History

Fact Explanation
An inherited form of Zinc deficiency Acrodermatitis enteropathica can be either congenital or acquired. Congenital acrodermatitis enteropathica is due to reduced intestinal absorption of zinc (mutation in Zip4 transporter in the intestinal cells) and it usually begins to manifest after breast feeding is stopped and weaning is started. [1]
History of diarrhea or malabsorption History of refractory diarrhea or malabsorption is present in many patients with acrodermatitis enteropathica. Chronic diarrhea leads to malabsorption and acquired acrodermatitis enteropathica. Secondary causes of acquired zinc deficiency includes chronic pancreatitis, malabsorption syndromes, blind loop syndrome and cirrhosis. Diets with high amounts of phytate, chronic alcoholism, total parenteral nutrition, anorexia nervosa and bulimia nervosa are other causes of zinc deficiency. [1,5]
Failure to thrive Failure to thrive is also a common feature seen in acrodermatitis enteropathica. [4]
Irritability Children are highly irritable. Mental slowness is another finding associated with zinc deficiency. These symptoms are believed to be due to brain atrophy. [4]
Skin lesions The lesions typically starts around the body's orifices and later spread diffusely. Inflamed patches of erythematous and dry skin are characteristic initial skin lesions. Later they become crusted and blisters appear. Rupture of blisters expose the purulent eroded skin lesions. [1,2,4]
Alopecia Diffuse hair loss in scalp, eye lashes and eye brows can be seen. In congenital acrodermatitis enteropathica these lesions begin to appear after weaning is started. [1,4]
History of malnutrition Malnutrition especially zinc deficiency leads to the development of acquired acrodermatitis enteropathica. [2]
Positive family history Congenital acrodermatitis enteropathica is an autosomal recessive disease. It usually begins to manifest after the breast feeding is quit. [1]
History of glucagonoma syndrome Glucagonoma syndrome is a rare paraneoplastic syndrome. It presents as erythematous skin lesions, hyperglucagonemia, diabetes mellitus, anemia, weight loss, glossitis, cheilitis, neuropsychiatric disturbances, steatorrhea and diarrhea. Diarrhea and steatorrhea are possible causes for the development of acquired acrodermatitis enteropathica. [1,3]
History of renal diseases [1] History of renal tubular disease, nephrotic syndrome, chronic kidney disease and hemodialysis can also cause zinc deficiency. [5]
History of major trauma [1] This is another cause of malnutrition. Major surgeries and severe burns are common causes.
References
  1. CHESHIRE H, STATHER P, VORSTER J. Acquired acrodermatitis enteropathica due to zinc deficiency in a patient with pre-existing Darier's disease J Dermatol Case Rep [online] , 3(3):41-43 [viewed 15 July 2014] Available from: doi:10.3315/jdcr.2009.1032
  2. MANKANEY GAUTAM NARESH, VIPPERLA KISHORE. Acquired Acrodermatitis Enteropathica. N Engl J Med [online] 2014 July, 371(1):67-67 [viewed 15 July 2014] Available from: doi:10.1056/NEJMicm1312911
  3. CASTRO PABLO, DE LEóN ALBERTO, TRANCóN JOSE, MARTíNEZ PALOMA, ÁLVAREZ PéREZ JOSE A, FERNáNDEZ FERNáNDEZ JOSE C, GARCíA BERNARDO CARMEN M, SERRA LUIS, GONZáLEZ GONZáLEZ JUAN J. Glucagonoma syndrome: a case report. Array [online] 2011 December [viewed 15 July 2014] Available from: doi:10.1186/1752-1947-5-402
  4. KüRY SéBASTIEN, KHARFI MONIA, SCHMITT SéBASTIEN, BéZIEAU STéPHANE. Clinical utility gene card for: acrodermatitis enteropathica. Eur J Hum Genet [online] December [viewed 15 July 2014] Available from: doi:10.1038/ejhg.2011.227
  5. BODEMER APPLE A, LLOYD RITA. Acrodermatits enteropathica vs. acquired zinc deficiency. Journal of the American Academy of Dermatology [online] 2004 March [viewed 15 July 2014] Available from: doi:10.1016/j.jaad.2003.10.178

Examination

Fact Explanation
Cutaneous manifestations Commonly seen cutaneous lesions are erythematous, dry, and scaly patches or plaques. Skin lesions can be secondarily infected with bacteria and pus will be seen in those instances. Later they become honey colored, crusted and erosive. Angular cheilitis, glossitis, conjunctivitis, blepharitis and punctate keratopathy are mucosal manifestations. [1]
Nail changes Nails dystrophy and paronychia are seen in nails. [1]
Growth retardation Physical growth retardation is seen in most of the affected patients. This can be secondary to chronic malabsorption. [2]
Alopecia Diffuse hair loss can be seen over the scalp, eye brows and in eye lashes. [2]
Mental slowness Mental slowness and irritability can be detected. [2]
References
  1. MANKANEY GAUTAM NARESH, VIPPERLA KISHORE. Acquired Acrodermatitis Enteropathica. N Engl J Med [online] 2014 July, 371(1):67-67 [viewed 15 July 2014] Available from: doi:10.1056/NEJMicm1312911
  2. BODEMER APPLE A, LLOYD RITA. Acrodermatits enteropathica vs. acquired zinc deficiency. Journal of the American Academy of Dermatology [online] 2004 March [viewed 15 July 2014] Available from: doi:10.1016/j.jaad.2003.10.178

Differential Diagnoses

Fact Explanation
Psoriasis Psoriasis is a chronic inflammatory dermatitis. The skin lesions are erythematous plaques mainly seen over the extensor surfaces. Psoriatic skin lesions may resemble skin lesions seen in acrodermatitis enteropathica. [3]
Atopic dermatitis Chronic relapsing pruritic and inflammatory skin lesions are seen in atopic dermatitis. In infants the skin lesions are seen over the scalp, face, neck, trunk and extensor surfaces of the extremities. [4,5]
Candidiasis Candidiasis can affect skin and mucosal membranes. [6]
Epidermolysis bullosa Epidermolysis bullosa is an inherited disease. Presence of skin and mucosal blisters is characteristic of the disease. It is often life threatening in neonates. [7]
Seborrheic dermatitis Seborrheic dermatitis is a chronic and relapsing skin inflammatory condition. It is commonly seen over the areas rich of seborrheic glands. Skin scaling and ill demarcated erythematous skin patches can be seen. [8]
Darier's disease This is an autosomal dominant disease that causes greasy and hyperkeratotic papules, predominantly affecting the seborrheic areas. The disease can involve the nails as well causing white and red longitudinal bands and longitudinal nail ridges. These nail lesions can cause longitudinal splitting of the nails and subungual hyperkeratosis. [1]
Impetigo contagiosa Impetigo contagiosa is superficial infection of the skin commonly seen in children. Skin vesicles and pustules heal by fibrosis. [9]
Transient neonatal zinc deficiency In this condition neonate develops symptoms and signs of zinc deficiency. It is due to reduced zinc levels in maternal milk. Zinc supplementation will resolve all the manifestations of the disease. [2]
References
  1. CHESHIRE H, STATHER P, VORSTER J. Acquired acrodermatitis enteropathica due to zinc deficiency in a patient with pre-existing Darier's disease J Dermatol Case Rep [online] , 3(3):41-43 [viewed 15 July 2014] Available from: doi:10.3315/jdcr.2009.1032
  2. KüRY SéBASTIEN, KHARFI MONIA, SCHMITT SéBASTIEN, BéZIEAU STéPHANE. Clinical utility gene card for: acrodermatitis enteropathica. Eur J Hum Genet [online] December [viewed 15 July 2014] Available from: doi:10.1038/ejhg.2011.227
  3. DUBOIS DECLERCQ SARAH, POULIOT ROXANE. Promising New Treatments for Psoriasis. The Scientific World Journal [online] 2013 December, 2013:1-9 [viewed 15 July 2014] Available from: doi:10.1155/2013/980419
  4. BIEBER THOMAS. Atopic Dermatitis. N Engl J Med [online] 2008 April, 358(14):1483-1494 [viewed 15 July 2014] Available from: doi:10.1056/NEJMra074081
  5. WATSON WADE, KAPUR SANDEEP. Atopic dermatitis. Array [online] 2011 December [viewed 15 July 2014] Available from: doi:10.1186/1710-1492-7-S1-S4
  6. PAPPAS PETER G., REX JOHN H., SOBEL JACK D., FILLER SCOTT G., DISMUKES WILLIAM E., WALSH THOMAS J., EDWARDS JOHN E.. Guidelines for Treatment of Candidiasis. CLIN INFECT DIS [online] 2004 January, 38(2):161-189 [viewed 15 July 2014] Available from: doi:10.1086/380796
  7. DAS BIBHUTI B, SAHOO SUNATI. Dystrophic Epidermolysis Bullosa. J Perinatol [online] 2004 January, 24(1):41-47 [viewed 15 July 2014] Available from: doi:10.1038/sj.jp.7211019
  8. NALDI LUIGI, REBORA ALFREDO. Seborrheic Dermatitis. N Engl J Med [online] 2009 January, 360(4):387-396 [viewed 15 July 2014] Available from: doi:10.1056/NEJMcp0806464
  9. PARKER MT, TOMLINSON AJ, WILLIAMS RE. Impetigo contagiosa: The association of certain types of Staphylococcus aureus and of Streptococcus pyogenes with superficial skin infections J Hyg (Lond) [online] 1955 Dec, 53(4):458-473 [viewed 15 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2217985

Investigations - for Diagnosis

Fact Explanation
Plasma zinc levels Plasma zinc levels of less than 50 mcg/dL are suggestive of acrodermatitis enteropathica. [2,3]
Skin biopsy Although not mandatory skin biopsy is helpful in excluding sinister pathology like skin malignancy. Parakeratosis, impetiginized regions, and chronic inflammation lead to dermal edema. These are non-specific findings seen in acrodermatitis enteropathica and none of these features are diagnostic. [1,2]
Serum alkaline phosphatase (ALP) Zinc is necessary for the synthesis of ALP. Severe zinc deficiency can lead to low ALP levels as well. [2,3]
Gene mapping Gene mapping is helpful in diagnosing congenital acrodermatitis enteropathica. It identifies the mutations in the gene SLC39A4. [4]
Skin swabs Secondary infection of the skin lesions can be diagnosed by using skin swabs. Gram stain and culture are useful in diagnosis of secondary bacterial infection. [2]
References
  1. MANKANEY GAUTAM NARESH, VIPPERLA KISHORE. Acquired Acrodermatitis Enteropathica. N Engl J Med [online] 2014 July, 371(1):67-67 [viewed 15 July 2014] Available from: doi:10.1056/NEJMicm1312911
  2. CHESHIRE H, STATHER P, VORSTER J. Acquired acrodermatitis enteropathica due to zinc deficiency in a patient with pre-existing Darier's disease J Dermatol Case Rep [online] , 3(3):41-43 [viewed 15 July 2014] Available from: doi:10.3315/jdcr.2009.1032
  3. KüRY SéBASTIEN, KHARFI MONIA, SCHMITT SéBASTIEN, BéZIEAU STéPHANE. Clinical utility gene card for: acrodermatitis enteropathica. Eur J Hum Genet [online] December [viewed 15 July 2014] Available from: doi:10.1038/ejhg.2011.227
  4. BODEMER APPLE A, LLOYD RITA. Acrodermatits enteropathica vs. acquired zinc deficiency. Journal of the American Academy of Dermatology [online] 2004 March [viewed 15 July 2014] Available from: doi:10.1016/j.jaad.2003.10.178

Investigations - Followup

Fact Explanation
Plasma zinc levels Zinc deficiency can recur, so assessment of plasma zinc levels is necessary.
References

Management - General Measures

Fact Explanation
Health education Acquired acrodermatitis enteropathica is caused by malnutrition. Patients should be advised to consume zinc rich foods like, shellfish, green leafy vegetables, legumes, nuts and whole grains. Also they are advised to consume a protein rich foods, which contain more zinc. Patients should be educated about the importance of zinc supplementation and about the possible side effects of zinc (abdominal pain, dyspeptic symptoms, nausea, vomiting, diarrhea, irritability, headache and lethargy). There should be no restriction on daily physical activities. [1]
References
  1. CHESHIRE H, STATHER P, VORSTER J. Acquired acrodermatitis enteropathica due to zinc deficiency in a patient with pre-existing Darier's disease J Dermatol Case Rep [online] , 3(3):41-43 [viewed 15 July 2014] Available from: doi:10.3315/jdcr.2009.1032

Management - Specific Treatments

Fact Explanation
Zinc supplementation Zinc supplementation (5-10 mg/kg/day) is the definitive treatment for acrodermatitis enteropathica. Zinc sulphate is the commonly used and the best tolerated preparation. Once the zinc deficiency is corrected the skin lesions slowly disappear. Congenital acrodermatitis enteropathica patients should be prescribed lifelong zinc supplementation. [1]
Dietary advice Acquired acrodermatitis enteropathica is caused by malnutrition. Patients should be advised to consume zinc rich foods like, shellfish, green leafy vegetables, legumes, nuts and whole grains. Also they are advised to consume a protein rich foods, which contain more zinc. [2]
References
  1. MANKANEY GAUTAM NARESH, VIPPERLA KISHORE. Acquired Acrodermatitis Enteropathica. N Engl J Med [online] 2014 July, 371(1):67-67 [viewed 15 July 2014] Available from: doi:10.1056/NEJMicm1312911
  2. CHESHIRE H, STATHER P, VORSTER J. Acquired acrodermatitis enteropathica due to zinc deficiency in a patient with pre-existing Darier's disease J Dermatol Case Rep [online] , 3(3):41-43 [viewed 15 July 2014] Available from: doi:10.3315/jdcr.2009.1032