Barta, Zsolt

Illés, Árpád

Miltényi, Zsófia

Tóth, László

World Journal of Gastroenterology

University of Debrecen Electronic Archive

• CASE REPORT •
Hypokalemic myopathy in a patient with gluten-sensitive
enteropathy and dermatitis herpetiformis Duhring: A case report
Zsolt Barta, Zsofia Miltenyi, Laszlo Toth, Arpad Illes 
ELSEVIER
PO Box 2345, Beijing 100023, China                                                                                                                                                          World J Gastroenterol  2005;11(13):2039-2040
www.wjgnet.com                                                                                                              World Journal of Gastroenterology  ISSN 1007-9327
wjg@wjgnet.com                                                                                                                                                                                       © 2005 The WJG Press and Elsevier Inc. All rights reserved.
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).
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).
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).
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.
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
2040           ISSN 1007-9327     CN 14-1219/ R     World J Gastroenterol     April 7, 2005   Volume 11   Number 13
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.
REFERENCES
1 Guardino JM, Hix JK, Seidner D. A case of hypokalemia and
rhabdomyolysis in a patient with short bowel syndrome. JPEN
J Parenter Enteral Nutr 2003; 27: 305
2 Williams SG, Davison AG, Glynn MJ. Hypokalaemic
rhabdomyolysis: an unusual presentation of coeliac disease.
Eur J Gastroenterol Hepatol 1995; 7: 183-184
3 Ertekin V, Selimoglu MA, Tan H, Kilicaslan B. Rhabdomyolysis
in celiac disease. Yonsei Med J 2003; 44: 328-330
4 Cervello A, Alfaro A, Chumillas MJ. Hypokalemic myopathy
induced by Giardia lamblia. N Engl J Med 1993; 329: 210-211
5 Criner JA, Appelt M, Coker C, Conrad S, Holliday J.
Rhabdomyolysis: the hidden killer. Medsurg Nurs 2002; 11:
138-143
Science Editor Li WZ  Language Editor Elsevier HK
Figure 1  Pruritic eruptions with multiple, herpetiform, erythema-
tous papulovesicles on the forehead.
Figure 2  Hematoxylin and eosin section of muscle shows no
alterations.
Figure 3  Marsh III-C, total villous atrophy, the villi are absent or
rudimentary, the mucosa resembles colonic mucosa.
Figure 4  Blood-chemical findings on admission and during hospi-
talization (CPK and LDH).
IU
/L
1    2   3    4   5    6   7    8    9   10  11  12  13
                             Time (d)
18 000
16 000
14 000
12 000
10 000
8 000
6 000
4 000
2 000
0
CPK
LDH


Hypokalemic myopathy in a patient with gluten-sensitive enteropathy and dermatitis herpetiformis Duhring: a case report

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Hypokalemic myopathy in a patient with gluten-sensitive enteropathy and dermatitis herpetiformis Duhring: a case report

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