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Dermatology > PEDIATRIC DISEASES
Acrokeratoelastoidosis
Article Last Updated: Feb 21, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Enrico Ceccolini, MD, Consulting Staff, Department of Dermatology, University of Bologna, Italy
Enrico Ceccolini is a member of the following medical societies: American Academy of Dermatology
Coauthor(s):
Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Editors: Carrie L Kovarik, MD, Assistant Professor, Department of Dermatology and Dermatopathology, University of Pennsylvania School of Medicine; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey; Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
Costa acrokeratoelastoidosis, Costa's acrokeratoelastoidosis, AK, AKE, keratoelastoidosis marginalis, type III punctate palmoplantar keratoderma, type 3 punctate palmoplantar keratoderma, focal acral hyperkeratosis
Background
Acrokeratoelastoidosis (AKE) is a rare genodermatosis characterized by small, firm papules or plaques on the sides of the hands and feet. These nodules may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. AKE was first described in 1953 by Costa.
Both autosomal dominant and sporadic forms have been observed. AKE is not congenital; it slowly arises at puberty, or sometimes later, and then remains stable. Usually, no treatment is necessary. AKE is similar to 2 other diseases: keratoelastoidosis marginalis and focal acral hyperkeratosis. The clinical and histologic differences among these diseases allow their distinction.
Pathophysiology
The cause of AKE is not known. Autosomal dominant transmission is common, but the clinical expressions vary widely. AKE-like lesions on the palms of patients have recently been noted in association with systemic or localized scleroderma, possibly due to an altered pattern of connective tissue metabolism similar to that of systemic scleroderma. In 2003, Yoshinaga et al reported on a patient with AKE in association with localized scleroderma. In 2002, Tajima et al found a high rate of AKE in patients with systemic scleroderma (7 in 26 systemic sclerodermas). No other reports have confirmed these findings, and the relationship between these 2 diseases is not conclusive. A possible linkage to chromosome 2 has also been proposed (Greiner, 1983), but further studies are needed to confirm this hypothesis.
Frequency
United States
AKE is rare.
International
The eruption is rare, and when the lesions are few, AKE often remains unnoticed.
Mortality/Morbidity
Once present, the eruption is stable, with no adverse effects.
Sex
Women appear to be affected more frequently than men.
Age
AKE is not congenital. It arises at puberty or sometimes later. Some cases have been described in the pediatric dermatologic literature (Hu, 2002).
History
- The patient may complain of the gradual onset of small bumps over the margins of the hands and feet.
- After onset, the eruption remains stable indefinitely.
- No local or systemic symptoms are associated with AKE. Some reports show AKE in patients with localized or systemic scleroderma, but the relationship between these 2 diseases is not conclusive.
Physical
- A cluster of small, discrete, grouped papules characterizes AKE.
- The papules are usually 2-5 mm in diameter and often occur in a linear distribution. These small round-oval– to rhomboid–shaped yellowish papules are most commonly localized to the palmar surfaces of the hands and, sometimes, on the plantar surfaces of the feet. The margins of both hands and 1 or both feet are the only areas affected. In rare instances, the lesions spread to the dorsum of the hands, feet, or both.
- The papules resemble plane warts, but they are more keratotic and firm; they do not coalesce.
- Some translucency is often evident.
- Occasionally, just a few papules are present.
- Recently, a case of AKE has been seen in association with nail dystrophic changes. Further observations may lead to the definition of a new entity.
Causes
- No local or systemic causes have been identified.
- Autosomal dominant transmission is common.
- Sporadic cases of AKE are also described.
Acrodynia
Acrokeratosis Verruciformis of Hopf
Other Problems to be Considered
Keratoelastoidosis marginalis, also called degenerative collagenous plaques of the hands, is related to sun exposure or local trauma.
Focal acral hyperkeratosis has no dermal changes, though these are characteristic features of AKE.
Lab Studies
- Laboratory studies or other studies are usually not necessary.
- Biopsy can be helpful for diagnostic purposes and for ruling out other disorders.
Histologic Findings
The typical epidermal features of AKE are hyperkeratosis with hypergranulosis, acanthosis, and epidermal hyperplasia. Some of these findings may be absent.
The changes in the dermal elastic fibers are characteristic. The number of elastic fibers is often diminished, and the fibers have a discrete-to-intense fragmentation. An elastic fiber stain, such as the Weigert, Verhoeff, or orcein stain, may be useful in demonstrating this fragmentation. Periodic acid-Schiff (PAS) is used to stain carbohydrates, and occasionally, the PAS test is used to rule out other conditions. The cell walls of fungi are rich in carbohydrates, and they stain positively with PAS. PAS does not stain elastic fibers.
Medical Care
- Treatment is not indicated in most patients.
- Mild keratolytics occasionally help, but recurrences are common.
- Topical retinoids are not effective.
Complications
- Once present, the eruption is stable, with no adverse effects.
Prognosis
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Acrokeratoelastoidosis excerpt Article Last Updated: Feb 21, 2007
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