The clinical symptoms disappear and serum zinc levels normalize after oral treatment with zinc. Acrodermatitis enteropathica from zincdeficient total. Acrodermatitis enteropathica ae presents in a very similar manner to other. Nov 09, 2011 acrodermatitis enteropathica ae is a disorder of zinc metabolism that can either be inherited or acquired.
An acrodermatitis enteropathica like syndrome occurred as a result of severe bowel disease and the use of zincdeficient hyperalimentation regimens. Acrodermatitis enteropathica ae is a rare dermatitis secondary to zinc deficiency most. The histopathology of the disorder is reported in detail. Pdf on feb 1, 1984, p s mortimer and others published acrodermatitis enteropathica find, read and cite all the research you need on.
Adult autoimmune enteropathy presenting initially with. The diagnosis is made by way of clinical presentation together with histopathology and laboratory tests. Medical definition of acrodermatitis enteropathica. The lack of zinc can cause skin inflammation with a rash pustular dermatitis around the mouth andor anus. We undertook a prospective study of 12 cases of acrodermatitis enteropathica in order to assess better the clinical and histologic features of the cutancous lesions as thev evolved over time. Acrodermatitis enteropathica ae is an autosomal recessive metabolic disorder characterized by periorificial and acral dermatitis, alopecia, diarrhea and hair loss tabanlioglu et al. The basic defect in acrodermatitis enteropathica a. Acrodermatitis enteropathica is a rare genetic autosomal recessive disorder. Acquired acrodermatitis enteropathica secondary to sleeve. This report details the ocular histopathology of a child who died before efficacious treatment was available. We undertook a prospective study of 12 cases of acrodermatitis. Symptoms of ae occur within the first few months after birth and tend to appear in nonbreastfed infants or in infants shortly after discontinuation.
Abstractin most of the few reports about the histopathology of acrodermatitis enteropathica, the changes have been considered to be nonspecific. Gianotticrosti syndrome papular acrodermatitis of childhood in the era of a viral recrudescence and vaccine opposition. It is a rare disorder caused by inherited or acquired zinc deficiency. Histopathologic features seen in gianotticrosti syndrome secondary to epsteinbarr virus. Acrodermatitis enteropathica an overview sciencedirect topics.
We present a case of severe acquired acrodermatitis enteropathica in a vegan adult female with multiple underlying comorbidities. Symptoms of zinc deficiency may include skin lesions, diarrhea, increased susceptibility to infections, night blindness, reduced taste and smell acuity, poor appetite, hair loss, slow wound healing, low. Acrodermatitis enteropathica and an overview of zinc metabolism. Zinc is an essential micronutrient with better bioavailability from human milk than cows milk or other infant formulae. Acrodermatitis enteropathica ae is a disorder of zinc metabolism that occurs in one of three forms. Acrodermatitis enteropathica is a rare disorder of zinc metabolism that occurs in one of two forms. Case report acrodermatitis enteropathica in an adult. In the last decade, increased number of reports have been published on the acquired form that presents in adulthood. Acrodermatitis enteropathica ae is a rare, bullous disorder that has a significant impact on the childs quality of life and is. Other articles where acrodermatitis enteropathica is discussed. Acrodermatitis enteropathica is an autosomal recessive metabolic disorder affecting the uptake of zinc through the inner lining of the bowel, the mucous membrane.
It is characterized by inflammation of the skin around bodily openings periorificial and the tips of fingers and toes acral, hair loss, and diarrhea. The inborn form of ae is a rare genetic disorder characterized by intestinal abnormalities that lead to the inability to absorb zinc from the intestine. It is characterized by acral dermatitis, alopecia, diarrhea and growth retardation. Pathology of acrodermatitis chronica atrophicans dr. A report is given on two siblings suffering from a. Other names sometimes used for this skin condition include gianotticrosti syndrome, papulovesicular acrodermatitis of childhood, infantile papular acrodermatitis, and acrodermatitis. Acrodermatitis enteropathica article about acrodermatitis. A permanent clinical remission could be achieved by. Part iii cases descriptions of acrodermatitis enteropathica. Read histopathological study of transient acrodermatitis enteropathica due to decreased zinc in breast milk, journal of cutaneous pathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. In most of the few reports about the histopathology of acrodermatitis enteropathica, the changes have been considered to be nonspecific. Acrodermatitis enteropathica genetic and rare diseases.
Acrodermatitis enteropathica is a lowincidence disease due to inherited or acquired zinc deficiency. The findings include corneal epithelial thinning and loss of polarity, anterior corneal scarring and loss. Unlike its inherited counterpart, acquired ae aae is often secondary to underlying pathologic or iatrogenic etiologies that interfere with. Histopathology of acrodermatitis enteropathica is very non specific and not an absolute criterion to diagnose this condition. Papular acrodermatitis of childhood is a characteristic response of the skin to viral infection in which there is a papular rash that lasts for several weeks. Acrodermatitis enteropathica, belonging to the family of necrolytic erythema and also referred as deficiency dermatoses, is a common finding in many nutritional deficiencies and is not specific.
Selenium deficiency, an essential trace element like zinc, may produce a cardiomyopathy and cutaneous. Acquired acrodermatitis enteropathica due to zinc deficiency in a patient with preexisting dariers disease. Acrodermatitis enteropathica is also called acrodermatitis enteropathy, primary zinc malabsorption syndrome, danboltcloss syndrome and brandt syndrome 68. Aug 21, 2019 acrodermatitis enteropathica is an autosomal recessive disorder occurring as a result of mutations in the slc39a4 gene located on band 8q24. It can also be related to deficiency of zinc due to other, i. The following case report of acrodermatitis enteropathica is unique. The symptoms of the disease usually start within the first few months when infants are weaned from breast milk. Acrodermatitis enteropathica ae is a rare autosomalrecessive disorder of zinc malabsorption, characterized by acral and periorificial dermatitis, alopecia, and diarrhea. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Bilateral cataract in acrodermatitis enteropathica. Some authors prefer to use the term acrodermatitis enteropathica only for the inherited disease. Corneal involvement with histochemical and electron micrographic studies. Ae is known to be caused by mutations of the slc39a4 gene.
The association between zinc deficiency and ae had not been. Ocular histopathology of acrodermatitis enteropathica. The acrodermatitis enteropathicalike syndrome jama. A case of acrodermatitis enteropathica longdom publishing sl. Acrodermatitis chronica atrophicans, the characteristic cutaneous manifestation of the late stage lyme borreliosis, typically occurs in elderly women. Acrodermatitis enteropathica and an overview of zinc. Acrodermatitis enteropathica in a patient with short bowel. The histopathology of acrodermatitis enteropathica. Acrodermatitis enteropathica ae is a rare dermatitis secondary to zinc deficiency most commonly seen as an inherited disease in infants. A skin biopsy at that time showed parakera tosis typical of acrodermatitis enteropathica fig. It usually presents with skin lesions, diarrhoea andor alopecia. We present a case of a 54yearold woman who developed characteristic skin lesions of acquired ae after zinc. We undertook a prospective study of 12 cases of acrodermatitis enteropathica in order to assess better the clinical and histologic features of the cutancou. Acquired acrodermatitis enteropathica or zincdeficiency dermatitis is the most common diagnosis than many practitioners realize with up to 10% of the patients in developed nations with the risk of zinc deficiency.
Acrodermatitis enteropathica ae is an autosomal recessive condition resulting in severe zinc deficiency. Histopathological examination of left thigh skin biopsy revealed. Acrodermatitis enteropathica ae in ophthalmology workup. The clinical presentation and histopathology of acquired and congenital acrodermatitis enteropathica are similar. Acrodermatitis enteropathica ae is the most representative zinc deficiency pathology resulting from a defect in the intestinal absorption or an inadequate.
Acrodermatitis enteropathica in an adult jama dermatology. The genetic defect has been mapped to 8q24 and the defective gene identified as slc39a4, which encodes the zinc transporter zip4. Histopathological study of transient acrodermatitis. The findings include corneal epithelial thinning and loss of polarity, anterior corneal scarring and loss of bowmans membrane, cataract formation, ciliary. Acquired ae is the result of decreased zinc intake, excessive zinc loss, or other malabsorptive processes. Acrodermatitis enteropathica is a rare autosomal recessive disorder of zinc deficiency. Maverakis e, fung ma, lynch pj, draznin m, michael dj, ruben b, et al. Acrodermatitis enteropathica ae is an autosomal recessive disorder with the clinical triad of acral dermatitis, diarrhea and alopecia. Both forms lead to the inability to absorb zinc from the intestine. Acrodermatitis enteropathica and an overview of zinc metabolism published online october 30, 2006. Small bowel small intestine acrodermatitis enteropathica. The patient is a 46yearold woman who has had a mild intermittent form of the disease most of her life.
We undertook a prospective study of 12 cases of acrodermatitis enteropathica in order to assess better the clinical and histologic features of. The deficiency is caused by a defect of dietary zinc absorption in the duodenum and jejunum. Acrodermatitis enteropathica nord national organization. Acrodermatitis enteropathica definition of acrodermatitis. The hair in acrodermatitis interopathicaa disease indicator. Acrodermatitis enteropathica or acquired zinc deficiency is the most likely diagnosis. Acrodermatitis enteropathica ae is a rare autosomal recessive disorder characterized by periorificial and acral dermatitis, alopecia, growth failure, gastrointestinal disturbance, and diarrhea. In this case, a diagnosis of acrodermatitis enteropathica was made, which was supported by detecting low serum zinc level 31. Acrodermatitis enteropathica is the clinical expression of congenital zinc deficiency and is now treated with supplemental zinc. Jul 31, 20 dermatopathology reference describes acrodermatitis enteropathica histopathology including histologic features and provides links to additional medical references. Hereditary acrodermatitis enteropathica in an adult. Emphasis is placed on early and adequate zinc replacement to effectively prevent or reverse this entity. Diiodohydroxyquin has resulted in a remission of the disease.
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