PEDIA 32 4 March 2019

SKIN: NUTRITIONAL DERMATOSES Natakneng 2020

Dr. Corpuz Mariano Marcos State University
those with maple syrup urine disease, organic aciduria,
Outline: methylmalonic acidemia, biotinidase deficiency, essential fatty
1. Acrodermatitis Enteropathica acid deficiency, severe protein malnutrition (kwashiorkor), and
2. Essential Fatty Acid Deficiency cystic fibrosis.
3. Kwashiorkor
4. Cystic Fibrosis o For those individuals with acquired zinc deficiency, oral
5. Pellagra replacement with 0.5-1.0 mg/kg/24 hr of elemental zinc should
6. Scurvy be undertaken and the cause of underlying malnutrition should
7. Vitamin A Deficiency be addressed.
o Replacement for individuals with inherited acrodermatitis
NUTRITIONAL DERMATOSES enteropathica is with 3 mg/kg/24 hr of elemental zinc found in
zinc sulfate, gluconate, or acetate (i.e., 220 mg of zinc sulfate
ACRODERMATITIS ENTEROPATHICA contains 50 mg of elemental zinc).
o Zinc gluconate carries less risk of gastrointestinal distress.
• Acrodermatitis enteropathica is a rare autosomal recessive disorder
caused by an inability to absorb sufficient zinc from the diet. ESSENTIAL FATTY ACID DEFICIENCY
• The genetic defect is in the intestinal zinc-specific transporter gene
SLC39A4. • Essential fatty acid deficiency
• Initial signs and symptoms usually occur in the 1st few months of life, causes a generalized, scaly
often after weaning from breast milk to cow’s milk. dermatitis composed of
• Lymphocyte function and free radical scavenging are impaired. thickened, erythematous,
• Without treatment, the course is chronic and intermittent but often desquamating plaques.
relentlessly progressive. • Individuals may also show
• When the disease is less severe, only growth retardation and failure-to-thrive, growth
delayed development may be apparent. retardation, alopecia,
• Clinical Manifestations thrombocytopenia, and poor wound healing.
o The cutaneous eruption • The horny layer of the skin contains microscopic cracks, the barrier
consists of vesiculobullous, function of the skin is disturbed, and transepidermal water loss is
eczematous, dry, scaly, or increased.
psoriasiform skin lesions • The eruption has been induced experimentally in animals fed a fat-
symmetrically distributed in the free diet and has been observed in patients with chronic severe
perioral, acral, and perineal malabsorption, such as in short-gut syndrome, and in those
areas and on the cheeks, sustained on a fat-free diet or fat-free parenteral alimentation.
knees, and elbows. • Treatment
o The hair often has a peculiar, o Topical application of linoleic acid, which is present in sunflower
reddish tint, and alopecia of seed and safflower oils, may ameliorate the clinical and
some degree is characteristic. biochemical skin manifestations.
o Ocular manifestations include o Oral and/or parenteral therapy can also be considered.
photophobia, conjunctivitis, blepharitis, and corneal dystrophy o Appropriate nutrition should be provided.
detectable by slit-lamp examination.
• Diagnosis KWASHIORKOR
o The diagnosis is established by the constellation of clinical
findings and detection of a low plasma zinc concentration. • Severe protein and essential amino acid deprivation in association
o Levels of alkaline phosphatase, a zinc-dependent enzyme, may with adequate caloric intake can lead to kwashiorkor, particularly at
also be decreased. the time of weaning to a diet that consists primarily of corn, rice (or
o Histopathologic changes in the skin are nonspecific and include rice milk), or beans.
parakeratosis and pallor of the upper epidermis. • Children can be fed such a restricted diet for cultural reasons or
o The variety of manifestations of the syndrome may be because because of misdiagnosis on the part of the child’s parents or
zinc has a role in numerous metabolic pathways, including those healthcare providers of perceived food allergies.
of copper, protein, essential fatty acids, and prostaglandins, and • The serum zinc level is often deficient, and in some cases, skin
that zinc is incorporated into many zinc metalloenzymes. lesions of kwashiorkor heal more rapidly when zinc is applied
o A serum zinc level less than 50 μg/dL is suggestive, but not topically.
diagnostic, of acrodermatitis enteropathica. • Clinical Manifestations
• Treatment o Diffuse fine reddish brown
o Oral therapy with zinc compounds is the treatment of choice. scaling (enamel/flaky paint
o Plasma zinc levels should be monitored every 3-6 mo, however, sign) is the classic
to individualize the dosage. cutaneous finding.
o A syndrome resembling acrodermatitis enteropathica has been o In severe cases, erosions
observed in patients with secondary zinc deficiency resulting and linear fissures
from long-term total parenteral nutrition without supplemental develop.
zinc or to chronic malabsorption syndromes. o Nails are thin and soft, and
o A rash similar to that of acrodermatitis enteropathica has also hair is sparse, thin, and
been reported in infants fed breast milk that is low in zinc and in depigmented, sometimes displaying a “flag sign” consisting of

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PEDIA 32: SKIN: Nutritional Dermatoses
alternating light and dark bands that reflect alternating periods of
adequate and inadequate nutrition.
o The cutaneous manifestations may closely resemble those of
acrodermatitis enteropathica, however; edema of the extremities
and face (“moon facies”) and a protuberant abdomen (“pot
belly”) are key features uniformly observed in kwashiorkor.
o Blisters and scales develop, and the skin increasingly becomes
dry, rough, thickened, cracked, and hyperpigmented.
o Nicotinamide supplementation and sun avoidance are the
mainstays of therapy in pellagra.
SCURVY (VITAMIN C OR ASCORBIC ACID DEFICIENCY)

• Scurvy manifests initially as follicular

CYSTIC FIBROSIS hyperkeratosis, coiling of the hair on
the upper arms, back, buttocks, and
lower extremities.
• Other features are perifollicular
erythema and hemorrhage,
particularly on the legs and advancing
to involve large areas of hemorrhage; swollen, erythematous gums;
stomatitis; and subperiosteal hematomas.
• In children, the most common risk factors are behavioral or
psychiatric disease that results in poor nutrition.
• The best method of confirmation of a clinical diagnosis of scurvy is
a trial of vitamin C supplementation.

VITAMIN A DEFICIENCY

• Vitamin A deficiency manifests

initially as impairment of visual
• Protein-calorie malnutrition develops in 5-10% of patients with cystic adaptation to the dark.
fibrosis. • Cutaneous changes include
• Rash in infants with cystic fibrosis and malnutrition is rare but may xerosis and hyperkeratosis and
appear by age 6 mo. hyperplasia of the epidermis,
• The initial eruption consists of scaling, erythematous papules and particularly the lining of hair
progresses in 1-3 mo to extensive desquamating plaques. follicles and sebaceous glands.
• The rash is accentuated around the mouth and perineum and on the • In severe cases, desquamation may be prominent.
extremities (lower > upper).
• Alopecia may be present, but mucous membranes and nails are
uninvolved.

PELLAGRA

• Pellagra develops in patients with insufficient dietary intake or

malabsorption of niacin and/or tryptophan.
• Administration of isoniazid, 6-mercaptopurine, or 5-fluorouracil may
also produce pellagra.
• Hartnup disease, caused by a mutation in SLC6A19 that encodes a
neutral amino acid transporter, is a rare autosomal recessive
disorder that presents in infancy with a “pellagra-like syndrome” as
a result of decreased absorption of tryptophan.
• Clinical Manifestations
o Pellagra manifests as
edema, erythema, and
burning of sun-exposed
skin on the face, neck,
and dorsal aspects of the
hands, forearms, and
feet.
o Lesions of pellagra may
also be provoked by
burns, pressure, friction,
and inflammation.
o The eruption on the face
frequently follows a butterfly distribution, and the dermatitis
encircling the neck has been termed “Casal’s necklace.”

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