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CASE REPORT
Year : 1997  |  Volume : 63  |  Issue : 5  |  Page : 312-313

Acrokerato - elastoidosis of costa: Report of three cases




Correspondence Address:
Renu Roy


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Source of Support: None, Conflict of Interest: None


PMID: 20944364

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  Abstract 

Three cases of acrokeratoelastoidosis of Costa are reported. All the patients were females with marginal keratodermas composed of cornified papules, some of which were umbilicated, distributed along the borders of the hands and feet. A positive family history was present in all the cases.


Keywords: Acrokeratoelastoidosis, Keratoderma


How to cite this article:
Roy R, Kalla G, Batra A. Acrokerato - elastoidosis of costa: Report of three cases. Indian J Dermatol Venereol Leprol 1997;63:312-3

How to cite this URL:
Roy R, Kalla G, Batra A. Acrokerato - elastoidosis of costa: Report of three cases. Indian J Dermatol Venereol Leprol [serial online] 1997 [cited 2019 Dec 14];63:312-3. Available from: http://www.ijdvl.com/text.asp?1997/63/5/312/4603


Acrokeratoelastoidosis (AKE) is a rare autosomal, dominantly inherited condition in which firm, shiny papules are seen at the periphery of the palms and soles with some extension to the dorsae of the fingers and the sides of the feet.[1] It is sometimes seen in the Indian patients, but since the treatment is unsatisfactory, the patients stop reporting for follow-up.[2] Only a few cases of AKE have been reported from India. We are reporting three cases of AKE seen in one year.


  Case Report Top


Three female patients (aged 50, 55 and 70 years, respectively) presented with marginal keratodermas composed of shiny, firm cornified papules, some of which showed umbilication, distributed along the borders of the hands [Figure - 1] and feet. These stationary and asymptomatic lesions were present for the last 5-20 years. Mucous membranes, nails, hair and rest of the skin were unremarkable.

Skin biopsies revealed dense keratinization of the epidermis. There was diminution and fragmentation of the elastic fibres, especially in the deeper portions of the dermis. Some of the fragmented elastic fibres appeared thickened and tortuous [Figure - 2]. These observations were further confirmed by Verhoeff's elastic tissue stain with appropriate skin controls. All the three female patients had positive family history (mother, sister, daughter respectively). Routine haemogram and urinalysis did not show any abnormality.


  Discussion Top


AKE is a rare autosomal dominant, marginal inverse keratoderma, involving hands and feet. The positive family history, extent of involvement and histopathology ruled out focal acral hyperkeratosis and degenerative collagenous plaques of hands. Rongioletti et al[3] suggest that focal acral hyperkeratosis, AKE and related disorders should be simply called marginal papular acrokeratodermas. Only a few cases have been reported from India.[4]



 
  References Top

1.Costa OG. Acrokeratosis elastoidosis. Arch Dermatol 1954;70:228-231.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Pasricha JS, Current literature, Dermatology. 1994;9.  Back to cited text no. 2    
3.Rongioletti F, Betti R, Crosti C, et al. Marginal papular acrokeratodermas : A united nosography for focal acral hyperkeratosis, acrokeratoelastoidosis and related disorders. Dermatol 1994;188:28-31.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Sing M, Kanwar A J, Bharija SC, et al. Acrokeratoelastoidosis linearis manus. Ind J Dermatol Venereol Leprol 1987;53:298.  Back to cited text no. 4    


    Figures

[Figure - 1], [Figure - 2]



 

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