscular proliferation and thrombotic occlusions ii
erythromelalgia [7J.
This pattern is similar to the histopathology ot reportei
cases in erythermalgia secondary to vasculitis, systemi<
lupus erythematosus in particular [10, 11], Laboratory test
ing in our patient revealed a high antinuclear antibody tite
and antibodies against gastric parietal mucosa cells whicl
suggests a coexisting, but not yet clearly defined, autoim
mune disorder. It is not clear whether the autoimmunity ii
our patient establishes a significant pathogenetic factor o
even a link.
In this respect, it should be noted that antinuclear anti
bodies can be found in 10-37% of the healthy elderly an<
parietal cell antibodies are seen in up to 16% of the norma
population [14, 15]. In another case of severe adult-onse
erythermalgia, no autoantibodies were detectable [12], Per
sisting (secondary) erythermalgia arising at the adult age i,
very rare, and treatment is difficult. Histopathologica
examination of lesional skin contributes to the differentia
diagnosis of primary and secondary erythermalgia.
References
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Drenth/Michicls/Van Joost/Vuzevski
Erythermalgia in Adults

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