Acrokeratoelastoidosis 

  • Author: Enrico Ceccolini, MD; Chief Editor: William D James, MD   more...
 
Updated: Nov 21, 2011
 

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.

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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.

Two cases of unilaterial acrokeratoelastoidosis have been reported.[9]

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Epidemiology

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.[10]

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Contributor Information and Disclosures
Author

Enrico Ceccolini, MD  Consulting Staff, Department of Dermatology, University of Bologna, Italy. Private Practice, Pesaro, Italy.

Enrico Ceccolini, MD is a member of the following medical societies: American Academy of Dermatology, International Society of Dermatology, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-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.

Specialty Editor Board

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.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

Warren R Heymann, MD  Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

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.

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

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

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

  1. Costa OG. Akrokerato-elastoidosis; a hitherto undescribed skin disease. Dermatologica. 1953;107(3):164-8. [Medline].

  2. Mengesha YM, Kayal JD, Swerlick RA. Keratoelastoidosis marginalis. J Cutan Med Surg. Jan-Feb 2002;6(1):23-5. [Medline].

  3. Rahbari H. Acrokeratoelastoidosis and keratoelastoidosis marginalis-any relation?. J Am Acad Dermatol. Sep 1981;5(3):348-50. [Medline].

  4. 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].

  5. 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].

  6. Yoshinaga E, Ohnishi Y, Tajima S. Acrokeratoelastoidosis associated with nodular scleroderma. Eur J Dermatol. Sep-Oct 2003;13(5):490-2. [Medline].

  7. 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].

  8. 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].

  9. Klekowski N, Shwayder T. Unilateral acrokeratoelastoidosis--second reported case. Pediatr Dermatol. Jan-Feb 2011;28(1):20-2. [Medline].

  10. Hu W, Cook TF, Vicki GJ, Glaser DA. Acrokeratoelastoidosis. Pediatr Dermatol. Jul-Aug 2002;19(4):320-2. [Medline].

  11. 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].

  12. Ramesh V, Avninder S. Endogenous ochronosis with a predominant acrokeratoelastoidosis-like presentation. Int J Dermatol. Aug 2008;47(8):873-5. [Medline].

  13. 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].

  14. Andersen BL, Bierring F. Acrokerato-elastoidosis: a case report. Acta Derm Venereol. 1981;61(1):79-82. [Medline].

  15. Bogle MA, Hwang LY, Tschen JA. Acrokeratoelastoidosis. J Am Acad Dermatol. Sep 2002;47(3):448-51. [Medline].

  16. Civatte J, Hincky M, Barranger C, Vivier O, Degos R. [Acrokerato-elastoidosis]. Ann Dermatol Venereol. Dec 1977;104(12):877-8. [Medline].

  17. 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].

  18. Haneke E, Schwarzenbach I, Hornstein OP. [Delayed manifestation of Costa's acrokeratoelastosis]. Z Hautkr. Mar 1 1977;52(5):170-2. [Medline].

  19. Jacyk WK. Marginal papular acrokeratodermas: classification. Dermatology. 1995;190(2):178-9. [Medline].

  20. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Acquired disorders of elastic tissue: Part II. decreased elastic tissue. J Am Acad Dermatol. Aug 2004;51(2):165-85; quiz 186-8. [Medline].

  21. 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].

  22. Matthews CN. Acrokerato-elastoidosis (without elastorrhexis). Proc R Soc Med. Dec 1974;67(12 Pt 1):1237-8. [Medline].

  23. 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].

  24. Rahbari H. Concerning the article "Acrokerato-elastoidosis. Apropos of 2 cases". Ann Dermatol Venereol. 1987;114(6-7):867-8. [Medline].

  25. 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].

  26. Tsai S, Kageyama N, Warthan M, Cockerell CJ. Acrokeratoelastoidosis. Int J Dermatol. May 2005;44(5):406-7. [Medline].

  27. Zhai Z, Yang X, Hao F. Acrokeratoelastoidosis. Eur J Dermatol. Mar-Apr 2006;16(2):201-2. [Medline].

 
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