• Users Online: 1552
  • Print this page
  • Email this page


 
  Table of Contents  
SKINDIA QUIZ
Year : 2015  |  Volume : 6  |  Issue : 2  |  Page : 134-135  

SkIndia Quiz 17: Linear dermatoses over the hand and foot


Department of Dermatology, Goa Medical College, Bambolim, Goa, India

Date of Web Publication11-Mar-2015

Correspondence Address:
Dr. Varadraj V Pai
Department of Dermatology, Goa Medical College, Bambolim - 430 002, Goa
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.148972

Rights and Permissions

How to cite this article:
Pai VV, Shukla P, Godhge R, Bhandhare P. SkIndia Quiz 17: Linear dermatoses over the hand and foot. Indian Dermatol Online J 2015;6:134-5

How to cite this URL:
Pai VV, Shukla P, Godhge R, Bhandhare P. SkIndia Quiz 17: Linear dermatoses over the hand and foot. Indian Dermatol Online J [serial online] 2015 [cited 2019 Aug 23];6:134-5. Available from: http://www.idoj.in/text.asp?2015/6/2/134/148972

A 20-year-old male student presented to the dermatology outpatient department with a history of thickening over the right palm and left foot since childhood. The lesions were asymptomatic. There was no history of similar lesions among the family members. On examination, linear hyperkeratosis was noted along the lateral margin of the right palm extending from the thenar eminence to the thumb tip. Similar linear hyperkeratosis was noted over the dorsum of the foot [Figure 1] and [Figure 2]. On investigation, all hematological and biochemical parameters were normal. Histopathology from the palm revealed massive hyperkeratosis with acanthosis of the epidermis [Figure 3]. Topical keratolytics provided only marginal improvement.
Figure 1: Linear hyperkeratosis along the lateral margin of the right palm extending from the thenar eminence the thumb tip

Click here to view
Figure 2: Linear hyperkeratosis over the left foot

Click here to view
Figure 3: Histopathology revealed massive hyperkeratosis with acanthosis of the epidermis. (H and E, ×40)

Click here to view



   What is Your Diagnosis? Top



   Answer Top


Striate palmoplantar keratoderma (Brunauer-Fuhs-Siemens type).


   Discussion Top


Hereditary palmoplantar keratodermas (PPK) are a heterogeneous group of diseases characterized by hyperkeratosis of the palms and soles with thickening of the stratum corneum, usually distinguishable by the mode of inheritance and associated clinical findings. [1],[2]

Clinically, three distinct patterns of palmoplantar keratodermas are seen, as follows: [1]

  1. Diffuse palmoplantar keratodermas, characterized by even, thick, and symmetrical hyperkeratosis over the palms or soles
  2. Focal palmoplantar keratodermas, in which a large, compact mass of keratin develops at the site of recent friction
  3. Punctuate palmoplantar keratodermas, in which multiple, tiny "rain drop" keratoses involve the palmoplantar surface.


Striate palmoplantar keratoderma (Brunauer-Fuhs-Siemens type) is a rare variant of focal PPK characterized clinically by linear and focal hyperkeratosis of the palms and soles. [1] Linear involvement of the dorsum of the foot has not been described.

The condition is of autosomal dominant inheritance. Genetically, the disease is heterogeneous as linkage has been found to two separate loci: To desmogleins (DSG1-3) and the desmocollins (DSC1-3) at chromosome 18q12 and to desmosomal plaque protein and desmoplakin at chromosome 6p21. [3] Thus, striate palmoplantar keratoderma is a genetically heterogeneous disorder of keratinization, which may account for the variable clinical manifestations. [4]

Onset of the disease is in infancy or during the first few years of life. Involvement of the sole is mainly seen, followed by that of palms. In our patient there was involvement of the dorsum of the foot in addition to the lesions over the palm, which has not been reported. The elbows and knees may also be involved. Skin fragility, nail, and hair involvement may be rarely seen. [1] Keratolytics are the mainstay of therapy. Use of topical and oral retinoids has also been described. [4]

 
   References Top

1.
Ketsell DP, Leigh IM. The inherited keratoderma of the palm and soles. In: Wolff K, Goldsmith LA, Katz SI, Gilchrist BA, Paller AS, Leffell DJ, editors Fitzpatrick's Dermatology in General Medicine. 7 th ed. New York: McGraw Hill; 2008. p. 424-31.  Back to cited text no. 1
    
2.
Pai VV, Rao S, Naveen KN. Sybert's keratoderma in three siblings. Indian J Dermatol 2010;55:297-9.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.
Whittock NV, Ashton GH, Dopping-Hepenstal PJ, Gratian MJ, Keane FM, Eady RA, et al. Striate palmoplantar keratoderma resulting from desmoplakin haploinsufficiency. J Invest Dermatol 1999;113:940-6.  Back to cited text no. 3
    
4.
White KL. Striate palmoplantar keratoderma. Dermatol Online J 2002;8:16.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
    Similar in PUBMED
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    What is Your Dia...
   Discussion
    What is Your Dia...
   Answer
    References
    Article Figures

 Article Access Statistics
    Viewed1611    
    Printed26    
    Emailed0    
    PDF Downloaded236    
    Comments [Add]    

Recommend this journal