IADVL
Indexed with PubMed and Science Citation Index (E) 
 
Users online: 7810 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  Navigate here 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 Resource links
   Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
   Article in PDF (115 KB)
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  

 
  In this article
   References
   Article Figures

 Article Access Statistics
    Viewed4109    
    Printed36    
    Emailed1    
    PDF Downloaded106    
    Comments [Add]    

Recommend this journal

 


 
NET LETTER
Year : 2006  |  Volume : 72  |  Issue : 4  |  Page : 326

Localized non-epidermolytic keratoderma


Department of Skin and VD, S.C.B. Medical College and Hospital, Cuttack, Orissa, India

Correspondence Address:
R Chhetia
Department of Skin and VD, S.C.B. Medical College and Hospital, Cuttack, Orissa
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.26719

Rights and Permissions



How to cite this article:
Chhetia R, Jena D K, Dash M L, Ram M K. Localized non-epidermolytic keratoderma. Indian J Dermatol Venereol Leprol 2006;72:326

How to cite this URL:
Chhetia R, Jena D K, Dash M L, Ram M K. Localized non-epidermolytic keratoderma. Indian J Dermatol Venereol Leprol [serial online] 2006 [cited 2020 May 31];72:326. Available from: http://www.ijdvl.com/text.asp?2006/72/4/326/26719


Sir,

Palmoplantar keratoderma is a heterogenous group of disorders, which encompass conditions characterizing thickening of palms and soles. It may be hereditary, acquired, syndromes with PPK as associated feature, diffuse or localized. Focal keratoderma is characterized by lesions located over pressure points. Focal NEPPK[1] or keratosis palmoplantaris varians or nummular PPK, is a hereditary transmitted condition with autosomal dominant inheritance. Clinical features usually appear in childhood and lesions are mainly located over the soles. Keratoderma may increase in severity in early adult life. There have been reports of intrafamilial and interfamilial variability, which accounts for the heterogenous nature of the disease. Palmar involvement is less common than plantar and can be nummular, linear and periungual, while plantar involvement is mostly over pressure points.

An eight year old boy presented with multiple painful, hyperkeratotic lesions over pressure points and bilateral soles. Keratoderma started at 5 years of age and was progressive since then. On examination, localized keratoderma over pressure points, bilateral soles and periungual distribution of great toe, was present [Figure - 1]. On paring, no bleeding points were observed. Lesions at presentation were secondarily infected. Follicular keratotic lesions over extremities were present. Oral cavity, nail and hair were normal.

Similar types of lesions over soles were present in the grandfather of the child. Routine laboratory tests and VDRL were negative. Histopathology findings were suggestive of non-epidermolytic keratoderma [Figure - 2]. The patient was started on topical keratolytics and later on oral isotretinoin therapy. Only mild reduction in intensity of keratoderma was observed.

Focal NEPPK, often mentioned under painful hereditary callosities,[2],[3] is characterized by focal hyperkeratotic lesions, which develop at pressure bearing sites and sites of friction, usually of feet. Palm involvement depends on occupational exposure and is seen more in manual workers.[4] Keratoderma manifests in childhood and increases in severity in early adult life.[1] Focal NEPPK is associated with other clinical features like oral leukokeratosis, follicular lesions and some loss of axillary hair.[4] Linkage and haplotype analysis in NEPPK pedigree, suggested linkage to type I keratin cluster on chromosome 17. Point mutation in K-16 gene highly conserved domain (1A), is reported in two different pedigrees and K-16 mutations in both PC-1 and FNEPPK suggest both diseases to be variants of the same genetic disease.[5],[6]

The other focal keratodermas can be seen in oculocutaneous tyrosinemia, pachyonychia congenita, keratosis palmaris and plantaris with carcinoma of esophagus and focal epidermolytic PPK. Other conditions to be differentiated are viral warts, psoriasis, callosities, syphilis, yaws and leprosy. In our case, painful keratoderma over pressure points, family history and histopathology, confirmed the diagnosis of NEPPK.

The most challenging aspect of keratoderma is its treatment, as the options available provide only symptomatic and short term improvement. The various treatment options available include topical treatment such as keratolytics, paring, topical retinoids and topical (5% 5-Fluorouracil). Systemic treatment includes oral retinoids. Other options available are excision and grafting of hyperkeratotic skin.

In our patient, topical treatment provided little benefit. Oral isotretinoin 0.05-1 mg/kg provided only mild benefit to the patient and was withdrawn due to unbearable side effects. Finally, the patient resorted to topical keratolytics and paring. The challenge posed in the treatment of this inherited condition necessitates the search for more viable and permanent therapeutic options and application of gene therapy to treat the condition.

 
  References Top

1.Lee R, Bowe WP, James WD, Guber PC, Ratnavel R. Keratosis palmaris et plantaris. In: Lebwohl MG, Wells MJ, Heymann WR, Quirk C, James WD. Editors. http://www.emedicine.com/DERM/topic589.htm, Last updated: March 23, 2006, Last accessed: April, 14, 2006.   Back to cited text no. 1    
2.Roth W, Penneys NS, Fawcett N. Hereditary painful callosities. Arch Dermatol 1978;114:591-2.  Back to cited text no. 2  [PUBMED]  
3.Wachters DH, Frensdorf EL, Hausman R, Van Dijk E. Keratosis palmoplantaris nummularis (hereditary painful callosities). J Am Acad Dermatol 1983;9:204-9.  Back to cited text no. 3  [PUBMED]  
4.Griffiths WA, Judge MR, Leigh IM. Disorders of keratinization. In: Rook/ Wilkinson/ Ebling Text book of Dermatology. Champion RH, Burton JL, Burns DA, Breathnach SM, editors. 6th ed. Oxford: Blackwell; 1998. p. 1483.  Back to cited text no. 4    
5.Kelsall DP, Stevens HP, Ratnavel R, Bryant SP, Bishop DT, Leigh IM, et al . Genetic linkage studies in non-epidermolytic palmoplantar keratoderma: Evidence for heterogeneity. Hum Mol Genet 1995;4:1021-5.  Back to cited text no. 5    
6.Shamsher M, Navsaria HA, Stevens HP, Ratnavel RC, Purkis PE, Kelsall DP, et al . Novel mutation in keratin 16 gene underlie focal non-epidermolytic palmoplantar keratoderma in 2 families. J Invest Dermatol 1995;4:1875-81.  Back to cited text no. 6    


    Figures

[Figure - 1], [Figure - 2]



 

Top
Print this article  Email this article
Previous article Next article

    

Online since 15th March '04
Published by Wolters Kluwer - Medknow