Summary: Inflammation involving the skin of the extremities, especially the hands and feet. Several forms are known, some idiopathic and some hereditary. The infantile form is called Gianotti-Crosti syndrome.

Top Publications

  1. Maverakis E, Lynch P, Fazel N. Acrodermatitis enteropathica. Dermatol Online J. 2007;13:11 pubmed
    ..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...
  2. Dufner Beattie J, Weaver B, Geiser J, Bilgen M, Larson M, Xu W, et al. The mouse acrodermatitis enteropathica gene Slc39a4 (Zip4) is essential for early development and heterozygosity causes hypersensitivity to zinc deficiency. Hum Mol Genet. 2007;16:1391-9 pubmed
    The human Zip4 gene (Slc39a4) is mutated in the rare recessive genetic disorder of zinc metabolism acrodermatitis enteropathica, but the physiological functions of Zip4 are not well understood...
  3. Park C, Lee M, Kim H, Lee G, Park J, Cinn Y. Congenital zinc deficiency from mutations of the SLC39A4 gene as the genetic background of acrodermatitis enteropathica. J Korean Med Sci. 2010;25:1818-20 pubmed publisher
    b>Acrodermatitis enteropathica (AE) is an autosomal recessive disorder with the clinical triad of acral dermatitis, diarrhea and alopecia. AE is known to be caused by mutations of the SLC39A4 gene on the chromosome band 8q24...
  4. Perafán Riveros C, França L, Alves A, Sanches J. Acrodermatitis enteropathica: case report and review of the literature. Pediatr Dermatol. 2002;19:426-31 pubmed
    b>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...
  5. Mullegger R, McHugh G, Ruthazer R, Binder B, Kerl H, Steere A. Differential expression of cytokine mRNA in skin specimens from patients with erythema migrans or acrodermatitis chronica atrophicans. J Invest Dermatol. 2000;115:1115-23 pubmed
    ..In contrast, acrodermatitis chronica atrophicans, the typical cutaneous manifestation of late Lyme borreliosis, is a chronic skin ..
  6. Li C, Yan S, Shen D, Li Q, Shao J, Xue C, et al. One novel homozygous mutation of SLC39A4 gene in a Chinese patient with acrodermatitis enteropathica. Arch Dermatol Res. 2010;302:315-7 pubmed publisher
    b>Acrodermatitis enteropathica, a rare autosomal recessive disease, manifests as periorificial and symmetrical acral dermatitis, alopecia, and diarrhea due to insufficient zinc uptake by the intestine...
  7. Kiszewski A, De Villa D, Scheibel I, Ricachnevsky N. An infant with acrodermatitis continua of Hallopeau: successful treatment with thalidomide and UVB therapy. Pediatr Dermatol. 2009;26:105-6 pubmed publisher
    b>Acrodermatitis Continua of Hallopeau is a rare, chronic, recurrent disorder classified as a form of pustular psoriasis, and most cases affect one or two digits...
  8. Török L, Husz S, Ocsai H, Krischner A, Kiss M. Pemphigus vegetans presenting as acrodermatitis continua suppurativa. Eur J Dermatol. 2003;13:579-81 pubmed
    The authors present a 51-year-old patient with clinical signs of acrodermatitis continua suppurativa of the toes...
  9. Pascual J, Matarredona J, Mut J. Acrodermatitis enteropathica-like dermatosis associated with ornithine transcarbamylase deficiency. Pediatr Dermatol. 2007;24:394-6 pubmed
    ..Patients can become deficient in several amino acids, either through the low-protein diet or directly through the primary enzyme deficiency; this in turn can cause an acrodermatitis enteropathica-like dermatosis.

More Information


  1. Kury S, Devilder M, Avet Loiseau H, Dreno B, Moisan J. Expression pattern, genomic structure and evaluation of the human SLC30A4 gene as a candidate for acrodermatitis enteropathica. Hum Genet. 2001;109:178-85 pubmed
    ..SLC30A4 coding sequences, and to the very similar clinical features encountered in the murine lm and in human acrodermatitis enteropathica, SLC30A4 has appeared to us to be a good candidate for acrodermatitis enteropathica...
  2. Kim B, Joo K, Lee H, Jeong Y, Suh H, Kim D, et al. A case of chronic pancreatitis associated with liver infarction and acrodermatitis enteropathica. Korean J Intern Med. 2002;17:263-5 pubmed
    Liver infarction and acrodermatitis enteropathica are rare complications of chronic pancreatitis...
  3. Andrews G. Regulation and function of Zip4, the acrodermatitis enteropathica gene. Biochem Soc Trans. 2008;36:1242-6 pubmed publisher
    ..Among these, ZIP4 is known to be particularly important for zinc homoeostasis. Mutations in this gene cause acrodermatitis enteropathica, a rare recessive-lethal human genetic disorder...
  4. Wang K, Pugh E, Griffen S, Doheny K, Mostafa W, al Aboosi M, et al. Homozygosity mapping places the acrodermatitis enteropathica gene on chromosomal region 8q24.3. Am J Hum Genet. 2001;68:1055-60 pubmed
    b>Acrodermatitis enteropathica (AE) is a rare autosomal recessive pediatric disease characterized by dermatitis, diarrhea, alopecia, and growth failure...
  5. Geiser J, Venken K, De Lisle R, Andrews G. A mouse model of acrodermatitis enteropathica: loss of intestine zinc transporter ZIP4 (Slc39a4) disrupts the stem cell niche and intestine integrity. PLoS Genet. 2012;8:e1002766 pubmed publisher
    Mutations in the human Zip4 gene cause acrodermatitis enteropathica, a rare, pseudo-dominant, lethal genetic disorder. We created a tamoxifen-inducible, enterocyte-specific knockout of this gene in mice which mimics this human disorder...
  6. Kambe T, Andrews G. Novel proteolytic processing of the ectodomain of the zinc transporter ZIP4 (SLC39A4) during zinc deficiency is inhibited by acrodermatitis enteropathica mutations. Mol Cell Biol. 2009;29:129-39 pubmed publisher
    The zinc transporter ZIP4 (SLC39A4) is mutated in humans with the rare, autosomal recessive genetic disease acrodermatitis enteropathica. In mice, this gene is essential during early embryonic development...
  7. Dufner Beattie J, Wang F, Kuo Y, Gitschier J, Eide D, Andrews G. The acrodermatitis enteropathica gene ZIP4 encodes a tissue-specific, zinc-regulated zinc transporter in mice. J Biol Chem. 2003;278:33474-81 pubmed
    The human ZIP4 gene (SLC39A4) is a candidate for the genetic disorder of zinc metabolism acrodermatitis enteropathica. To understand its role in zinc homeostasis, we examined the function and expression of mouse ZIP4...
  8. Nakano A, Nakano H, Nomura K, Toyomaki Y, Hanada K. Novel SLC39A4 mutations in acrodermatitis enteropathica. J Invest Dermatol. 2003;120:963-6 pubmed
    b>Acrodermatitis enteropathica is an autosomal recessive disease characterized by skin involvement due to defective intestinal zinc absorption...
  9. Kury S, Dreno B, Bezieau S, Giraudet S, Kharfi M, Kamoun R, et al. Identification of SLC39A4, a gene involved in acrodermatitis enteropathica. Nat Genet. 2002;31:239-40 pubmed
    ..The chromosomal location and expression of SLC39A4, together with mutational analysis of eight families affected with acrodermatitis enteropathica, suggest that SLC39A4 is centrally involved in the pathogenesis of this condition.
  10. Wang K, Zhou B, Kuo Y, Zemansky J, Gitschier J. A novel member of a zinc transporter family is defective in acrodermatitis enteropathica. Am J Hum Genet. 2002;71:66-73 pubmed
    The rare inherited condition acrodermatitis enteropathica (AE) results from a defect in the absorption of dietary zinc...
  11. Wang F, Kim B, Dufner Beattie J, Petris M, Andrews G, Eide D. Acrodermatitis enteropathica mutations affect transport activity, localization and zinc-responsive trafficking of the mouse ZIP4 zinc transporter. Hum Mol Genet. 2004;13:563-71 pubmed
    ..The human ZIP4 gene (SLC39A4) was identified because of its association with acrodermatitis enteropathica (AE), a genetic disorder of zinc absorption...
  12. Leverkus M, Finner A, Pokrywka A, Franke I, Gollnick H. Metastatic squamous cell carcinoma of the ankle in long-standing untreated acrodermatitis chronica atrophicans. Dermatology. 2008;217:215-8 pubmed publisher
    b>Acrodermatitis chronica atrophicans (ACA) represents the persistent late stage of borreliosis in which Borrelia species may survive for decades...
  13. Yuzbasiyan Gurkan V, Bartlett E. Identification of a unique splice site variant in SLC39A4 in bovine hereditary zinc deficiency, lethal trait A46: An animal model of acrodermatitis enteropathica. Genomics. 2006;88:521-6 pubmed an autosomal recessive disorder first described in 1964, with a clinical presentation similar to that of acrodermatitis enteropathica (AE) in humans. The molecular basis of the defect has not been previously identified...
  14. Schmitt S, Kury S, Giraud M, Dreno B, Kharfi M, Bezieau S. An update on mutations of the SLC39A4 gene in acrodermatitis enteropathica. Hum Mutat. 2009;30:926-33 pubmed publisher
    b>Acrodermatitis enteropathica (AE) is a very rare inherited recessive disease caused by severe zinc deficiency. It typically occurs in early infancy and is characterized by periorificial and acral dermatitis, alopecia, and diarrhea...
  15. Lee J, Chang S, Suh C, Choi J, Sung K, Moon K, et al. A case of acrodermatitis enteropathica-like dermatosis caused by ornithine transcarbamylase deficiency. J Am Acad Dermatol. 2002;46:965-7 pubmed
  16. Kharfi M, El Fekih N, Aounallah Skhiri H, Schmitt S, Fazaa B, Kury S, et al. Acrodermatitis enteropathica: a review of 29 Tunisian cases. Int J Dermatol. 2010;49:1038-44 pubmed publisher
    b>Acrodermatitis enteropathica is a rare autosomal recessive disease due to an abnormality in a zinc transporting molecule...
  17. Inamadar A, Palit A. Acrodermatitis enteropathica with depigmented skin lesions simulating vitiligo. Pediatr Dermatol. 2007;24:668-9 pubmed
  18. Maverakis E, Fung M, Lynch P, Draznin M, Michael D, Ruben B, et al. Acrodermatitis enteropathica and an overview of zinc metabolism. J Am Acad Dermatol. 2007;56:116-24 pubmed
    b>Acrodermatitis enteropathica is a rare autosomal recessive disorder of zinc deficiency. The genetic defect has been mapped to 8q24 and the defective gene identified as SLC39A4, which encodes the zinc transporter Zip4...
  19. Muñiz A, Bartle S, Foster R. Edema, anemia, hypoproteinemia, and acrodermatitis enteropathica: an uncommon initial presentation of cystic fibrosis. Pediatr Emerg Care. 2004;20:112-4 pubmed
    ..Less frequently, the presenting features in infants may include edema, anemia, hypoproteinemia, and acrodermatitis enteropathica. Liver involvement may produce hepatomegaly and mild elevation of transaminases...
  20. Mullegger R, Means T, Shin J, Lee M, Jones K, Glickstein L, et al. Chemokine signatures in the skin disorders of Lyme borreliosis in Europe: predominance of CXCL9 and CXCL10 in erythema migrans and acrodermatitis and CXCL13 in lymphocytoma. Infect Immun. 2007;75:4621-8 pubmed
    ..Europe include erythema migrans, an acute, self-limited lesion; borrelial lymphocytoma, a subacute lesion; and acrodermatitis chronica atrophicans, a chronic lesion...
  21. Abbas O, Itani S, Ghosn S, Kibbi A, Fidawi G, Farooq M, et al. Acrodermatitis continua of Hallopeau is a clinical phenotype of DITRA: evidence that it is a variant of pustular psoriasis. Dermatology. 2013;226:28-31 pubmed publisher
    b>Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, sterile, pustular eruption that predominantly affects the fingertips with nail involvement...
  22. Jensen S, McCuaig C, Zembowicz A, Hurt M. Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency: a report of two cases and review of the literature. J Cutan Pathol. 2008;35 Suppl 1:1-13 pubmed publisher
    b>Acrodermatitis enteropathica (AE) is a rare disorder associated with poor absorption of zinc. A variety of clinical and histological findings have been reported in the literature, described mainly in isolated case reports...
  23. Bordignon M, Zattra E, Albertin C, Belloni Fortina A. Successful treatment of a 9-year-old boy affected by acrodermatitis continua of Hallopeau with targeted ultraviolet B narrow-band phototherapy. Photodermatol Photoimmunol Photomed. 2010;26:41-3 pubmed publisher
    b>Acrodermatitis continua of Hallopeau (ACH) consists of a relapsing pustular eruption of the distal portions of hands and feet...
  24. Zawar V, Chuh A. Gianotti-crosti syndrome in India is not associated with hepatitis B infection. Dermatology. 2004;208:87 pubmed
  25. Virgili A, Trincone S, Zampino M, Corazza M. Acroangiodermatitis of amputation stump. Eur J Dermatol. 2003;13:402-3 pubmed
    ..We describe a case of acroangiodermatitis in an above-knee amputation stump in a 48-year-old-female affected by Down's syndrome. ..
  26. Ruzic Sabljic E, Maraspin V, Lotric Furlan S, Jurca T, Logar M, Pikelj Pecnik A, et al. Characterization of Borrelia burgdorferi sensu lato strains isolated from human material in Slovenia. Wien Klin Wochenschr. 2002;114:544-50 pubmed
    ..Heterogeneity of Borrelia strains may play a significant role in the virulence and pathogenesis of the infection. Differences in antigenic components have an important impact on serological testing and vaccine development. ..
  27. Iorizzo L, Scott G, Tausk F. Gianotti-Crosti syndrome: a case report in an adult. Cutis. 2012;89:169-72 pubmed
    ..The clinical and histologic correlation was consistent with GCS in an adult. This condition may not be as rare in adults as previously thought. Clinicians should keep GCS in their differential diagnosis when examining adult patients. ..
  28. Farro P, Zalaudek I, Ferrara G, Fulgione E, Cicale L, Petrillo G, et al. Unusual association between acrokeratosis verruciformis of Hopf and multiple keratoacanthomas. Successful therapy with acitretin. J Dtsch Dermatol Ges. 2004;2:440-2 pubmed
    ..A 62-year-old man presented with the unusual association of acrokeratosis verruciformis of Hopf and multiple keratoacanthomas. Both conditions were treated successfully with acitretin. ..
  29. Canpolat F, Canpolat F, Eskioglu F. Acrodermatitis enteropathica in a full-term exclusively breast-fed infant. Eur J Dermatol. 2008;18:192-3 pubmed publisher
  30. Thielen A, Barde C, Marazza G, Saurat J. Long-term control with etanercept (Enbrel) of a severe acrodermatitis continua of Hallopeau refractory to infliximab (Remicade). Dermatology. 2008;217:137-9 pubmed publisher
    Hallopeau's acrodermatitis is characterized by the presence of aseptic pustules on an inflammatory basis of the periungual or subungual region. The cyclic recurrences induce important physical and psychological morbidity...
  31. Geiser J, De Lisle R, Finkelstein D, Adlard P, Bush A, Andrews G. Clioquinol synergistically augments rescue by zinc supplementation in a mouse model of acrodermatitis enteropathica. PLoS ONE. 2013;8:e72543 pubmed publisher
    ..of the intestinal zinc transporter Zip4 (Slc39a4) in mice creates a model of the lethal human genetic disease acrodermatitis enteropathica (AE)...
  32. Tee S, Martínez Escanamé M, Zuriel D, Fried I, Wolf I, Massone C, et al. Acrodermatitis chronica atrophicans with pseudolymphomatous infiltrates. Am J Dermatopathol. 2013;35:338-42 pubmed publisher, we describe the clinicopathologic features of pseudolymphomatous infiltrates found within lesions of acrodermatitis chronica atrophicans (ACA). We studied 11 patients (10 females, 1 male, age range 60-88 years)...
  33. Barcelos A, Nico M. Bazex-Dupré-Christol syndrome in a 1-year-old boy and his mother. Pediatr Dermatol. 2008;25:112-3 pubmed publisher
    ..Pili bifurcatti is an uncommon hair shaft dysplasia that has not before been observed in Bazex-Dupré-Christol syndrome. ..
  34. Drago F, Javor S, Ciccarese G, Parodi A. Gianotti-Crosti syndrome as presenting sign of cytomegalovirus infection: A case report and a critical appraisal of its possible cytomegalovirus etiology. J Clin Virol. 2016;78:120-2 pubmed publisher
    ..In our case, GCS was related to cytomegalovirus (CMV) primary infection and may be considered the presenting sign of the infection. ..
  35. Bin B, Bhin J, Kim N, Lee S, Jung H, Seo J, et al. An Acrodermatitis Enteropathica-Associated Zn Transporter, ZIP4, Regulates Human Epidermal Homeostasis. J Invest Dermatol. 2017;137:874-883 pubmed publisher
    b>Acrodermatitis enteropathica is an autosomal recessive disorder characterized by scaly eczematous dermatosis accompanied by alopecia and diarrhea...
  36. Jat K, Marwaha R, Panigrahi I, Kaur S. Fulminant candida infection in an infant with Acrodermatitis Enteropathica. Indian J Pediatr. 2009;76:941-2 pubmed publisher
    b>Acrodermatitis Enteropathica (AE) is an important nutritional disorder of children affecting both innate and cell mediated immunity. It predisposes to secondary bacterial and candida superinfections...
  37. Jäckle R. [Reddish-blue color lower leg with distinct vessel design. "Venous insufficiency" was Lyme borreliosis]. MMW Fortschr Med. 2003;145:18 pubmed
  38. Wanat K, Bandow G, Klekotka P. Palmar-plantar erythrodysesthesia caused by mercaptopurine and mesalamine. Arch Dermatol. 2008;144:1079-81 pubmed publisher
  39. De la Torre C. Gianotti-Crosti syndrome following milkers' nodules. Cutis. 2004;74:316-8 pubmed
    Gianotti-Crosti syndrome (GCS), or papular acrodermatitis of childhood, is a distinctive self-limiting rash with symmetrically distributed papules on the face, buttocks, and extremities that has been related to several underlying viral ..
  40. Weisshaar E, Diepgen T. Successful etanercept therapy in therapy refractory acrodermatitis continua suppurativa Hallopeau. J Dtsch Dermatol Ges. 2007;5:489-92 pubmed
    A 50-year-old patient presented with erythema, vesicles and pustules as well as interphalangeal joint pain. Acrodermatitis continua suppurativa Hallopeau was diagnosed...
  41. Sanchez J, Barham K, Sangueza O. Acquired acrodermatitis enteropathica: case report of an atypical presentation. J Cutan Pathol. 2007;34:490-3 pubmed
    b>Acrodermatitis enteropathica (ADE) is a rare genetic or acquired disorder of hypozincemia. It can be caused by impaired intestinal absorption of zinc or by poor consumption of the mineral...
  42. Yazbeck N, Muwakkit S, Abboud M, Saab R. Zinc and biotin deficiencies after pancreaticoduodenectomy. Acta Gastroenterol Belg. 2010;73:283-6 pubmed
    ..Early diagnosis is essential to prevent irreversible sequelae. Adequate supplementation of zinc and biotin as well as dietary advice is needed for clinical improvement. ..
  43. Lovett A, Kokta V, Maari C. Diffuse dermatitis: An unexpected initial presentation of cystic fibrosis. J Am Acad Dermatol. 2008;58:S1-4 pubmed publisher
    ..We report such a case and relate the difficulties of establishing a diagnosis. We also discuss possible pathophysiological mechanisms, histopathology, prognosis, and treatment. ..
  44. Kumar P, Lal N, Mondal A, Mondal A, Gharami R, Maiti A. Zinc and skin: a brief summary. Dermatol Online J. 2012;18:1 pubmed
    ..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 ..
  45. Yu H, Shan Y, Lin P. Zinc deficiency with acrodermatitis enteropathica-like eruption after pancreaticoduodenectomy. J Formos Med Assoc. 2007;106:864-8 pubmed
    ..Herein, we report two patients who experienced zinc deficiency with acrodermatitis enteropathica-like eruption, alopecia, glossitis and nail dystrophy after PD...
  46. Arisi M, Rossi M, Sala R, Petrilli G, Marocolo D, Ungari M, et al. A Case of Acrodermatitis Continua of Hallopeau Following Chronic Pustular Cheilitis. Dermatol Ther (Heidelb). 2016;6:89-94 pubmed publisher
    We describe the case of a young male affected by chronic pustular psoriasis of the lips that remained the only manifestation of acrodermatitis continua of Hallopeau (ACH) for years before the onset of the characteristic hand lesions.
  47. Jahfari S, Krawczyk A, Coipan E, Fonville M, Hovius J, Sprong H, et al. Enzootic origins for clinical manifestations of Lyme borreliosis. Infect Genet Evol. 2017;49:48-54 pubmed publisher
    ..from Lyme borreliosis cases with distinct clinical manifestations (erythema migrans, neuroborreliosis, acrodermatitis chronica atrophicans, and Lyme arthritis) and isolates from Ixodes ricinus ticks feeding on rodents, birds and ..
  48. Mishra P, Sirka C, Das R, Nanda D. Secondary acrodermatitis enteropathica-like lesions in a child with newly diagnosed coeliac disease. Paediatr Int Child Health. 2016;36:72-5 pubmed publisher
    ..A boy aged 19 months presented with persistent diarrhoea, was diagnosed with CD, and developed severe zinc deficiency with acrodermatitis enteropathica-like disease while on GFD and zinc supplementation.
  49. Chen M, Song Y. An infant with acrodermatitis enteropathica-like symptoms but without hypozincemia. Pediatr Dermatol. 2005;22:280-1 pubmed
  50. Lenz C. [With skin and bones]. MMW Fortschr Med. 2006;148:57 pubmed
  51. Silpa Archa N, Wongpraparut C. A recalcitrant acrodermatitis continua of Hallopeau successfully treated with etanercept. J Med Assoc Thai. 2011;94:1154-7 pubmed
    b>Acrodermatitis continua of Hallopeau (ACH) is considered as a localized form of pustularpsoriasis that is usually refractory to the treatment...
  52. Murthy S, Udagani M, Badakali A, Yelameli B. Symptomatic zinc deficiency in a full-term breast-fed infant. Dermatol Online J. 2010;16:3 pubmed
    ..This report highlights the occurrence of hypozincemia among a population at risk, consisting of infants who are exclusively breast-fed for a prolonged period. ..
  53. Eisendle K, Zelger B. The expanding spectrum of cutaneous borreliosis. G Ital Dermatol Venereol. 2009;144:157-71 pubmed
    ..manifestations of cutaneous borreliosis like erythema (chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica atrophicans evidence is growing that at least in part also other skin manifestations, especially ..