eMedicine Specialties > Dermatology > Pediatric Diseases

Acrokeratoelastoidosis

Enrico Ceccolini, MD, Consulting Staff, Department of Dermatology, University of Bologna, Italy
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

Updated: Dec 10, 2008

Introduction

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. Acrokeratoelastoidosis was first described in 1953 by Costa.¹

Both autosomal dominant and sporadic forms have been observed. Acrokeratoelastoidosis is not congenital; it slowly arises at puberty, or sometimes later, and then remains stable. Usually, no treatment is necessary. Acrokeratoelastoidosis is similar to 2 other diseases: keratoelastoidosis marginalis^2,3 and focal acral hyperkeratosis.^4,5 The clinical and histologic differences among these diseases allow their distinction.

Pathophysiology

The cause of acrokeratoelastoidosis is not known. Autosomal dominant transmission is common, but the clinical expressions vary widely. Acrokeratoelastoidosis-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 Acrokeratoelastoidosis in association with localized scleroderma.⁶ In 2002, Tajima et al found a high rate of Acrokeratoelastoidosis in patients with systemic scleroderma (7 in 26 systemic sclerodermas).⁷ 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,⁸ but further studies are needed to confirm this hypothesis.

Frequency

United States

Acrokeratoelastoidosis is rare.

International

The eruption is rare, and when the lesions are few, Acrokeratoelastoidosis 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

Acrokeratoelastoidosis is not congenital. It arises at puberty or sometimes later. Some cases have been described in the pediatric dermatologic literature.⁹

Clinical

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 acrokeratoelastoidosis. Some reports show acrokeratoelastoidosis 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 acrokeratoelastoidosis.
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.
A case of acrokeratoelastoidosis has been seen in association with nail dystrophic changes. Further observations may lead to the definition of a new entity.¹⁰

Causes

No local or systemic causes have been identified.
Autosomal dominant transmission is common.
Sporadic cases of acrokeratoelastoidosis are also described.
One report described a patient with endogenous ochronosis showing clinical features similar to acrokeratoelastoidosis.¹¹

Differential Diagnoses

Acrodynia
Acrokeratosis Verruciformis of Hopf

Workup

Laboratory 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 acrokeratoelastoidosis 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.

Treatment

Medical Care

Treatment is not indicated in most patients.
Mild keratolytics occasionally help, but recurrences are common.
Topical retinoids are not effective.
The erbium:YAG laser has been effective in improving one patient. No recurrence developed during a follow-up of 6 months.¹²
The Medscape Dermatologic Surgery Resource Center may be of interest.

Follow-up

Complications

Once present, the eruption is stable, with no adverse effects.

Prognosis

Recurrences are common.

Multimedia

Media file 1: Courtesy of William D James, MD.

References

Costa OG. Akrokerato-elastoidosis; a hitherto undescribed skin disease. Dermatologica. 1953;107(3):164-8. [Medline].
Mengesha YM, Kayal JD, Swerlick RA. Keratoelastoidosis marginalis. J Cutan Med Surg. Jan-Feb 2002;6(1):23-5. [Medline].
Rahbari H. Acrokeratoelastoidosis and keratoelastoidosis marginalis-any relation?. J Am Acad Dermatol. Sep 1981;5(3):348-50. [Medline].
Erkek E, Kocak M, Bozdogan O, Atasoy P, Birol A. Focal acral hyperkeratosis: a rare cutaneous disorder within the spectrum of Costa acrokeratoelastoidosis. Pediatr Dermatol. Mar-Apr 2004;21(2):128-30. [Medline].
Rongioletti F, Betti R, Crosti C, Rebora A. Marginal papular acrokeratodermas: a unified nosography for focal acral hyperkeratosis, acrokeratoelastoidosis and related disorders. Dermatology. 1994;188(1):28-31. [Medline].
Yoshinaga E, Ohnishi Y, Tajima S. Acrokeratoelastoidosis associated with nodular scleroderma. Eur J Dermatol. Sep-Oct 2003;13(5):490-2. [Medline].
Tajima S, Tanaka N, Ishibashi A, Suzuki K. A variant of acrokeratoelastoidosis in systemic scleroderma: report of 7 cases. J Am Acad Dermatol. May 2002;46(5):767-70. [Medline].
Greiner J, Kruger J, Palden L, Jung EG, Vogel F. A linkage study of acrokeratoelastoidosis. Possible mapping to chromosome 2. Hum Genet. 1983;63(3):222-7. [Medline].
Hu W, Cook TF, Vicki GJ, Glaser DA. Acrokeratoelastoidosis. Pediatr Dermatol. Jul-Aug 2002;19(4):320-2. [Medline].
van Steensel MA, Verstraeten VL, Frank J. Acrokeratoelastoidosis with nail dystrophy: a coincidence or a new entity?. Arch Dermatol. Jul 2006;142(7):939-41. [Medline].
Ramesh V, Avninder S. Endogenous ochronosis with a predominant acrokeratoelastoidosis-like presentation. Int J Dermatol. Aug 2008;47(8):873-5. [Medline].
Erbil AH, Sezer E, Koç E, Tunca M, Tastan HB, Demiriz M. Acrokeratoelastoidosis treated with the erbium:YAG laser. Clin Exp Dermatol. Jan 2008;33(1):30-1. [Medline].
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Fiallo P, Pesce C, Brusasco A, Nunzi E. Acrokeratoelastoidosis of Costa: a primary disease of the elastic tissue?. J Cutan Pathol. Nov 1998;25(10):580-2. [Medline].
Haneke E, Schwarzenbach I, Hornstein OP. [Delayed manifestation of Costa's acrokeratoelastosis]. Z Hautkr. Mar 1 1977;52(5):170-2. [Medline].
Jacyk WK. Marginal papular acrokeratodermas: classification. Dermatology. 1995;190(2):178-9. [Medline].
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Masse R, Quillard A, Hery B, Toudic L, Le Her G. [Costa's acrokerato-elastoidosis. Ultrastructural study (author's transl)]. Ann Dermatol Venereol. Jun-Jul 1977;104(6-7):441-5. [Medline].
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Matthews CN, Harman RR. Acrokerato-elastoidosis in a Somerset mother and her two sons. Br J Dermatol. Jul 1977;97 Suppl 15:42-3. [Medline].
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Sehgal VN, Singh M, Korrane RV, Nayyar M, Chandra M. Degenerative collagenous plaque of the hand (linear keratoelastoidosis of the hands). A variant of acrokeratoelastosis. Dermatologica. 1980;161(3):200-4. [Medline].
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Keywords

acrokeratoelastoidosis, Costa acrokeratoelastoidosis, Costa's acrokeratoelastoidosis, AK, AKE, keratoelastoidosis marginalis, type III punctate palmoplantar keratoderma, type 3 punctate palmoplantar keratoderma, focal acral hyperkeratosis

Contributor Information and Disclosures

Author

Enrico Ceccolini, MD, Consulting Staff, Department of Dermatology, University of Bologna, Italy
Enrico Ceccolini, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Carrie L Kovarik, MD, Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine
Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

Further Reading