Pityriasis Rubra Pilaris

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Pityriasis Rubra Pilaris Ϣ Σήϟ΍ ϦϤΣήϟ΍ Ϳ΍ ϢδΑ 1 Papulosquamous Dermatoses Rania AlSaied MD 2 Pityriasis Rubra Pilaris 3 It is a chronic papulosquamous disorder of unknown etiology characterized by reddish orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. The disease may progress to erythroderma with distinct areas of uninvolved skin, the so-called islands of sparing. 4 Griffiths divided PRP into 5 categories: classic adult type, atypical adult type, classic juvenile type, circumscribed juvenile type, and atypical juvenile type. More recently, an HIV-associated type has been added to this classification system. 5 Pathophysiology 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 PRP may be related to an abnormal immune response to an antigenic trigger. Case reports have described PRP occurring after streptococcal infections. 6 Mortality/Morbidity Patients with PRP can have painful and disabling palmoplantar keratoderma. Nail dystrophy and shedding may be present. However, most of the morbidity associated with PRP is associated with the erythroderma 7 Sex , Age PRP occurs equally among men and women The familial form of PRP typically begins in early childhood and has an autosomal dominant inheritance pattern. The acquired form of PRP has a bimodal age distribution, with peaks in the first and fifth decades of life, but it can begin at any age. 8 History The familial form of PRP has a gradual onset, whereas the acquired form has an acute onset. The disease typically spreads in a craniocaudal direction. Patients first notice redness and scales on the face and the scalp. This is often followed by redness and thickening of the palms and the soles The lesions may expand and coalesce to cover the entire body. 9 Physical Skin PRP is characterized by orange-red or salmon-colored scaly plaques with sharp borders, which may expand to involve the entire body Often, areas of uninvolved skin, referred to as islands of sparing, are present. Follicular hyperkeratosis is commonly seen on the dorsal aspects of the proximal phalanges, the elbows, and the wrists 10 11 12 Palmoplantar keratoderma occurs in most patients and tends to have an orange hue. Painful fissures may develop in patients with palmoplantar keratoderma. Pruritus, although not a major symptom, may occur in the early stages of the disease. 13 Nails Nail changes include distal yellow- brown discoloration, subungual hyperkeratosis, longitudinal ridging, nail plate thickening, and splinter hemorrhages. Nail pitting is not typical. 14 Histologic Findings Histologic features are not pathognomonic, but they are useful to rule out other possible papulosquamous and erythrodermic disorders. Features on light microscopy include hyperkeratosis with alternating orthokeratosis and parakeratosis forming a checkerboard pattern in the stratum corneum, focal or confluent hypergranulosis, follicular plugging with perifollicular parakeratosis forming a shoulder effect, thick suprapapillary plates, broad rete ridges, narrow dermal papillae, and sparse superficial dermal lymphocytic perivascular infiltration 15 Medical Care Topical medications Topical corticosteroids may provide some patient comfort, but they are believed to have little long- term therapeutic effect. Emollients reduce fissuring and dryness, providing some patient comfort. Petroleum jelly or one of the many proprietary emollients may be used. 16 Systemic therapy More severe cases may require: Systemic retinoids Immunosuppressants like Azathioprine Methotrexate Cyclosporin 17 Complications PRP can cause painful and disabling palmoplantar keratoderma. Nail dystrophy and shedding may occur. Erythroderma is a reaction pattern of the skin that can occur in the setting of several different skin disorders, most commonly including psoriasis, eczema, lymphoma, drug reactions, and PRP. It is characterized by generalized erythema and scales, hair loss, and onycholysis. 18 Prognosis Each type of PRP has its own prognosis. In general, the familial form of the disease may be persistent throughout life, and the acquired form of the disease may resolve spontaneously within 1-3 years. 19 Pityriasis Rosea 20 Background Pityriasis rosea (PR) is a common benign papulosquamous disease . Pityriasis denotes fine scales, and rosea translates as rose colored or pink. PR can have a number of clinical variations. Its diagnosis is important because it may resemble secondary syphilis. 21 Pathophysiology PR has often been considered to be a viral exanthem. Its clinical presentation supports this concept. PR has been linked to upper respiratory infections, it can cluster within families and close contacts, and it has an increased incidence in individuals who are immunocompromised. As with viral exanthems, the incidence may increase in the fall and the spring. 22 recent work demonstrated human herpesvirus (HHV)ƛ7 viral DNA in both the lesions and the plasma in patients with PR. 23 Sex PR is more common in women than in men. One study found it to be twice as common in women as in men. Age PR commonly develops in children and young adults, although any age group can be affected. Most patients are aged 10-35 years. 24 History The history should include questions about close contacts with similar eruptions. This finding is uncommon because most cases of PR are sporadic, as PR is thought to reflect a weakly contagious disease. A history of medication intake should be obtained because several medications have been shown to cause a similar exanthem. 25 The disease typically begins with a solitary macule that heralds the eruption (called the herald spot/patch), which is usually a salmon-colored macule. This initial lesion enlarges over a few days to become a patch with a collarette of fine scale just inside the well-demarcated border. 26 Within the next 1-2 weeks, a generalized exanthem usually appears, although it may occur from hours to months after the herald patch. This secondary phase consists of bilateral and symmetric macules with a collarette scale oriented with their long axes along cleavage lines. This phase tends to resolve over the next 6 weeks, but variability is common. Pruritus is common, usually of mild-to-moderate severity, and it occurs in 75% of patients. 27 Physical The herald patch is usually a single pink patch, 2-10 cm in diameter, on the neck or the trunk with a fine collarette scale. It is observed in more than 50% of patients, and it may occur as multiple lesions or in atypical locations. 28 About 1-2 weeks after the herald patch is seen, the generalized eruption appears, although it has been known to occur from hours to 3 months later. It consists of salmon-colored macules or patches, 0.5- 1.5 cm in diameter, with a collarette scale, often described as having a cigarette paperƛlike appearance. The long axes of the lesions are oriented in a parallel fashion along cleavage lines, giving the classic Christmas tree pattern. These secondary lesions most commonly occur on the trunk, the abdomen, the back, and the proximal upper extremities. 29 30 31 D.D Lichen Planus Nummular Dermatitis Psoriasis, Guttate Seborrheic Dermatitis Syphilis Tinea Corporis Drug induced PR like eruptions: captopril, levamisole, omeprazole, Barbiturates, aspirin, ketokonazole 32 Lab studies One must be careful to rule out syphilis. A screening rapid plasma reagin (RPR) test or a VDRL test should be ordered for appropriate individuals. 33 Histopathology It shows superficial perivascular dermatitis Focal parakeratosis in mounds, hyperplasia, and focal spongiosis are observed in the epidermis. The epidermis may show exocytosis of lymphocytes, variable spongiosis, mild acanthosis, and a thinned granular layer. In the dermis, extravasated red blood cells are a helpful finding along with a perivascular infiltrate of lymphocytes, histiocytes, and eosinophils. A number of monocytes are also commonly present. 34 35 Treatment Emollients Antihistamines ( if there is pruritus) UVB phototherapy ( for persistent PR) 36 Complications The main morbidity is from pigmentary changes, which are possible with the healing lesions. Both postinflammatory hyperpigmentation and hypopigmentation may occur. 37 Prognosis The prognosis for PR is excellent. Patients may return to work or school because they are not considered to be contagious. 38 Psoriasis 39 Objectives Identify the pathogenic factors for development of psoriasis List the clinical features of psoriasis Describe the progressive management of the clinical features of psoriasis List the adverse effects of psoriatic treatments 40 Psoriasis Chronic skin disorder; "itch" = psora Incidence Other derm conditions 41 Psoriasis T-cell mediated inflammatory dz Epidermal hyperproliferation 2O to activation of immune system Altered maturation of skin Inflammation Vascular changes 42 43 44 Background Epidemiology Age Genetic Scandinavian/European descent Risk Factors 45 Psoriasis, an inherited disease If you have psoriasis, what is the risk to: Your unrelated neighbor? About 2% Your sibling? 15-20% Your identical twin? 65-70% Your child? 25% 46 N P Disorganized O STRATUM S R CORNEUM O Neutrophil M STRATUM R accumulation GRANULOSUM A I L STRATUM A SPINOSUM S Immaturity I S Proliferation STRATUM BASALE DERMIS 47 48 Psoriasis: Associated Factors Genetic Factors: - 30% of people with psoriasis have had psoriasis in family - Autosomal dominant inheritance
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