SHORT REPORT

Cutaneous presentation of kwashiorkor due to infantile

Crohn's disease
Luluah Al-Mubarak & Sultan Al-Khenaizan &
Talal Al Goufi
Received: 5 January 2009 / Accepted: 24 March 2009 / Published online: 8 April 2009
# Springer-Verlag 2009
Abstract Kwashiorkor is one of the severe forms of
protein-energy malnutrition. Many characteristic dermato-
ses can be seen in children suffering from kwashiorkor, and
some are pathognomonic. Here, we report an infant who
presented with diarrhea and skin signs of kwashiorkor, and
duodenal biopsy was consistent with Crohns disease. The
patient was treated with prednisolone administered orally in
a tapering course plus azathioprine, in addition to nutri-
tional supplementation. The general condition of the patient
quickly improved and his skin lesions completely resolved
within 2 weeks. Kwashiorkor is a serious potentially fatal
disease that occurs less often in developed countries leading
to low index of suspicion by physicians and pediatricians in
those regions. Occasionally, dermatologists have the rare
chance of alerting pediatricians to the diagnosis of kwash-
iorkor, thus making a difference in the care of this disease.
Keywords Kwashiorkor
.
Crohns disease
.
Protein-energy
malnutrition
Introduction
Kwashiorkor is a form of protein-energy malnutrition in
which the caloric intake remains adequate; the protein
amount is deficient while carbohydrates are in relative
excess [1]. Kwashiorkor is more prevalent between
6 months and 8 years of age. Skin findings are important
clue to the diagnosis and may predict worse prognosis [3].
Here, we report an infant who presented with diarrhea and
skin signs of kwashiorkor and later was proven to have
Crohns disease.
Case report
An 8-month-old full-term male baby presented to the King
Fahad National Guard Hospital, with history of prolonged
diarrhea and vomiting for 4 months, worsening in the last
2 weeks necessitating admission for investigation. Bowel
motions were 15 times per day, watery with no blood or
mucous. He was exclusively breast-fed and was weaned to
solids at the age of 6 months. There was no history of fever,
jaundice, or mouth ulcers. Past medical and surgical
histories were unremarkable. His immunization was up-to-
date with no similar illness in the family and no
consanguinity. Pregnancy and delivery were uneventful
with a birth weight of 3 kg. The mother was healthy and on
normal diet. On examination, the child was irritable and
severely dehydrated with generalized edema. His weight,
length, and head circumference were at 5th, 25th, and 10th
percentiles, respectively. Skin examination revealed multi-
Eur J Pediatr (2010) 169:117119
DOI 10.1007/s00431-009-0981-5
L. Al-Mubarak
:
S. Al-Khenaizan (*)
Division of Dermatology, Department of Medicine,
King Saud Bin Abdulaziz University,
King Fahad National Guard Hospital,
King Abdulaziz Medical City, P.O. Box 22490, Riyadh 11426,
Kingdom of Saudi Arabia
e-mail: khenaizans@ngha.med.sa
S. Al-Khenaizan
College of Medicine, King Saud Bin Abdulaziz University,
King Fahad National Guard Hospital,
King Abdulaziz Medical City,
Riyadh, Kingdom of Saudi Arabia
T. Al Goufi
Division of Gastroenterology, Department of Pediatric,
King Saud Bin Abdulaziz University for Health Sciences,
King Fahad National Guard Hospital,
King Abdulaziz Medical City,
Riyadh, Kingdom of Saudi Arabia
ple well-defined brownish peeling plaques over both
inguinal folds and anticubital fossae. Multiple circum-
scribed purpuric to brownish erosions were seen over the
scalp and flexures. Multiple brownish peeling erosions
were seen in the diaper area reminiscent of peeling paint
dermatitis (Fig. 1). On elbows and knees, there were
multiple sharply defined fissured brownish hyperpigmenta-
tion reminiscent of crazy pavement dermatitis (Fig. 2).
Hair examination revealed hypopigmentation at bases with
course texture. Mucous membranes and nails were normal.
Investigation revealed hypoalbuminemia of 21 g/l. Com-
plete blood count revealed hemoglobin of 61 g/l and white
blood cells of 14.610
9
/l. Erythrocyte sedimentation rate
was normal. Zinc level was 5.4 mol/l (normal 10.7
22.9 mol/l). Stool culture and sensitivity revealed normal
flora and enzyme-linked immunosorbent assay for rota
virus was negative. The stool tested negative for alpha-1-
antitrypsin. Two skin biopsies were obtained from the scalp
and the left groin revealed acanthosis, hyperkeratosis,
hypergranulosis, and focal pallor of keratinocyte consistent
with nutritional deficiency, thus confirming the clinical
suspicion of kwashiorkor and alerting the pediatrician to
this diagnosis. Patient underwent upper and lower GI
endoscopy, which did not reveal any gross abnormality.
Duodenal biopsy revealed moderate chronic active inflam-
mation containing mixed inflammatory cells within lamina
propria with multiple crypt microabscesses and granuloma
formation. The diagnosis of Crohns disease inducing
kwashiorkor was rendered. The patients nutritional status
was managed by total parenteral nutrition and slow
introduction of elemental diet with supplementation of
vitamins, zinc, and trace elements. Concurrently, predniso-
lone administered orally in a dose of 2 mg/kg/day weaned
in a course of 8 weeks. Additionally, the patient was started
on azathioprine in a dose of 0.5 mg/kg/day escalated
gradually to 2 mg/kg/day as prednisolone was tapered. The
patient general condition quickly improved and his skin
lesions totally resolved within 2 weeks. The patient is
currently off steroid and maintained on azathioprine given
orally.
Discussion
Protein-energy malnutrition encompasses a group of dis-
orders that includes marasmus and kwashiorkor with the
latter being the most prevalent nutritional deficiency in
humans [6]. It occurs when the caloric intake remains
adequate, the protein amount is deficient, and carbohydrates
are in relative excess [10]. It is more frequent in the
Fig. 1 Multiple well-defined brownish peeling plaques and erosions
over both inguinal folds and diaper area representing peeling paint
dermatitis. Note the scrotal edema
Fig. 2 Right outer elbow revealing sharply defined fissured brownish
hyperpigmentation representing crazy pavement dermatitis
Table 1 Specific and non-specific skin manifestations of kwashiorkor
Skin manifestations of kwashiorkor
Non-specific
Flacky paint dermatitis
Crazy pavement dermatitis
Flag sign of hair
Specific
Erosions in areas of friction
Vesicles or bullae
Hyperpigmentation
Hypopigmentation
Edema
Xerophthalmia
Cheilosis
Vulvovaginitis
118 Eur J Pediatr (2010) 169:117119
impoverished pediatric population, especially after the first
year of life, when breastfeeding is discontinued [6].
Patients with kwashiorkor generally maintain their sub-
cutaneous fat and possess a chubby appearance with a moon
face and they usually have an edematous rather than wasted
appearance [3]. Kwashiorkor is more prevalent between
6 months and 8 years of age, starting with gradual failure to
thrive, as well as irritability, apathy, and anorexia. Edema of
the extremities, muscle wasting, photophobia, diarrhea, and
hypoalbuminemia follow [3]. Cutaneous findings of kwash-
iorkor can be specific or non-specific as seen in Table 1.
The precise mechanisms for this disorder are not entirely
clear with many theories postulated. The classical theory
that kwashiorkor has a dietary cause was based on many
observations including development in those on diets with a
low protein/energy ratio [12]. Another theory relates
kwashiorkor to multiple nutritional deficiency including
essential amino acids, vitamins, and trace elements,
particularly zinc [1]. The most recent emphasized hypoth-
esis is an imbalance between the production of free radicals
and their safe disposal [4]. Free radicals, generated during
infections, damage tissues by affecting mitochondrial and
lipid membranes causing cell injury.
Other differential diagnoses to be considered for eczem-
atous, scaly, or desquamative dermatosis include inborn
errors of metabolism, acrodermatitis enteropathica, maras-
mus, and immunodeficiency disorders [3]. Underlying
factors for malabsorption including intestinal parasitosis,
cystic fibrosis, and other metabolic diseases should be kept
in mind [3]. Psychiatric and neurologic evaluation should
be pursued if suspected.
Inflammatory bowel disease (IBD) is a chronic, relaps-
ing, inflammatory disorder of the gastrointestinal tract. The
estimated incidence of IBD in the UK was 5.2 per 100,000
per year in children aged younger than 16 years [11].
Pediatric Crohns disease can be associated with many
nutritional deficiencies [8, 13]. On reviewing the literature,
we could find only one report of kwashiorkor complicating
Crohns disease. Tetsuharu et al. reported a case of Crohns
disease presented with severe marasmic kwashiorkor [9].
Treatment of kwashiorkor is directed to replace proteins
and correct the underlying disease [2]. As mentioned
earlier, zinc alone can adequately treat the skin findings of
kwashiorkor [5]. Mortality in classical kwashiorkor is
considered high with infections being the main cause of
death [12]. Skin rash might be a strong predictor of
mortality [7].
In conclusion, nutritional deficiencies can occur in
developed countries and may be initially overlooked with
catastrophic results. Characteristic dermatoses seen in young
children suffering from kwashiorkor may help in making the
diagnosis. Early recognition of these signs can help in early
diagnosis and potentially prevents complications.
Declaration The authors declare no funding source and no conflict
of interest.
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