pityriasis rubra pilaris (prp). etiology the etiology is unknown a familial form of the disease...
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PITYRIASIS RUBRA PILARIS
(PRP)
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Etiology
• The etiology is unknown• A familial form of the disease exists, with
an autosomal dominant inheritance pattern; however, most cases are sporadic
• One hypothesis is that pityriasis RPR may be related to an abnormal immune response to an antigenic trigger
• Case reports have described PRP occurring after streptococcal infections
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• The incidence has been reported to be 1 case in 3500-5000 patients presenting to dermatologic clinics
• PRP occurs equally among men and women
• The familial form typically begins in early childhood
• The acquired form has peaks in the first and fifth decades of life, but it can begin at any age
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Clinically• The familial form of PRP has a gradual
onset, whereas the acquired form has an acute onset
• The disease
typically spreads in
a craniocaudal
(from head to body)
direction
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• PRP is characterized by orange-red or salmon-colored scaly plaques with sharp borders, which may expand to involve the entire body
• A characteristic feature is presence
of islands of unaffected skin within
the plaques
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• The plaques are formed of follicular hyperkeratotic papules
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• Palmoplantar keratoderma occurs in most patients and tends to have an orange hue
• Painful fissures may develop in patients with palmoplantar keratoderma
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• Nail changes include distal yellow-brown discoloration, subungual hyperkeratosis, longitudinal ridging, nail plate thickening, and splinter hemorrhages
• Mucous membranes: Patients may complain of pain and irritation in the mouth. Mucous membrane changes include a diffuse whitish appearance of the
buccal mucosa, lacy whitish plaques, grayish-white papules and plaques, erythema, or possible erosions
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• Pruritus, although not a major symptom, may occur in the early stages of the disease
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Griffiths classificationTypeC/PDistrib.Course
I-Classic adult (>50 of cases)
As mentioned before
Gene-ralized
Often resolves within an
average of 3 y
II-Atypical adult (5% of cases)
Follicular hyper-keratosis and ichthyosiform lesions on the legs, sparse
scalp hair
Gene-ralized
Long duration (> 20 y)
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TypeC/PDistrib.Course
III-Classic juvenile (10 of cases)
Similar to type I but appears in
year 1 or 2 of life
General-ized
Often resolves within an
average of 1-2 y
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TypeC/PDistrib.Course
IV- Circu-mscribed juvenile
(25% of cases)
Prepubertal children; well-
demarcated scaly, erythematous plaques on the
elbows and knees, resembling
localized psoriasis
Locali-zed
Some cases
clear in the late teens
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TypeC/PDistrib.Course
V- Atypical juvenile
(5% of cases)
Begins in first few years, accounts for most familial cases;
follicular hyperkeratosis, scleroderma-like
appearance of the hands and feet
Gener-alized
Chronic course
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Histopathology
• Folliculat Plugging:
The follicles are filled with dense, horny (keratinous)plugs
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• There are foci
of parakeratosis
in the perifo-
llicular shoulder
and in the epi-
dermis between
the follicles• At other sites, a basket-weave hyper-keratosis overlies
a prominent granular layer and there is little parakeratosis
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•Although the epidermis is acanthotic, it is not, as in psoriasis, thinned above the dermal papillae, and there is no tendency for polymorphs to invade the epidermis
•There is a dermal
infiltrate of
lymphocytes
and histiocytes
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Treatment• Currently, oral retinoids are the first line of
therapy. Isotretinoin has been reported to be of value, although a comprehensive review suggests that acitretin (0.5 to 0.75 mg/kg per day) may be more effective in clearing lesions. Accordingly, most patients are treated first with acitretin today
• Therapy with methotrexate(10 to 25 mg weekly, intramuscularly or orally, in divided doses, once a week) has shown variable rates of success
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• Some cases respond well to photochemotherapy, some may flare, and others require combination treatment with retinoids or methotrexate
• Several cases of adult-type PRP showed significant clearance in 2 to 4 weeks with cyclosporine(5 mg/kg/day) Although most studies show lack of efficacy
• Some patients are helped by azathioprine (100 to 150 mg/day) , but this effect is also inconsistent