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Image Number #8623 (Papillon Lefevre syndrome)

Diagnosis: Papillon Lefevre syndrome

Description:

Red scaly rash

Morphology: Red,scaly

Site: Hand,dorsum

Sex: M

Age: 20

Type: Clinical

Submitted By: Ian McColl

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Differential Diagnosis
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History:

Patient of Dr

edentulous   young man presented   with   skin lesions as

shown

not itchy

no   history of psoriasis   in the family

has been there for years

Look up erythrokeratoderma variabilis because I think that is the diagnosis. Histologically it is like psoriasis but inherited as an autosomal dominant. May clear with oral retinoids but can relapse.

(Submitted By: Dr Ian McColl)

its a   diagnosed   case   of Papillon-Lefevre syndrome (autosomal recessive) (Submitted By: Dr Ranjith Senanayake)

Should have concentrated on the fact he was edentulous with palmoplantar keratoderma. Mind you bad caries and loss of teeth is also a feature of erythrokeratoderma! (Submitted By: Dr Ian McColl)
 

DermNetNZ   eMedicine   PubMed   Dermatology Online   Archives   JAAD for "Papillon Lefevre syndrome"

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