Signs and Symptoms of PRP
From the Editor…
How does pityriasis rubra pilaris present? What are the “signs and symptoms” available to a dermatologist that signal the possibility of a pityriasis rubra pilaris diagnosis. What are the clues dermatologists seek? Here is what a search of the internet will find.
PRP causes pink, red, or orange-red scaly patches on your skin. The patches are usually itchy. You may have the scaly patches only on some parts of your body. They most often occur on the elbows, knees, hands, feet, and ankles. The skin on the palms of your hands and the soles of your feet may also become red and thickened. The scaly patches may eventually spread over the entire body.
Pityriasis rubra pilaris is initially characterized by skin lesions described as, sharply pointed, horn-like, brownish-red to rosy yellow- colored papules. These papules usually occur on the back of the wrists, the outside of the forearms, underarm folds, elbows, knees, backs of the hands, and fingers. When the papules grow and connect together they produce dry, scaly, rough, red plaques over large areas of the skin. Gray, brittle nails and excessive oiliness of the glands on the scalp (seborrhea) and face may also occur. Often the edges of the eyelids are turned outward (ectropion).
Classical adult-onset PRP most often starts on the head, neck and upper trunk as a red scaly rash. Often there is a solitary lesion but within a few weeks multiple patches appear and they join together to form groups of reddish-orange lesions. Over a few weeks these spread downwards and may cover most of the body (erythroderma).
Rough, dry plugs can be felt within the patches and are due to plugged hair follicles, often most obvious on the backs of the fingers. Patients may also complain of itching in the early stages of the disease.
The palms and soles become thickened and yellow coloured in PRP. Cracks may develop which can be painful and make walking and using the hands difficult.
The nails may become thickened and discoloured at the free nail edge and may show linear black streaks (splinter haemorrhages).
The hair may thin considerably.
PRP in childhood can be classical (generalised) or circumscribed (limited in extent). The appearance is similar to in adults, with prominent thickening of the skin of the palms and soles. Other affected areas can be orange-red in colour with follicular prominence.
Features of this condition vary greatly between affected individuals. The onset is gradual in the familial type and can be more rapid in the acquired type. Redness and scaling of the face and scalp are often seen first, followed by redness and thickening of the palms and soles. Overall, the elbows, knees, backs of the hands and feet, and ankles are most commonly affected. A more widespread eruption consisting of scaling orange-red plaques can be observed on the trunk and extremities. The lesions may expand and coalesce and eventually cover the entire body. When the disease becomes widespread, the nails, mucous membranes and eyes may be affected. The familial type often persists throughout life, but the acquired form may have periods of remission (periods of time where symptoms improve or completely resolve).
The rash can be itchy in its early stages. Thick skin on the palms and soles can split and become painful. Walking may be sore. Shivering, heat and fluid loss may occur if the rash covers large areas of skin.
Orange-red scaly patches and thick skin develop on the hands and feet.
The scaly areas cover much of the body. Small islands of normal skin (islands of sparing) are seen within the areas of the scaly skin. The scaly areas may be itchy. There may be changes in the nails.