Skin lesions in acquired zinc deficiency due to parenteral nutrition.

The skin lesions seen in 10 patients who received parenteral nutrition during treatment of chronic enteropathy are described. All of these patients had a lowered serum zinc concentration. The skin lesions were similar to those seen in acrodermatitis enteropathica. After supplementation with zinc sulphate, the skin lesions disappeared completely. A decrease in the serum alkaline phosphatase level can be regarded as a sign of an impending zinc deficiency. Parenteral nutrition formulae should contain a sufficient amount of zinc.     

Acrodermatitis enteropathica in full-term breast-fed infant

BACKGROUND: Acrodermatitis enteropathica is a rare autosomal recessive disorder, caused by impaired absorption of zinc from the gastrointestinal tract. Symptoms of acrodermatitis enteropathica occur within the first few months after birth and tend to appear shortly after discontinuation of breast-feeding. We report a breast-fed infant with acrodermatitis enteropathica. CASE REPORT: A full term, 4-month-old girl, consulted in dermatologic department for persistent and refractory anogenital lesions since the age of 1 month, with progressive erythematous, vesiculous and squamous lesions, sometimes erosive in a peri orificial and acral pattern. She was calm and healthy baby. She was breast feeding. The diagnosis of acrodermatitis enteropathica was confirmed by decreased plasma zinc level (14 microg/100 ml). Breast milk zinc levels was low (46 microg/100 ml), as plasma zinc level of the mother (94 microg/100 ml). A genetic study showed that she was homozygous for the mutation, whereas her brother and parents were heterozygous. She was given zinc sulphate, and her condition has improved significantly. DISCUSSION: Acrodermatitis enteropathica is characterized by a characteristic clinical feature and the diagnosis is confirmed by decreased plasma zinc level. Acrodermatitis enteropathica in exclusively breast fed infant is rare, it was essentially reported in premature babies. Our case report is particular because it's concerning a full-term breast-fed infant, with zinc deficiency in breast milk and mother's decreased plasma zinc level.     

Acrodermatitis enteropathica

A 13-year-old girl presented with a history of red scaly plaques involving the chest, arms and legs beginning in infancy. Punch biopsy revealed psoriasiform hyperplasia and pallor of the epidermis. The patient's serum zinc level was 36 mug/dl [nl. 66-144 mug/dl]. A diagnosis of acrodermatitis enteropathica was established and the patient responded well to zinc replacement therapy. Acrodermatitis enteropathica is a rare autosomal recessive disorder caused by mutations in SLC39A4, which encodes the tissue-specific zinc transporter ZIP4.     

Acrodermatitis enteropathica with Pseudomonas aeruginosa sepsis

Acrodermatitis enteropathica is characterized by eczematous and scaly plaques on the face, scalp, acral, and anogenital regions. In addition to typical lesions, unusual prominent vesiculobullous lesions are also described. We report a full-term, 9-month-old boy who has acrodermatitis enteropathica and Pseudomonas sepsis. In this patient there were clinical findings of sepsis and eczematous vesiculobullous lesions on the periorificial and acral areas. Serum zinc level was extremely low. Pseudomonas aeruginosa was identified in cultures of blood and fluid which was aspirated from the bullous lesions. After oral zinc sulfate and intravenous antibiotic treatment his condition improved within 2 weeks.     

Zinc and skin: a brief summary

Zinc is an essential trace element that is an integral component of many metallo-enzymes in the body and thus serves many biological functions. The clinical presentation of zinc deficiency varies and depends on serum zinc level. Whereas a significantly low serum zinc level results in clinical features similar to acrodermatitis enteropathica, mild hypozincemia presents with a less characteristic appearance; hence it may be underdiagnosed. Recognition of various cutaneous lesions is required for suspecting and identifying cases of zinc deficiency. Although many laboratory tests are useful, therapeutic response in suspected cases remains the gold standard of diagnosis. Serum zinc estimation alone is not very reliable because disease activity may not necessarily correlate with serum zinc level. Zinc supplementation results in a rapid response and the skin lesions heal without permanent sequelae. However, pigmentary alterations may persist longer. Predisposing factors should be identified and corrected. This brief review summarizes the identification and management of clinical zinc deficiency.     

AN ACQUIRED FORM OF ACRODERMATITIS ENTEROPATHICA DUE TO LONGTERM LACTOSE-FREE MILK ALIMENTATION

A 4-month-old boy, fed on lactose-free milk for the treatment of intractable diarrhea for about 3 months, developed acrodermatitis enteropathica-like skin lesions. All the symptoms dramatically disappeared 2 weeks after switching from this milk to a general cow's milk formula, and his low serum zinc level also rapidly returned to normal. The zinc deficiency seen in this case was presumed to be closely related to longterm lactose-free milk alimentation. Acrodermatitis enteropathica, a human zinc deficiency, should be classified into two types, hereditary and acquired. The latter form can develop from intravenous hyperalimentation, longterm lactose-free milk alimentation and longterm penicillamine administration.     

Self-Limiting Acrodermatitis Enteropathica

A variant of acrodermatitis enteropathica is described that has its onset before weaning and clears when the child starts its normal solid diet. A pedigree with three interrelated families is reported where 10 children were afflicted with this variant. They had symptoms of hypozincemia for a brief period during infancy. At the time of this study, they were symptom-free and their serum zinc levels were found to be within normal limits. The term "self-limiting acrodermatitis enteropathica" is proposed for the variant. In one lactating mother, the mammary zinc secretion was determined and was found to be deficient and unresponsive to oral zinc supplements. The possible mode of inheritance is also discussed.     

Zinc deficiency with transitory acrodermatitis enteropathica in a boy of low birth weight

A premature male baby fed on his mother's milk developed zinc deficiency and a skin disorder inseparable from acrodermatitis enteropathica. Following zinc therapy the skin lesions healed. Later the treatment was withheld and no recurrence was seen during 30 months’ observation. The boy's zinc deficiency was thought to be due to a high requirement secondary to rapid growth, to poor zinc supply in food and, possibly, to inefficient zinc absorbtion.     

肠病性肢端皮炎1例

患儿男,3个月.鼻周、耳廓、肢端及臀部红斑、水疱、糜烂和结痂半个月,腹泻1周.血清锌45.02 μmol/L,明显低于正常值.诊断为肠病性肢端皮炎.采用葡萄糖酸锌口服配合外用药物治疗疗效明显.     

Acrodermatitis enteropathica in total parenteral nutrition caused by Crohn disease

A 26 years old patient was suffering from Morbus Crohn and showed the signs and symptoms of an acrodermatitis enteropathica. The beginning of the parenteral nutrition preceded the signs of acrodermatitis for 30 days. The serum zinc level was normal. After therapy with zinc aspartate the dermatitis healed within one week.     

ACUTE ZINC DEPLETION SYNDROME DURING PARENTERAL HYPERALIMENTATION

ABSTRACT: Zinc is an essential trace element whose malabsorption in early childhood may result in a skin disorder known as acrodermatitis enteropathica. Cutaneous lesions typical of acrodermatitis enteropathica have been described during total parenteral nutrition on zinc-deficient intravenous solutions in both adults and children. This condition has been named the “acute zinc depletion syndrome.” A case is described in which a patient, despite a zinc intake of double the daily requirement, manifested the acute zinc depletion syndrome during therapy with combined liquid diet plus parenteral hyperalimentation. Predisposing factors in this individual included a short bowel syndrome and a large oral load of calcium lactate. Zinc metabolism is reviewed with attention to alterations in disease and during hyperalimentation. The clinical manifestations, predisposing factors, therapy and prevention of the acute zinc depletion syndrome are discussed.     

A case of acrodermatitis enteropathica

Acrodermatitis enteropathica is a rare hereditary disorder affecting zinc metabolism that is characterized by dermatitis, alopecia, gastrointestinal disturbances, eye infections, and growth failure. We report a 17-month-old girl with acrodermatitis enteropathica. Physical examination showed a cutaneous eruption consisting of vesiculobullous and psoriasiform skin lesions symmetrically distributed in the perioral, acral, and perineal areas. Her plasma zinc level was decreased (75 micrograms/dl), but within the normal range (60.00-135.00 micrograms/dl). The patient was given zinc sulfate 50 mg/day. At the end of two months, she had significantly improved.     

Zinc salts in dermatology

Zinc is an essential trace element for the human organism. It acts like cofactor for the metalloenzymes involved in many cellular processes. Its anti-inflammatory activity, which is the basis of therapeutic use, other than acrodermatitis enteropathica, is not well known: production of cytokines, antioxidant activity. Its toxicity is very low, but marked at high doses during chronic administration by the risk of hypocupremia. It is not teratogenic and can be given during pregnancy. Its absorption, through the duodenum, is inhibited by excessive phytate intake. Maximum concentration is reached after 2 to 3 hours. It is widely distributed in the organism, mainly in muscles and bone. Excretion is predominantly digestive. Its spectacular effect in acrodermatitis enteropathica, through compensation of genetically determined malabsorption was discovered in 1973. Its usefulness in acne is based on the anti-inflammatory action and was first described with zinc sulfate, then with better tolerated gluconate. Many controlled studies have shown an efficacy on inflammatory lesions. Doses varied from 30 to 150 mg of elemental zinc and studies against cyclines have shown that minocycline has a superior effect; but zinc might be an alternative treatment when cyclines are contraindicated. To date we don't have convincing data for its use in other indications (leishmaniosis, warts, cutaneous ulcers). Tolerance at usual doses (200 mg of zinc gluconate or 30 mg of elemental zinc) is good. Major side effects are abdominal with nausea, vomiting, but are fleeting and dose dependent.     

A Diagnostic Challenge: A Case of Acrodermatitis Enteropathica without Hypozincemia and with Maternal Milk of Low Zinc Level

Abstract: Acrodermatitis enteropathica is a rare and distinct form of zinc deficiency with a requirement of life‐long zinc supplementation and inherited in a recessive manner. Transient nutritional zinc deficiency is also a well known condition mimicking acrodermatitis enteropathica like skin changes in preterm and term infants who are generally breastfed with a low level of zinc containing milk. Here, a 4‐month‐old male, term and fully breastfed acrodermatitis enteropathica case without hypozincemia and with maternal milk of low zinc level is presented.     

ACRODERMATITIS ENTEROPATHICA*

A case of acrodermatitis enteropathica is reported in which there was a clinical response to zinc therapy despite a normal pretreatment plasma zinc level. Unusual features are the absence of diarrhoea and the large doses of zinc required for control of the disorder.     

Acquired zinc deficiency disease of skin

Two patients, who were on long term parenteral hyperalimentation, developed skin lesions similar to those seen in acrodermatitis enteropathica. Both patients were treated with oral zinc sulphate and their skin lesions cleared completely. These patients are presented as an acquired zinc deficiency syndrome.     

Acrodermatitis enteropathica: an uncommon differential diagnosis in childhood – first description of a new sequence variant

An 11‐month‐old boy was brought to our clinic with superinfected, sharply‐defined, symmetrical, erythematous macules and vesicles, some with yellowish‐brownish crusts, on the cheeks, fingers, and in the diaper region. The suspected impetigo contagiosa had failed to respond to both topical antiseptic therapy and systemic antibiotics. Because of the unusual clinical picture and course, we measured the serum zinc level. A significantly reduced level of 2 μmol/l (normal range 9.2–18.4 μmol/l) was identified. Initial skin lesions had appeared one week after weaning (5th week after birth). Since the age of 8 months the infant had also had recurrent diarrhea. Two weeks after zinc‐histidine substitution, the diarrhea ceased and skin lesions slowly disappeared. Molecular genetic testing for the SLC39A4 (zinc transporter) gene revealed compound heterozygosity for the previously unidentified mutations c.1465_1474+4del (p.?) and c.295G>A (p.Ala99Thr). The parents are healthy heterozygous gene carriers. The same compound heterozygosity was later detected in the newborn brother of our patient shortly after birth. A zinc deficiency could therefore be identified and treated before symptoms occurred. The inherited autosomal recessive zinc transporter deficiency is termed acrodermatitis enteropathica. Lifelong zinc substitution is recommended. A differential diagnosis can be difficult because bacterial and fungal superinfection is common in zinc deficiency. Precise diagnosis requires testing family members for the gene.     

Transient zinc deficiency syndrome in a breast-fed infant due to decreased zinc in breast milk (type II hypozincemia of infancy): A case report and review of the literature

Type II hypozincemia of infancy is a rare, hereditary zinc deficiency occurring in infants while exclusively on breast feeding. It is caused by defective transfer of zinc into breast milk. Only a few dozen cases have been reported. A 6-month-old, full-term, breast-fed female infant presented with a 3-week history of erythematous to dusky red papules and annular plaques over the perioral and diaper area as well as the digits. The eruption was accompanied by poor appetite and irritable crying. Serum zinc was low (4.896 μmol/L, normal = 10.71?18.36 μmol/L) in the patient but was normal in the mother. Interestingly, the zinc level in the breast milk was very low (2.142 μmol/L; normal postpartum zinc = 18.36 μmol/L at 6 months). Histopathology of a skin biopsy specimen showed spongiotic psoriasiform dermatitis with pallor of superficial keratinocytes, consistent with deficiency disease. With oral zinc sulfate supplement, her skin lesions improved significantly within 4 days. Type II hypozincemia needs to be differentiated from the classical hereditary acrodermatitis enteropathica, which typically develops symptoms after weaning because of poor intestinal absorption of zinc in the affected infants. Mutations in zinc transporter genes have been detected in SLC39A4 (Zip4) and SLC30A2 (ZnT2), respectively, in classical acrodermatitis enteropathica and type II hypozincemia. No mutation was found in these two genes in the present pedigree. Therefore, the genetic defect in our patient might involve other zinc transporter genes.     

Malignant melanoma and dermatofibrosarcoma in a 60-year-old patient with lifelong acrodermatitis enteropathica

The life course of acrodermatitis enteropathica is recorded in a 62-year-old white man. Initially saved in infancy by breast-feeding and good medical care, later in his twenties he responded well to diiodohydroxyquinoline (Diodoquin) therapy, his only residua being dermatitis, hoarseness, and short stature. Subsequently untreated, this patient years later developed not only a dermatofibrosarcoma but also a large amelanotic malignant melanoma. Both were successfully excised. Subsequently, oral zinc therapy initiated for the first time cleared his acrodermatitis, which had been present for 60 years. It is suggested that this patient's malignancies developed as a result of an immune deficiency state typically found in acrodermatitis enteropathica. On this basis, acrodermatitis enteropathica may be viewed as having a malignant potential over the long term. The zinc-dependent nature of the immune deficit, however, suggests that lifelong daily zinc supplementation is an appropriate prophylactic measure.     

Acrodermatitis enteropathica: case report and review of the literature

Acrodermatitis enteropathica (AE) is a rare hereditary disorder caused by impaired absorption of zinc from the gastrointestinal tract. It is characterized by acral and periorificial dermatitis, alopecia, and diarrhea. Symptoms usually begin on weaning from breast or formula feeding. We report a full-term, 21-month-old boy with typical skin lesions and decreased plasma zinc level (12 micro g/dl). The patient was given zinc sulfate 40 mg/day and at the end of 1 month his condition had improved significantly. After reviewing the literature we emphasize the important role of zinc in human metabolism and the difference between AE and acquired zinc deficiencies.