Pityriasis rubra Pilar

Pityriasis rubra Pilar Pityriasis rubra papuloscuamoasa Pilar is a chronic disease of unknown etiology characterized by scaly red-orange plates, keratodermie palmoplantara papules and follicular keratosis. The disease may progress to erythroderma with distinct areas of skin unaffected. The disease is divided into five types: classic adult, adult atypical, classic juvenile delinquency and atypical juvenile circumscribed. More recently, the type associated with HIV was added to this classification. Pityriasis rubra pilaris patients may be painful and debilitating keratodermie palmoplantara. Nail dystrophy and their rupture may be present. However, morbidity associated with pityriasis rubra pilaris is related eritodermie.
Topical corticosteroids may improve the condition but not a long term effect. Calcipotriol is an analogue of vitamin D that is used in topical regimens for psoriasis, with beneficial effects for pityriasis rubra Pilar. Topical retinoids are also used, emolientele, extracorporeal fotochimioterapia. Pityriasis rubra pilaris Each type has its evolution foe. In general, familial form of the disease may be persistent throughout life and gained form can resolve spontaneously within 1-3 years.
Pathogenesis and causes
The etiology is unknown. There is a familial form of the disease with autosomal dominant inheritance, though most cases are sporadic. One hypothesis is that as pityriasis rubra Pilar which is related to an abnormal immune response to an antigenic trigger. There were reported cases after streptococcal infections.
Signs and symptoms
Familial form of pityriasis rubra Pilar begins in childhood and has an autosomal dominant inheritance. The form of pityriasis rubra Pilar won by the age distribution is bimodal with peaks in the first and fifth decade of life, but can occur at any age. Onset familial form has gradually gained while one acute form. The disease typically extends in craniocaudala directives. Patients notice first the redness and scaling on the face and scalp. These are followed by redness and thickening of palms and plants. Lesions can expand and merge to cover the entire body.
Physical Exam Pilar pityriasis rubra is characterized by orange-red plates with scales, with clearly defined edges that can be extended to cover the entire body. Frequently unaffected skin areas called islands are present. Follicular hyperkeratosis is seen on the back of the proximal phalanges, wrists and ankles. Keratodermia palmoplantara occurs in all patients and tends to be orange. It can develop painful cracks. Itching although there is a major symptom may occur in the early stages of the disease. Nail disorders include distal yellow discoloration, hyperkeratosis subunghiala, longitudinal thickening, bleeding subunghiale. Patients may experience pain and mouth irritation. White matter changes include diffuse mucous membranes of the mouth, white boards, boards and gray-white papules, erythema and erosions. Patients with extensive disease may ectropion. They accuse blurred vision and dry eye.
Griffiths Classification:
Classic adult-type I is the most common form of pityriasis rubra Pilar, numbers more than 50% of cases, acute onset, CALS elements, shows the best prognosis, remission in more than 80% in 3 years Type II atypical adult-count 5% of all cases of pityriasis rubra Pilar, characterized by lesions ihtiosiforme, eczematizare areas, alopecia and long evolution -Type III classic juvenile counts 10% of cases, is similar to type I still have a debut in the first 2 years of life, remission may occur earlier compared to type I, In a year on average Circuscris juvenile-type IV, count 25% of cases occur in prepubertal children and is characterized by well-demarcated areas of follicular hyperkeratosis and erythema of calcium and ankles, the prognosis is unclear, this form rarely progresses Atypical juvenile-type V-count 5% of cases, most cases of pityriasis rubra Pilar family belongs to this group, has an early onset and a chronic evolution, characterized by hyperkeratosis Pilar, sclerodermalike lesions on the palms and soles, erythema infrequent VI-type HIV-associated pityriasis rubra Pilar, nodular acne patients have similar lesions, follicular plugs, lichen spinulosum similar lesions, patients tend to be resistant to standard treatment regimes but may respond to antiretroviral drugs.
Complications Painful and debilitating-keratodermia palmoplantara Nail-dystrophy -Eritodermia-generalized erythema, scaling, hair loss, onycholysis, malaise, fatigue, anorexia, fever and chills, patients develop limadenopatie, hepatomegaly, splenomegaly and abnormal transepidermal water loss by increasing alectrolitice, heart failure can occur in patients with heart disease Background.
Diagnosis The differential diagnosis is made with the following conditions: cutaneous T cells lymphoma, erythroderma, eritrokeratodermia varaibilis boards of psoriasis.
Treatment
Corticosterizii topics may be a degree of improvement, but are not beneficial to long term. Calcipotriol is a vitamin D analogue used in the topical treatment of psoriasis, but also with good results for pityriasis rubra pilaris. Tazarotene topical retinoids showed beneficial effects in circumscribed juvenile form. Emoleintele are used to reduce skin dryness and cracking. Fotochimioterapia involves extracorporeal exposure of peripheral blood ex vivo mononuclear cells from ultraviolet Leukapheresis in the presence of a DNA intercalation agent and treated cells re-infused. They are also using methotrexate, azathioprine, cyclosporine, etanercept, infliximab, vitamin A. Each type of pityriasis rubra Pilar has its own evolution. In general, familial form of the disease can be persistent for life and gained form can resolve spontaneously within 1-3 years.