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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-

d ate with n o similar i llness in the family and n o

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: [email protected]

S. Al-Khenaizan

College of Medicine, King Saud Bin Abdulaziz University,


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 Abdulaziz Medical City,

Riyadh, Kingdom of Saudi Arabia


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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 14.6109

/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 consistentwith 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. Thepatient 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 remainsadequate, 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


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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

faceand 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 ofthe 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 radicalsand 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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