acrodermatitis

What is Acrodermatitis?

Acrodermatitis refers to a group of inflammatory skin conditions that primarily affect the extremities, such as the arms and legs [15]. It can present in various forms, each with distinct characteristics, causes, and treatment options.

Types of Acrodermatitis

There are several types of acrodermatitis, including:

• Papular Acrodermatitis of Childhood: Also known as Gianotti-Crosti syndrome, this is a benign rash associated with various viral illnesses [10]. It typically affects young children and presents as an itchy red rash on the arms, legs, buttocks, and face.
• Acrodermatitis Enteropathica: This is a rare inherited form of zinc deficiency that can cause periorificial and acral dermatitis, alopecia, diarrhea, and growth failure [5].
• Primary Acrodermatitis Enteropathica: This is an autosomal recessive genetic disorder that manifests when two defective genes are inherited, one from each parent [8].

Symptoms of Acrodermatitis

The symptoms of acrodermatitis can vary depending on the type. However, common symptoms include:

• Rash or patch on the skin
• Brownish-red or copper-colored patch that is firm and flat on top
• String of bumps may appear on the affected area [2]
• Flu-like symptoms and a general feeling of malaise [13]

Causes and Treatment

The causes of acrodermatitis can vary depending on the type. However, it is often associated with viral infections or zinc deficiency. Treatment options include topical creams, oral medications, and in some cases, hospitalization for severe cases.

References:

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Common Signs and Symptoms of Acrodermatitis

Acrodermatitis, also known as papular acrodermatitis of childhood or Gianotti-Crosti syndrome, is a rare skin condition that can cause various symptoms. The signs and symptoms of acrodermatitis can vary depending on the type and underlying cause, but here are some common ones:

• Skin Lesions: Erythematous (red), pustular (pus-filled), or blistering lesions on the skin, which can be itchy and painful [12].
• Itching and Redness: Itchy red or purple blisters that form on the body, which can be accompanied by skin inflammation [1].
• Hair Loss: Alopecia, or hair loss, is a common symptom of acrodermatitis enteropathica [2].
• Diarrhea: Diarrhea is another frequent manifestation of acrodermatitis, especially in cases caused by zinc deficiency [3].
• Nail Dystrophy: Abnormal nails, including nail dystrophy and Beau-Reil lines on the nails, are also common symptoms [6].
• Mouth Ulcers and Angular Cheilitis: Mouth ulcers and angular cheilitis (inflammation of the corners of the mouth) can be early signs of acquired acrodermatitis enteropathica [3].
• Rash on the Buttocks, Face, and Neck: Red, elevated lesions that do not contain pus can occur on the limbs, buttocks, face, and neck in cases of Gianotti-Crosti disease [14].

These symptoms can vary depending on the underlying cause and type of acrodermatitis. If you suspect you or your child has this condition, it's essential to consult a healthcare professional for proper diagnosis and treatment.

References: [1] - Search result 10 [2] - Search result 3 [3] - Search result 4 [6] - Search result 6 [12] - Search result 12 [14] - Search result 14

Diagnostic Tests for Acrodermatitis

Acrodermatitis, a condition characterized by skin lesions and other symptoms, can be diagnosed through various tests. Here are some diagnostic tests used to diagnose different forms of acrodermatitis:

• Clinical Evaluation: A thorough medical history and physical examination are essential in diagnosing acrodermatitis. This includes identifying potential genetic, infectious, or environmental factors that may contribute to the condition.
• Laboratory Tests: Various laboratory tests can help confirm the diagnosis of acrodermatitis. These include:
  • Zinc level testing: Measuring plasma zinc concentration is a crucial test for diagnosing acrodermatitis enteropathica (AE). A serum zinc level of 50 µg/dL confirms the diagnosis of AE [13].
  • Serum alkaline phosphatase level: A low serum alkaline phosphatase level can be helpful in confirming the diagnosis of AE [13].
• Skin Biopsies: Skin biopsies may be performed to rule out other conditions that may present with similar symptoms.
• Genetic Testing: Genetic testing, such as detecting mutations in the zinc transporter gene SLC39A4 (solute carrier family 39 member 4), can help diagnose inherited forms of acrodermatitis [3].
• Serologic Testing: Serologic testing, including ELISA and Western blot tests, may be used to confirm the diagnosis of Lyme borreliosis, which is associated with acrodermatitis chronica atrophicans (ACA) [9].

Additional Diagnostic Tests

Other diagnostic tests that may be performed to diagnose specific forms of acrodermatitis include:

• Zinc absorption tests: In vitro or in vivo zinc absorption tests using radioisotopes (65Zn or 69mZn) may be performed in cases of doubt [15].
• Plasma zinc levels testing: Plasma zinc levels should be tested to confirm the diagnosis of AE. Specimens should be collected in plastic syringes or acid-washed Vacutainer tubes with no rubber stoppers [14].

It's essential to note that establishing care with a healthcare provider and conducting thorough diagnostic tests can help determine the best course of treatment for acrodermatitis.

References:

[3] The Blueprint Genetics Hereditary Acrodermatitis Enteropathica Panel (test code DE0101)

[9] Acrodermatitis chronica atrophicans (ACA) is a late and chronic manifestation of Lyme borreliosis.

[13] A serum zinc level of 50 µg/dL confirms the diagnosis of AE.

[14] Plasma zinc levels should be tested to confirm the diagnosis of AE.

[15] In vitro or in vivo zinc absorption tests using radioisotopes (65Zn or 69mZn) may be performed in cases of doubt.

Treatment Options for Acrodermatitis

Acrodermatitis, a rare inflammatory skin disease, can be challenging to treat due to its chronic and recurrent nature. While there is limited evidence from case reports and small case series, various treatment options have been reported in the medical literature.

• Zinc Supplementation: Treatment of acrodermatitis enteropathica requires lifelong zinc supplementation. Typically, 1-3 mg/kg of zinc gluconate or sulfate is administered orally each day [5][6].
• Topical Therapy: Topical corticosteroids have been reported as an effective treatment option for some patients with acrodermatitis continua of Hallopeau [11].
• Biologic Therapies: Biologics such as etanercept and secukinumab have shown promise in treating acrodermatitis continua of Hallopeau, particularly in combination therapy [10][14]. However, the development of anti-drug antibodies can contribute to treatment failure.
• Spesolimab: A recent case report suggests that spesolimab may be considered for the treatment of acrodermatitis continua of Hallopeau, especially in patients with intractable nail lesions [4].
• TNF-Blocking Agents: TNF-blocking agents such as etanercept and adalimumab have been reported to be effective in treating acrodermatitis continua of Hallopeau, although treatment failure can occur due to the development of anti-drug antibodies [15].

It is essential to note that each patient's response to treatment may vary, and a comprehensive treatment plan should be tailored to individual needs. Further research is necessary to establish the most effective treatment strategies for acrodermatitis.

References: [4] P Wen (2024) - Our observation suggests that spesolimab should be considered for the treatment of ACH, especially in the patients with intractable nail lesions. [5] Kazinski K, Joyce KM, Hodson D. The successful use of etanercept in combination therapy for treatment of acrodermatitis continua of hallopeau. J Drugs Dermatol. 2005;4(3):360–364. [6] Nikkels A, et al. Etanercept and recalcitrant acrodermatitis continua of Hallopeau. J Drugs Dermatol. 2006;5(8):705–706. [10] Kazinski K, Joyce KM, Hodson D. The successful use of etanercept in combination therapy for treatment of acrodermatitis continua of hallopeau. J Drugs Dermatol. 2005;4(3):360–364. [11] Acrodermatitis continua of Hallopeau is a rare inflammatory disease characterised by pustular eruptions beginning in the tips of fingers and toes. The pustules may vary in extent over a chronic, recurrent course. ... Some treatment options reported in the medical literature to be effective include: Topical therapy. Topical corticosteroids; [14] Acrodermatitis continua of Hallopeau (ACH) is a rare pustular psoriasis variant refractory to many conventional treatments. We report the successful treatment with secukinumab of a patient with a long history of ACH with marked onychodystrophy with frank pustulosis on the nail bed and with accompanying arthritis. [15] Successful treatment of acrodermatitis continua of Hallopeau coexisting with generalized pustular psoriasis with spesolimab: a case report ... It is important to note that the development of anti-drug antibody (ADA) is a significant factor contributing to treatment failure with TNF-α inhibitors (27 ...)

The differential diagnosis for acrodermatitis enteropathica, also known as acrodermatitis-like conditions, includes a range of skin and systemic disorders that can present with similar symptoms.

Common Differential Diagnoses:

• Atopic eczema (typically spares the napkin area, does not result in hair loss, and usually presents after early infancy) [2]
• Psoriasis (involves groin flexures, also known as "napkin psoriasis," with typical plaques elsewhere) [2]
• Contagious impetigo
• Candidiasis
• Other skin diseases related to pathogens [6][7]

Rare but Important Differential Diagnoses:

• Eosinophilic fasciitis (a condition characterized by inflammation of the skin and subcutaneous tissue)
• Endemic syphilis
• Erysipelas
• Erysipeloid
• Lichen sclerosus

Systemic Disorders:

• Sickle cell disease and related diseases
• Protein-energy malnutrition
• Glucagonoma syndrome
• Metabolic disorders that result in a condition known as acrodermatitis dysmetabolic [10]

Other Conditions:

• Papular acrodermatitis of childhood (includes other viral exanthems, insect bites, scabies, papular urticaria, atopic dermatitis, erythema multiforme, Langerhans cell histiocytosis, lichenoid dermatoses) [11]
• Acrodermatitis chronica migrans (a skin sign of the late stage of Lyme disease)
• Acrodermatitis perstans and dermatitis repens (rare subtypes of pustular psoriasis) [9]

Important Considerations:

• A proper diagnosis is essential to avoid unnecessary zinc supplementation, which may lead to copper deficiency and immunological malfunction.
• A skin biopsy may show non-specific histopathology, such as epidermal pallor and confluent parakeratosis [4].
• The inherited autosomal recessive zinc transporter deficiency is termed acrodermatitis enteropathica, and lifelong zinc substitution is recommended [13].

References:

[1] Schwartz RA, et al. (2007). Acrodermatitis enteropathica: a review of the literature.

[2] Atopic eczema and psoriasis are common differential diagnoses for acrodermatitis enteropathica.

[3] Contagious impetigo, candidiasis, and other skin diseases related to pathogens can present with similar symptoms.

[4] A skin biopsy may show non-specific histopathology in cases of acrodermatitis enteropathica.

[5] Eosinophilic fasciitis, endemic syphilis, erysipelas, erysipeloid, lichen sclerosus are rare but important differential diagnoses.

[6] Sickle cell disease and related diseases, protein-energy malnutrition, glucagonoma syndrome, and metabolic disorders can result in acrodermatitis dysmetabolic.

[7] Papular acrodermatitis of childhood includes other viral exanthems, insect bites, scabies, papular urticaria, atopic dermatitis, erythema multiforme, Langerhans cell histiocytosis, lichenoid dermatoses.

[8] Acrodermatitis chronica migrans is a skin sign of the late stage of Lyme disease.

[9] Acrodermatitis perstans and dermatitis repens are rare subtypes of pustular psoriasis.

[10] The inherited autosomal recessive zinc transporter deficiency is termed acrodermatitis enteropathica, and lifelong zinc substitution is recommended.