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   Abstract
   Introduction
   Case Report
   Discussion
   References
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CASE REPORT
Year : 1995  |  Volume : 61  |  Issue : 2  |  Page : 116-117

Progressive symmetric erythokeratoderma


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Correspondence Address:
A Ghorpade


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PMID: 20952910

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  Abstract 

Progressive symmetric erythrokeratoderma (PSEK, Gottron's Syndrome) in a 10 year-old-boy is reported. The diagnosis was confirmed by histopathology.


Keywords: Erythrokeratoderma, Symmetric progressive erythrokeratoderma, Gottron′s syndrome


How to cite this article:
Ghorpade A, Ramanan C. Progressive symmetric erythokeratoderma. Indian J Dermatol Venereol Leprol 1995;61:116-7

How to cite this URL:
Ghorpade A, Ramanan C. Progressive symmetric erythokeratoderma. Indian J Dermatol Venereol Leprol [serial online] 1995 [cited 2014 Jul 29];61:116-7. Available from: http://www.ijdvl.com/text.asp?1995/61/2/116/4163



  Introduction Top


Gottron's syndrome is a rare cornification disorder inherited as an autosomal dominant trait with variable penetrance and characterized by rapid epidermal cell proliferation.[1] There are scanty reports of this condition in Indian literature.


  Case Report Top


A 10 year-old-boy presented to us with history of skin lesions on both the knees, elbows, hands and feet. The asymptomatic lesions had started about 5 year ago and had gradually increased in size initially for one year, after which they became stable. There was no family history of similar lesions and no seasonal variation. Cutaneous examination revealed symmetrical, well defined, erythematous, raised plaques with fine scales on both the knees and dorsa of hands [Figure - 1]. There were similar lesions on both the upper arms, extensor aspect of elbows, and lateral malleoli. There was no palmoplantar keratoderma, and no lesions on trunk. The scalp, nails and mucous membranes were free. The systemic examination was normal. Histopathology from the plaque over knee revealed hyperkeratosis, focal parakeratosis, an intact granular layer, acanthosis [Figure - 2] and mild chronic inflammatory infiltate in upper dermis around blood vessels. Some of the prickle cells had perinuclear vacuolation. The patient improved with topical keratolytic ointment but the lesions recurred after discontinuing treatment.


  Discussion Top


In PSEK, the symmetric, finely scaly, erythematous plaques start in early childhood and are stable after initial progression. The lesions commonly involve shoulder girdle, buttocks and face, ankles and wrists. Palmoplantar keratoderma may be seen in half the cases and in some, the lesions regress after puberty. Both the sexes are affected equally and the general health is good.[2] Our clinical impression was confirmed by the classical histopathological features in our patient.

Erythrokeratoderma variabilis (EKV) which may resemble PSEK differs from the latter, as the lesions of EKV continuously changes and may be induced by external mechanical pressure and temperature changes. The lesions may improve in summer and aggravate during pregnancy.[4] In addition, the lesions in EKV tend to involve abdomen and thorax also, unlike PSEK, EKV may be associated with deafness, physical retardation and peripheral neuropathy.[5] Localised pityriasis rubra pilaris differs from PSEK, in that the former shows follicular change as follicular erythema and keratosis.[2] The treatment is mainly symptomatic. However, oral retinoids have also been found effective.[2].[5]

 
  References Top

1.Hopsu-Havu VK, Tuohimata P. Erythrokeratodermia congenitalis progressive symmetrica (Gottron): An analysis of kinetics of epidermal cell proliferation. Dermatologica 1971;142:137-44.  Back to cited text no. 1    
2.Nazzaro V, Bardon CB. Progressive symmetric erythrokeratodermia: Histological and ultrastructural study of patients before and after treatment with etretinate. Arch Dermatol 1986;122:434-40.  Back to cited text no. 2    
3.Gewritzman GB, Winkler NW, Dobson RL. Erythrokeratodermia variabilis. A family study. Arch Dermatol 1978;14:259-61.  Back to cited text no. 3    
4.Beare JM, Nevin NC, Frogatt P, et al. Atypical erythrokeratodermia with deafness, physical retardation and peripheral neuropathy. Br J Dermatol 1972;87:308-14.  Back to cited text no. 4    
5.Tàmoyo L, Ruiz-Maldonado R. Oral retinoid (RO 10-9359) in children with lamellar ichthyosis, epidermal hyperkeratosis and symmetrical progressive erythrokeratodermia. Dermatologica 1980;161:305-14.  Back to cited text no. 5    


    Figures

[Figure - 1], [Figure - 2]



 

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