Acrokeratoelastoidosis - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Acrokeratoelastoidosis or AKE is a rare dermal condition affecting childhood. Acrokeratoelastoidosis or AKE is one of the inherited forms of marginal keratoderma. The symptoms of acrokeratoelastoidosis or AKE are observed in palmoplantar regions such as palms, soles, and dorsal hands. Acrokeratoelastoidosis, or 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, or AKE, was first described in 1952 by a Brazilian dermatologist named Oswaldo costa. Acrokeratoelastoidosis, or AKE, is an inherited form of marginal keratoderma and a type of palmoplantar keratoderma. Acrokeratoelastoidosis or AKE is characterized as flesh-colored papules on the lateral aspect of the palms, in the dorsal aspect of the hands, and on the soles. Acrokeratoelastoidosis or AKE is also known as inverse papular acrokeratoelastoidosis or AKE. The primary mode of inheritance of acrokeratoelastoidosis or AKE is autosomal dominant. The exact etiology of acrokeratoelastoidosis or AKE is unknown. Acrokeratoelastoidosis, or AKE, 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 of acrokeratoelastoidosis or AKE 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 or AKE.

The causes for acrokeratoelastoidosis or 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 Are the Symptoms of Acrokeratoelastoidosis or AKE?

The symptoms are present in the palmoplantar regions as it is called palmoplantar keratoderma. Acrokeratoelastoidosis, or AKE, 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, or AKE, usually shows a crateriform to umbilicated appearance. Acrokeratoelastoidosis or AKE, 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 acrokeratoelastoidosis or AKE are mostly bilateral and symmetrical and sometimes show unilateral involvement. The papules of acrokeratoelastoidosis or AKE coalesce to form plaques. Acrokeratoelastoidosis, or AKE, is an asymptomatic condition.

The symptoms of acrokeratoelastoidosis or AKE 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 or AKE?

Acrokeratoelastoidosis diagnosis or AKE 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 or AKE. The stains used for the histopathological study of acrokeratoelastoidosis or AKE is the hematoxylin-eosin or Hand E stain, Verhoeff’s Van Gieson stain, Weigert and Orcein stain. Doppler study is also done, and it is a highly useful and noninvasive method of examination for acrokeratoelastoidosis or AKE.

The various diagnoses of acrokeratoelastoidosis or AKE 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 Treatment of Acrokeratoelastoidosis or AKE?

Acrokeratoelastoidosis or AKE 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 or AKE are given below.

• Topical therapy for the treatment of acrokeratoelastoidosis or AKE 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 or AKE?

Acrokeratoelastoidosis or AKE 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 or AKE 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 verruciform of Hopf.

• Degenerative collagenous plaques.

• Digital papular calcinosis.

• Verruca plana.

• Primary cutaneous amyloidosis.

• Mosaic acral keratosis.

Conclusion

Acrokeratoelastoidosis or AKE is a benign asymptomatic dermal condition that occurs due to chronic sun exposure and an autosomal dominant gene. Acrokeratoelastoidosis, or AKE, is characterized by the papules in the palms and soles of the hands and feet. Symptoms of acrokeratoelastoidosis or 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 histopathological finding in acrokeratoelastoidosis or AKE. The treatment is done to maintain the cosmesis, and it is found that oral retinoid therapy will improve the symptoms. Acrokeratoelastoidosis or AKE is not a fatal disease, and the prognosis of acrokeratoelastoidosis or AKE is good.