Acrokeratoelastoidosis
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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.
The cause of acrokeratoelastoidosis (AKE) is not known. Autosomal dominant transmission is common, but the clinical expressions vary widely. Acrokeratoelastoidosis-like lesions on the palms of patients have 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 two 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 unilateral acrokeratoelastoidosis have been reported. [9]
No local or systemic causes have been identified. Autosomal dominant transmission is common. Sporadic cases of acrokeratoelastoidosis (AKE) are also described. One report described a patient with endogenous ochronosis showing clinical features similar to acrokeratoelastoidosis. [10]
United States
Acrokeratoelastoidosis (AKE) is rare.
International
The eruption is rare, and when the lesions are few, acrokeratoelastoidosis often remains unnoticed. A survey of 500 consecutive elderly outpatient dermatology patients at the medical school in Puducherry, India found acrokeratoelastoidosis marginalis in 2 (0.9%) females. [11]
Women appear to be affected more frequently than men.
Acrokeratoelastoidosis is not congenital. It arises at puberty or sometimes later. Some cases have been described in the pediatric dermatologic literature. [12]
Once present, the eruption is stable, with no adverse effects. Recurrences are common.
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. 2002 Jan-Feb. 6(1):23-5. [Medline].
Rahbari H. Acrokeratoelastoidosis and keratoelastoidosis marginalis-any relation?. J Am Acad Dermatol. 1981 Sep. 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. 2004 Mar-Apr. 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. 2003 Sep-Oct. 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. 2002 May. 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].
Klekowski N, Shwayder T. Unilateral acrokeratoelastoidosis–second reported case. Pediatr Dermatol. 2011 Jan-Feb. 28(1):20-2. [Medline].
Ramesh V, Avninder S. Endogenous ochronosis with a predominant acrokeratoelastoidosis-like presentation. Int J Dermatol. 2008 Aug. 47(8):873-5. [Medline].
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].
Hu W, Cook TF, Vicki GJ, Glaser DA. Acrokeratoelastoidosis. Pediatr Dermatol. 2002 Jul-Aug. 19(4):320-2. [Medline].
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].
Uribe P, Ortiz E, Wortsman X, Gonzalez S. Acrokeratoelastoidosis of the Foot with Clinical, Dermoscopic, Ultrasonographic, and Histopathologic Correlation. J Am Podiatr Med Assoc. 2018 Mar. 108 (2):178-181. [Medline].
Liu JH, Luo DQ, Wu LC, Zhang HY. Plaques: a rare presentation of acrokeratoelastoidosis. Cutis. 2014 Aug. 94(2):E1-2. [Medline].
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].
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].
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].
Duchatelet S, Hovnanian A. Olmsted syndrome: clinical, molecular and therapeutic aspects. Orphanet J Rare Dis. 2015 Mar 17. 10:33. [Medline].
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].
Lopes JF, de Almeida HL Jr, da Cunha Filho RR, Viganó Lattman B, Suíta de Castro LA. Ultrastructure of acrokeratoelastoidosis. J Eur Acad Dermatol Venereol. 2018 May. 32 (5):e165-e167. [Medline].
Rambhia KD, Khopkar US. Acrokeratoelastoidosis. Indian Dermatol Online J. 2015 Nov-Dec. 6 (6):460-1. [Medline].
Shiiya C, Hata H, Inamura Y, Imafuku K, Kitamura S, Yanagi T, et al. Acrokeratoelastoidosis successfully treated with 10% salicylic acid ointment. J Dermatol. 2017 Mar. 44 (3):e46-e47. [Medline].
Mu EW, Mir A, Meehan SA, Nguyen N. Acrokeratoelastoidosis. Dermatol Online J. 2015 Dec 16. 21 (12):[Medline].
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].
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.
Enrico Ceccolini, MD Consulting Staff, Department of Dermatology, University of Bologna; 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.
Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology
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.
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: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.
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.
Acrokeratoelastoidosis
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