Acrokeratoelastoidosis Clinical Presentation

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

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.
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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. Courtesy of William D James, MD. Courtesy of William D James, MD.
  • 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.[11]
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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.[12]
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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

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  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].

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  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].

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  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].

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  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].

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  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].

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