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Zsolt Barta,
Zsofia Miltenyi, Laszlo Toth, Arpad Illes,
3rd Department of Medicine, Medical and Health Science Centre,
University of Debrecen, Debrecen, Hungary
Zsofia Miltenyi, Department of Pathology, Medical and Health
Science Centre, University of Debrecen, Debrecen, Hungary
Correspondence to: Zsolt Barta, MD., 3rd
Department of Medicine, University of Debrecen, Moricz Zs. krt. 22.,
4004 Debrecen, Hungary. barta@iiibel.dote.hu
Telephone: +36-52-453-337
Fax: +36-52-414-969
Received: 2004-08-29
Accepted: 2004-11-29
Abstract
The case of a 22-year-old patient with symptomatic hypokalemia
caused by rhabdomyolysis is presented as a rarely reported
complication of gluten-sensitive enteropathy
(GSE) and dermatitis herpetiformis Duhring. The patient’s
myopathy ceased on potassium supplementation and her other
complaints resolved while on gluten-free diet. Recovery was
otherwise uneventful with a rapid decline in serum CPK level. At the
time of her last follow-up a few months later, she was free of
symptoms and CPK remained stable. Patients with GSE may present with
hypokalemia in association with diarrhea and emesis, and if
potassium loss is rapid, rhabdomyolysis may occur.
© 2005 The WJG Press and Elsevier Inc. All rights reserved.
Key words: Gluten-sensitive enteropathy; Hypokalemia;
RhabdomyolysisAbstract
Barta Z, Miltenyi Z, Toth
L, Illes A. Hypokalemic myopathy in a patient with
gluten-sensitive enteropathy and dermatitis herpetiformis Duhring: A
case report. World J Gastroenterol
2005; 11(13): 2039-2040
http://www.wjgnet.com/1007-9327/11/2039.asp
INTRODUCTION
Symptomatic hypokalemia caused by diarrhea is uncommon in
adults. Rhabdomyolysis is an acute skeletal muscle disorder
characterized by altered integrity of the cell membranes of muscle
fiber cells and can be caused by a variety of factors whose nature
is not necessarily traumatic. Hypokalemia is one of the most common
metabolic causes, accounting for about 14-28% of rhabdomyolytic
syndromes. Isolated cases of hypokalemic myopathy have been
described in infections (salmonella, strongyloides, yersinia or
giardia) or in patients with malabsorption[1-4].
We present a case of hypokalemic myopathy as a rarely reported
complication in a patient with gluten-sensitive enteropathy and
dermatitis herpetiformis Duhring treated at our department.
CASE REPORT
A 22-year-old female patient with chronic diarrhea, frequent
vomiting, weight loss, fatigue and progrediating proximal type
muscle weakness in her extremities was admitted to our clinic. There
was no family history of renal or musculoskeletal disease nor had
she experienced any trauma, drug abuse or vigorous exercise. She
presented with bullous, intensely itching cutaneous lesions which
clinically resembled Herpes zoster or dermatitis herpetiformis (Duhring’s
disease) (Figure1).
Figure 1 Pruritic
eruptions with multiple, herpetiform, erythematous papulovesicles on
the forehead.
On physical examinations she was found
undernourished. Laboratory findings confirmed hypokalemia (2.1 mmol/L)
and high creatine phosphokinase, raised aldolase and lactic acid
dehydrogenase levels. Other biochemical and hematological laboratory
values were normal or in the normal range. She had no fever, fecal
cultures were all negative, and serology for viruses was also
negative, so no recent infection was to be suspected. There was
a symmetrical and dominantly proximal muscular weakness of
all four extremities. Mentation,
cranial nerves and the results of sensory examination were
normal. She developed symptoms suggestive of polymyositis,
which is an autoimmune disease characterized by muscle weakness
progressing over weeks or months with myalgia, muscle fiber
necrosis, regeneration and mononuclear cellular infiltrate and
myopathy, but further investigations (muscle biopsy, EMG and
immunological investigations) excluded the disease (Figure 2).
Figure 2 Hematoxylin
and eosin section of muscle shows no alterations.
Jejunal biopsy confirmed the typical histological
findings of GSE; both IgG and IgA type antigliadin, antiendomysial
and anti-tissue transglutaminase antibodies were positive (Figure
3).
Figure 3 Marsh
III-C, total villous atrophy, the villi are absent or rudimentary,
the mucosa resembles colonic mucosa.
The dermatologist confirmed Duhring’s disease
(diagnosis was made by skin biopsy). Both ECG and motor- as well as
sensory-nerve conduction
studies were normal but tests for urinary myoglobin were positive.
Blood-chemical findings on
admission and during hospitalization are shown in
Figure 4.
Figure
4 (PDF) Blood-chemical
findings on admission and during hospitalization (CPK and LDH).
The patient’s myopathy ceased on potassium
supplementation and her other complaints resolved while on
gluten-free diet. Recovery was otherwise uneventful with a rapid
decline in serum CPK level. At the time of her last follow-up 18 mo
later, she was symptom-free and CPK remained stable.
DISCUSSION
Dissolution of striated
muscle fiber with leakage of muscle enzymes,
myoglobin, potassium, calcium and other intracellular
constituents can occur in
anyone under particular circumstances and the consequences can be
severe and sometimes fatal[5].
There are no studies on
the incidence of rhabdomyolysis in
the literature and many mild cases probably go unrecognized.
Rhabdomyolysis is defined as an acute increase in serum
concentrations of
creatine kinase to more than five times the upper normal
limit after myocardial infarction has been excluded
as a cause (CK-MB fraction less than 5%). Visible
myoglobinuria occurs when
urinary myoglobin exceeds
250 µg/mL (normal <5 µg/mL), corresponding
to the destruction of more than 100 g of muscle.
The causes of rhabdomyolysis are diverse but all
lead to a critical increase in sarcoplasmic calcium and
intracellular damage by activation
of calcium-dependent proteases and phospholipases.
Risk is increased by pre-existing metabolic factors such as
hypokalemia, hypophosphatemia, and hyponatremia. Single
episodes are most commonly caused by infections (viral,
bacterial or other),
drugs, or physical factors such as compartment syndromes,
ischemia, reperfusion (including surgical procedures) and
pressure from hard surfaces in comatose patients.
We present here a rare case of rhabdomyolysis. No
symptoms of recurrent rhabdomyolysis were present suggesting that an
inherited disorder of muscle metabolism was highly improbable. In
addition, other precipitating factors (e.g., alcohol, trauma,
strenuous exercise or - less frequently - hypothyroidism, metabolic
myopathies or drugs) were either absent or could be excluded.
Two different possible interpretations for the
pathophysiology of the myopathy were raised at start-up:
polymyositis in which an infection played an indirect role as a
trigger; and infection per se, both of which were excluded. In our
patient, the clinical and histopathological features entirely met
the diagnostic criteria for celiac disease with the co-existence of
dermatitis herpetiformis.
Dermatitis herpetiformis or as formerly described
in the literature, Duhring’s disease is an important associated
disorder or complication of celiac disease which is manifested in
the form of a skin rash and has a typical onset in the teens or in
the third or fourth decades of life. Dermatitis herpetiformis is
often referred to as “celiac disease of the skin”, while CD is
referred to as “celiac disease of the gut.”
Patients with GSE may present with hypokalemia in
association with diarrhea and emesis; if potassium loss is rapid,
rhabdomyolysis may occur. As severe progressive neuromuscular
symptoms do not seem to result from acute
hypokalemia, unless there is preexisting potassium depletion,
chronic diarrhea or malabsorption may be crucial for the
development of hypokalemic myopathy. Malabsorption-associated
hypokalemic rhabdomyolysis may be a severe complication and should
be considered as a
potential cause of rhabdomyolysis
in GSE.
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Science Editor Li WZ Language
Editor Elsevier HK
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