Indian Dermatology Online Journal

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

SkIndia Quiz 17: Linear dermatoses over the hand and foot


Varadraj V Pai, Pankaj Shukla, Rakhi Godhge, Prachi Bhandhare 
 Department of Dermatology, Goa Medical College, Bambolim, Goa, India

Correspondence Address:
Dr. Varadraj V Pai
Department of Dermatology, Goa Medical College, Bambolim - 430 002, Goa
India




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;6:134-135


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 2021 Jun 25 ];6:134-135
Available from: https://www.idoj.in/text.asp?2015/6/2/134/148972


Full Text

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}{Figure 2}{Figure 3}

 What is Your Diagnosis?



 Answer



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

 DISCUSSION



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]

Diffuse palmoplantar keratodermas, characterized by even, thick, and symmetrical hyperkeratosis over the palms or solesFocal palmoplantar keratodermas, in which a large, compact mass of keratin develops at the site of recent frictionPunctuate 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

1Ketsell 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.
2Pai VV, Rao S, Naveen KN. Sybert's keratoderma in three siblings. Indian J Dermatol 2010;55:297-9.
3Whittock 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.
4White KL. Striate palmoplantar keratoderma. Dermatol Online J 2002;8:16.