ARTICLE
Auteur(s) : Sónia Coelho1,
Bárbara Fernandes2, Fernanda Rodrigues3, José
Pedro Reis1, Ana Moreno1, Américo
Figueiredo1
1Department of Dermatology, Hospital da Universidade,
Praceta Mota Pinto, P.3000-075 Coimbra, Portugal
2Department of Dermatology, Infante D. Pedro Hospital,
SA, Aveiro
3Paediatric Hospital, Coimbra, Portugal
accepté le 30 Août 2005
Transient symptomatic zinc deficiency (TSZD) is a disease
clinically indistinguishable from idiopathic acrodermatitis
enteropathica, although with different pathologic mechanisms. It
occurs in the first six months of life, usually in infants with
increased zinc requirements and/or inadequate diet concentrations
of this oligoelement. The majority of reported cases in breast-fed
premature and also full-term infants were associated to low zinc
values in maternal milk [1-15]. This report describes a rare case
of clinical zinc deficiency that developed in spite of normal zinc
values in maternal milk [16].
Case report
A 5-month-old-male infant was referred to the dermatology
department with an acrodermatitis enterophatica-like skin eruption.
He was born prematurely at 27 weeks weighing 1,220 g. His neonatal
course was complicated by respiratory distress syndrome, sepsis due
to Staphylococcus epidermidis and a grade I subependimary
haemorrage. Nutritional support initially included total parenteral
nutrition which provided trace elements including zinc and was
removed on day thirteen. Exclusive oral feeding with expressed
breast milk began on day two. A protein mineral supplement
containing zinc was added to maternal milk during the five weeks of
hospitalization. At discharge, the baby presented a normal clinical
exam and had been on full oral breast-feeding for a few days.
During the second month of life the infant developed an
erythematous, exfoliating rash in the genital and peri-anal areas
(( figure 1 ))
which responded partially to clotrimazole. At the age of four
months the eruption spread, also involving the face in a
peri-orificial distribution (nasal, oral, auricular) (( figure 2 )) and the hands
(peri-ungueal). The erythematous eruption was symmetrical, sharply
demarcated and associated with erosions, crusting and exudate. At
the time of our observation the infant was five months old; he had
been irritable for the last two weeks and had no diarrhoea,
alopecia or fever. He had been fed exclusively with breast milk
until the fourth month of life and had had no relevant medical
problems since he left the neonatal care unit. There were no
similar family cases, his 10-year-old brother was born at full-term
and was also breast-fed.
The clinical diagnosis of zinc deficiency was confirmed by a low
serum zinc level [0.3mg/L (N = 0.7-1.20 mg/L)]. The mother had
normal serum [0.7 mg/L (N = 0.7-1.50 mg/L)] and milk [1.2 mg/L (N =
0.17-3.02 mg/L)] zinc concentrations. The remaining routine
laboratory investigation, namely alkaline phosphatase values, was
normal.
Treatment with a zinc sulphate solution was instituted, at a
dosage of 4 mg/kg once a day. The patient was also treated with
topical miconazole due to oral candidiasis. Skin lesions markedly
improved in one week and cleared in three weeks. Two months after
the beginning of treatment, the infant had a normozincemia, with
maintenance of the mother’s serum and milk normal zinc values. He
still presented mild and intermittent genital and peri-anal lesions
that ceased to appear during the next month. The infant maintained
the same zinc sulphate dosage during the twenty-two months of
treatment. During follow-up he remained without lesions and with
normal serum zinc levels. Observed three, six and twelve months
after treatment interruption, he had no recurrence of the
dermatosis and maintained a good general condition and mental
development.
Discussion
Transient symptomatic zinc deficiency is a self-limited disease
mainly observed in breast-fed infants. The increase in zinc
requirements is the preponderant risk factor, justifying the high
proportion of reported cases in premature infants [1-11, 16],
although some cases were also described in full term babies [6,
12-15]. A low zinc level in maternal milk is an important cofactor.
This defect seems to result from a rare abnormality of zinc uptake
from plasma by the mammary gland, probably due to a deficiency or
malfunction of a zinc binding ligand [3]. Two thirds of
mother-foetus zinc transfer occurs in the last ten weeks of
gestation [11], therefore premature infants are prone to a negative
zinc balance for up to 60 days [6, 13]. Besides the inadequate
stores, this deficit is also secondary to a poor intestinal
absorption, an increased urinary and intestinal secretion and a
rapid growth with increased zinc demands [5, 6, 16]. Full term
infants, especially those with neonatal complications, can also
have a negative balance. Even though prematurity and defects in
mammary zinc secretion are the most frequently observed causes of
TSZD in children, disorders with intestinal malabsorption are other
possible etiologic factors [17]. In rare cases, TSZD is the first
symptom of cystic fibrosis [18]. The pancreatic exocrine
dysfunction induces a hypoproteinaemia and a decrease in zinc
absorption by the gut, justifying the appearance of the eruption.
Besides the cutaneous lesions, observed in all patients, TSZD
can be accompanied by diarrhoea, irritability, alopecia, low grade
fever, conjunctivitis and, rarely, seizures. The eruption presents
as sharply demarcated erythemato-vesiculous lesions associated with
erosions, crusting and exudate, in a peri-orificial and acral
distribution. The oral mucosa can also be affected, with frequent
concomitant candidiasis [6, 16]. Zincemia is low in all infants,
and rapidly normalizes after oral zinc supplementation. Some cases
present [6, 9, 12, 15, 16] a diminished serum alkaline phosphatase
level which is a late and moderately sensitive indicator of zinc
deficiency.
Different zinc therapeutic regimens have been applied, but some
authors outline that 1 mg/kg/d is sufficient [5, 6] and that
treatment can be stopped shortly after alimentary
diversification.
Idiopathic acrodermatitis enteropathica is a hereditary
autosomal recessive disease due to the deficit or inexistence of an
intestinal zinc ligand. Because maternal milk has a zinc
bioavailability superior to cow’s milk, breast feeding has a
protective role, justifying the clinical presentation after its
interruption [19] and the need for unending zinc supplementation in
most cases. There are reported cases without hypozincemia
apparently related to an abnormal zinc-binding ligand that impairs
zinc release to the tissues without affecting its absorption by the
intestinal mucosa [17, 20].
The absence of general complaints can hamper the differentiation
of diaper and seborrheic dermatitis from TSZD [5]. Other
differential diagnoses include impetigo, chronic mucocutaneous
candidiasis, biotin and other decarboxylase deficiencies, organic
acidurias, essential fatty acid deficiencies, atopic dermatitis and
also contact dermatitis [5, 6, 19].
There is a growing concern about zinc involvement in children’s
development due to its participation in several metabolic and
immunologic mechanisms. The clinical manifestations of zinc
deficiency can be subtle, reason why some authors suggest, in all
at-risk babies, the surveillance of zinc values [5] and a superior
zinc concentration in the regularly administered supplements [16].
In fact in our case the infant developed TSZD despite maternal milk
with normal zinc content and a milk fortifier containing zinc.
Because the manifestations of zinc deficiency may be easily
misdiagnosed, knowledge of the several possible causes and a high
degree of suspicion are essential to improve its recognition in
affected children.
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