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Detailed information of PITYRIASIS RUBRA PILARIS
PITYRIASIS RUBRA PILARIS
DEFINITION:
A chronic cutaneous disorder characterized by small follicular
papules, disseminated scaling patches, and palmoplantar
hyperderatosis.
EPIDEMIOLOGY:
- incidence: ?
- age of onset:
- Type II (first 5 years of life); Type I (51-55 years)
- risk factors:
- familial - autosomal dominant (Type II)
- M = F
PATHOGENESIS:
- unknown etiology and attempts to link this disorder with a
defect in vitamin A metabolism have not been definitive
2. Types
- classic adult form
- makes up 60% of cases
- good prognosis with 80% involuting after 3 years
Type II
- juvenile-onset (3 forms)
- make up 40% of cases
- poorer prognosis for involuting
CLINICAL FEATURES:
- insidious onset with diffuse scaling and erythema of the
scalp
2. Primary Rash
- a firm acuminate, tiny papule, reddish-brown in colour
topped by a central keratotic (horny) plug pierced by a vellus
hair
- masses of these papules coalesce to form large
erythematous sharply demarcated plaques within which islands
of normal skin can be found
- initially found on the sides of the neck, the trunk, and
on the extensor surfaces of the extremities but then extensive
areas become involved which look like exaggerated goose-flesh
and feel like a nutmeg grater
- rash takes on an orange salmon-yellow colour
- may be pruritic and the Koebner phenomenon may be present,
i.e., new onset of lesions at the site of trauma
3. Others
- thick hyperkeratosis of the palms and soles with a
tendency to fissure (may get a sandal-like plantar
hyperkeratosis)
- nails - dull, rough, thickened, brittle, striated, but
pitting is not evident
INVESTIGATIONS:
1. Skin Biopsy
- follicular hyperkeratosis
- inflammatory changes in the dermis
MANAGEMENT:
1. Rash
- topical bland emollients
- isotretinoin (Accutane) in Type I
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Pediatric Database - PITYRIASIS RUBRA PILARIS
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