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Acrokeratoelastoidosis Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Jun 08, 2016
 

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. Acrokeratoelastoidosis evident as a unilateral sporadic keratosis on the hand and foot has been described in a prepubescent child.[12]

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 one or both feet are the only areas affected. In rare instances, the lesions spread to the dorsum of the hands, feet, or both. See the image below.

Acrokeratoelastoidosis. Courtesy of William D JameAcrokeratoelastoidosis. 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. It may rarely appear as plaques.[13]

A case of acrokeratoelastoidosis has been seen in association with nail dystrophic changes. Further observations may lead to the definition of a new entity.[14]

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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.[15]

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

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, Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

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. 2002 Jan-Feb. 6(1):23-5. [Medline].

  3. Rahbari H. Acrokeratoelastoidosis and keratoelastoidosis marginalis-any relation?. J Am Acad Dermatol. 1981 Sep. 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. 2004 Mar-Apr. 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. 2003 Sep-Oct. 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. 2002 May. 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. 2011 Jan-Feb. 28(1):20-2. [Medline].

  10. Durai PC, Thappa DM, Kumari R, Malathi M. Aging in elderly: chronological versus photoaging. Indian J Dermatol. 2012 Sep. 57(5):343-52. [Medline]. [Full Text].

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

  12. AlKahtani HS, AlHumidi AA, Al-Hargan AH, Al-Sayed AA. A sporadic case of unilateral acrokeratoelastoidosis in Saudi Arabia: a case report. J Med Case Rep. 2014 May 8. 8:143. [Medline]. [Full Text].

  13. Liu JH, Luo DQ, Wu LC, Zhang HY. Plaques: a rare presentation of acrokeratoelastoidosis. Cutis. 2014 Aug. 94(2):E1-2. [Medline].

  14. van Steensel MA, Verstraeten VL, Frank J. Acrokeratoelastoidosis with nail dystrophy: a coincidence or a new entity?. Arch Dermatol. 2006 Jul. 142(7):939-41. [Medline].

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

  16. Poiraud C, Vourc'h-Jourdain M, Cassagnau E, Barbarot S. Aquagenic palmoplantar keratoderma associated with acrokeratoelastoidosis. Clin Exp Dermatol. 2014 Jul. 39(5):671-2. [Medline].

  17. Koudoukpo C, Bourrat E, Rausky J, Yédomon H, Bagot M, Blanchet-Bardon C, et al. [Ainhum and "African acral keratoderma": three cases]. Ann Dermatol Venereol. 2015 Mar. 142 (3):170-5. [Medline].

  18. Duchatelet S, Hovnanian A. Olmsted syndrome: clinical, molecular and therapeutic aspects. Orphanet J Rare Dis. 2015 Mar 17. 10:33. [Medline].

  19. Costa MC, Bornhausen Demarch E, Hertz A, Pereira FB, Azulay DR. Case for diagnosis. Acrokeratoelastoidosis. An Bras Dermatol. 2011 Nov-Dec. 86(6):1222-3. [Medline].

  20. Rambhia KD, Khopkar US. Acrokeratoelastoidosis. Indian Dermatol Online J. 2015 Nov-Dec. 6 (6):460-1. [Medline].

  21. Mu EW, Mir A, Meehan SA, Nguyen N. Acrokeratoelastoidosis. Dermatol Online J. 2015 Dec 16. 21 (12):[Medline].

  22. Erbil AH, Sezer E, Koç E, Tunca M, Tastan HB, Demiriz M. Acrokeratoelastoidosis treated with the erbium:YAG laser. Clin Exp Dermatol. 2008 Jan. 33(1):30-1. [Medline].

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Acrokeratoelastoidosis. Courtesy of William D James, MD.
 
 
 
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