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Acrokeratoelastoidosis - Causes, Symptoms, Diagnosis, and Treatment

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Acrokeratoelastoidosis is a benign condition that is observed in childhood. It is an inherited form of marginal keratoderma. Read below to know more.

Written byDr. Karthika Rp

Medically reviewed byDr. Filza Hafeez

Published At November 25, 2022
Reviewed AtAugust 9, 2024

Introduction

Acrokeratoelastoidosis, or AKE, is a rare dermal condition affecting childhood. It is one of the inherited forms of marginal keratoderma. The symptoms of AKE are observed in palmoplantar regions such as palms, soles, and dorsal hands. AKE is the autosomal dominant type of dermal condition.

What Is Acrokeratoelastoidosis or AKE?

Acrokeratoelastoidosis, or AKE, is a rare and benign dermal condition typically observed in childhood. Acrokeratoelastoidosis was first described in 1952 by a Brazilian dermatologist named Oswaldo Costa. Acrokeratoelastoidosis is an inherited form of marginal keratoderma and a type of palmoplantar keratoderma. It is characterized as flesh-colored papules on the lateral aspect of the palms, in the dorsal aspect of the hands, and on the soles.

AKE is also known as inverse papular acrokeratoelastoidosis. The primary mode of inheritance of acrokeratoelastoidosis is autosomal dominant. The exact etiology is unknown. It is a benign and asymptomatic condition, and the treatment is done to correct the cosmesis.

What Causes Acrokeratoelastoidosis or AKE?

The exact cause for the pathogenesis of acrokeratoelastoidosis or AKE is unknown. It is observed in many cases that the cause of the incidence is the autosomal dominant type of dermal condition. Reduced expression of cytosolic p34 protein causes cell proliferation in the epidermis layer of the skin. There are some other factors found to be contributory factors to acrokeratoelastoidosis.

The causes for AKE are given below.

  • Autosomal dominant gene.

  • Marginal keratoderma.

  • Repeated trauma to palms.

  • Irritation in ill-fitting footwear.

  • Palmoplantar keratoderma.

  • Chronic trauma.

  • Scleroderma.

  • Abnormal metabolism in the connective tissues.

  • Excessive or prolonged sun exposure.

  • Ultraviolet rays or UV rays.

  • Other factors include handwashing, clothing, and minor trauma.

What Is the Pathophysiology of Acrokeratoelastoidosis?

Acrokeratoelastoidosis (AKE) is a rare inherited skin disorder characterized by small, firm, yellowish papules that typically appear on the sides of the hands and feet. This condition is generally benign but can be cosmetically concerning for affected individuals. The pathophysiology of AKE involves genetic, histological, and structural skin changes.

What Are the Symptoms of Acrokeratoelastoidosis?

The symptoms are present in the palmoplantar regions, as it is called palmoplantar keratoderma. Acrokeratoelastoidosis is a benign dermal condition. It is often characterized as flesh-colored or translucent or yellowish-colored firm papules; clusters of small papules in the round to oval shape are observed in the palmoplantar regions such as palms, soles, and feet. Acrokeratoelastoidosis usually shows a crateriform to umbilicated appearance. It is also known as papular acrokeratosis, and the symptoms are mostly seen in the palmar aspect of the hands and feet. Symptoms are also observed in the feet and the tibia posterior aspect. Symptoms of AKE are mostly bilateral and symmetrical and sometimes show unilateral involvement. The papules of AKE coalesce to form plaques, an asymptomatic condition.

The symptoms of acrokeratoelastoidosis are listed below.

  • Small round to oval papules.

  • The color papules are skin-colored and translucent.

  • Yellowish hue papules are firm.

  • Rough surfaced keratotic papules.

  • Distribution of papules present with crateriform to umbilical appearance.

  • In papular acrokeratosis, lesions are seen on the hands and feet.

  • The pretibial region and posterior part of the feet are occasionally involved.

  • Plaques are present.

  • Mild itching.

  • Hyperhidrosis.

  • Palmoplantar keratoderma may be present.

What Is the Diagnosis of Acrokeratoelastoidosis?

Acrokeratoelastoidosis diagnosis is based on dermoscopy, histopathological findings, and ultrasonography results. Dermoscopy is the polarized video dermoscopic evaluation of affected areas. The lesions on the thumbs and fingers are examined with dermoscopy. Histopathological findings are the gold standard diagnostic tool for diagnosing acrokeratoelastoidosis. The stains used for the histopathological study of acrokeratoelastoidosis are the hematoxylin-eosin or Hand E stain, Verhoeff’s Van Gieson stain, Weigert and Orcein stain. A Doppler study is also conducted, and it is a highly useful and noninvasive method of examination for acrokeratoelastoidosis.

The various diagnoses of acrokeratoelastoidosis are mentioned below.

  • History of sun exposure.

  • History of chronic trauma.

  • Physical examination of papular lesions on the palmar aspect of the hands and in the feet.

  • Dermoscopy involves the polarized video dermoscopic tool that will examine the affected areas such as the index and thumbs, and pale yellow colored papules in affected areas are present.

  • Histopathological findings of AKE will include the following features.

  • Altered epidermis.

  • Dermis show thick and fragmented elastic fibers.

  • Elastorrhexis is present.

  • Ultrasonography reveals focal hypoechoic and hypovascular areas.

What Is the Prognosis of Acrokeratoelastoidosis?

Individuals with acrokeratoelastoidosis generally have a good prognosis as the condition is benign and does not typically cause significant health problems beyond the cosmetic changes. There is no specific cure for acrokeratoelastoidosis and the main focus on treatment is managing symptoms and cosmetic concerns.

What Is the Treatment of Acrokeratoelastoidosis?

Acrokeratoelastoidosis is an asymptomatic benign condition, and treatment is done only to maintain the cosmesis of the patient. Patients must be informed about the effects that arise due to the stoppage of drugs and the side effects of the drugs.

The different treatment methods for acrokeratoelastoidosis are given below.

  • Topical therapy for the treatment of acrokeratoelastoidosis is given below.

  • Emollients.

  • Keratolytic agents include salicylic acid, sulfur, coal tar, urea, and tretinoin.

  • Topical corticosteroids.

  • Oral therapy.

  • Oral administration of corticosteroids.

  • Oral retinoids and antibiotics.

  • Dapsone.

  • Methotrexate.

  • Isotretinoin.

  • Acitretin.

  • Surgical therapy.

  • Cryotherapy.

  • Liquid nitrogen.

  • In laser surgery, the laser used is the yttrium-aluminum-garnet or Er.YAG.

What Is the Differential Diagnosis of Acrokeratoelastoidosis?

Acrokeratoelastoidosis is a type of marginal keratoderma, and the symptoms are similar to the other types of marginal and acral keratoderma.

The differential diagnosis of acrokeratoelastoidosis is listed below.

  • Focal acral hyperkeratosis.

  • Ramos and Silva marginal keratoderma, also known as keratoelastoidosis marginalis.

  • Hereditary papulotranslucent acrokeratoderma.

  • Acrokeratoderma hereditary punctatum.

  • Punctate palmoplantar keratoderma.

  • Acrokeratosis verruciformis of Hopf.

  • Degenerative collagenous plaques.

  • Digital papular calcinosis.

  • Verruca plana.

  • Primary cutaneous amyloidosis.

  • Mosaic acral keratosis.

Conclusion

Acrokeratoelastoidosis is a benign asymptomatic dermal condition that occurs due to chronic sun exposure and an autosomal dominant gene. It is characterized by the papules in the palms and soles of the hands and feet. Symptoms of AKE include the yellow or skin-colored papules present in the hands and feet. Elastorrhexis, called the cluster of fragmented elastic fibers, is the hallmark of histopathological findings in acrokeratoelastoidosis. The treatment is done to maintain the cosmesis, and it is found that oral retinoid therapy will improve the symptoms. Acrokeratoelastoidosis is not a fatal disease, and the prognosis is good.

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Frequently Asked Questions

Focal acral hyperkeratosis is a less common skin disorder in which multiple small, firm, yellowish to white papules (a solid or a cystic raised spot on the skin surface which is less than 1 cm. in width) and plaques are present on the palms and soles. These are predominantly found in palmar (palms) and plantar (foot) regions. Many forms of hyperkeratosis are localized conditions with good outcomes (prognosis).
Marginal keratoderma is a papular (papules are small, solid, raised bumps on the skin that have edges lined with borders and can be easily differentiated) palmoplantar (involving the hands and the feet) keratoderma characterized by small firm, warty, or pearly papules located on the sides of the hand and also, on the feet in a few cases. 
Treatment for keratoderma includes the use of topical medications such as emollients (substances that can soften or soothe the skin), keratolytic (compounds that break down the epidermis of the skin and decrease the thickness), retinoids (group of compounds that are derived from Vitamin A), and steroids. Systemic treatment includes various retinoids. Topical and systemic treatments may be used in combination to get relief from the symptoms.
Though no permanent cure exists, palmoplantar keratoderma can be treated symptomatically. Medical treatments and surgical excision (removal) are the current treatment modalities, but these treatments are associated with limited success rates, recurrence, and significant morbidity. Inherited keratoderma cannot be cured entirely, but the acquired forms can be cured completely.
The inherited forms of keratoderma are caused by gene mutations that form an abnormal skin protein called keratin. Acquired keratoderma occurs due to changes in the person's environment or health. In the keratoderma of the feet, there is an abnormal thickening of the skin's outer layer due to dead skin cells on the soles of the feet. This occurs when the soles are subjected to too much pressure, which triggers the production of excessive keratin, which causes excessive skin thickening.
Acquired palmoplantar keratoderma can be caused by drugs, menopause, chemicals like arsenic, malnutrition, mechanical stimulation, systemic conditions like thyroid disease, malignancies like lung cancer colon, cancer, skin conditions like eczema, psoriasis, and infectious diseases like tuberculosis, syphilis, and so on. Inherited keratoderma can be transferred in families, and most cases are due to a defective gene-producing keratin 9.
Keratosis pilaris, also called 'chicken skin,' is a common skin condition characterized by patches of rough-feeling bumps on the skin. If creams or other treatments are unsuccessful, the dermatologist (skin specialist) may suggest surgical treatments such as gentle acne extraction, a minor therapy. Instruments are used in the surgical method to remove the entrapped coiled hairs or keratin plugs below the skin that lead to keratosis.
Palmoplantar keratoderma is the most common heterogeneous group of inherited and acquired disorders with abnormal thickening of the palms and soles. Multiple case reports have shown the association of palmoplantar keratoderma with autoimmune thyroiditis (a condition in which antibodies are produced against the thyroid cells). Autoimmune conditions are associated with palmoplantar keratoderma.
The hereditary type of keratoderma is not that rare and can be identified and diagnosed genetically, but treatment is based on the symptoms. Palmoplantar keratoderma is a rare disease that manifests in the palms and soles and can vary in different persons. Acquired keratoderma depends on the individual's exposure to extrinsic factors and is likely to develop during adulthood.
A hyperkeratotic (abnormal thickening of the skin's outer layer) lesion that forms due to the accumulation of dead skin cells on the soles of the feet is called plantar keratosis. It usually occurs when the soles of the feet are subjected to too much pressure. This pressure triggers the production of excessive keratin, which causes excessive skin thickening. The term 'plantar' refers to the 'sole'.
The palms and soles of the feet in humans may be around 0.8 to 1.4 mm. thick to offer protection against frequent pressure and rubbing. The epithelium on the skin in other body parts is usually 0.1 mm. thick. The extra thickness in the palms is due to the presence of a protein called keratin, which is present in the skin's deep layers but forms a layer of dead cells at the skin's surface.
Keratosis pilaris is a skin condition characterized by a single rash or irregular patterns of rashes that consist of tiny bumps. When these bumps get inflamed, itching is a common symptom. Scratching an itchy rash could lead to infection, which needs to be treated. Besides the formation of hyperkeratotic layers in keratoderma, papules, and plaques are common, which can cause an itching sensation. It is better to consult a doctor in such cases.
Keratoderma that occurs on the feet is called plantar keratoderma. Treatment options to treat keratoderma on the feet include the use of emollients (substances that can soften or soothe the skin), keratolytics (compounds that break down the epidermis of the skin and decrease the thickness) such as salicylic acid or urea, antifungal cream, or tablets if required, topical retinoids (derivatives of Vitamin A) like calcipotriol and systemic retinoids.
Even with appropriate treatment, the symptoms of keratosis pilaris can take some time to go away. With proper and regular treatment, improvements can be noticed within 4-6 weeks. Without treatment, most cases of keratosis pilaris resolve around the mid-20s and mostly disappear completely by 30 years of age. Hormonal changes during puberty and pregnancy can cause flare-ups and are most common in fair-skinned people.
Keratoderma can be both inherited as well as acquired. Acquired forms of keratoderma are the most common. The inherited forms are caused by gene abnormalities or mutations that result in the formation of an abnormal skin protein called keratin. Acquired keratoderma occurs due to changes in the person's environment or health.
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Dr. Filza Hafeez from iCliniq
Dr. Filza Hafeez

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