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Pityriasis Rubra Pilaris

Pityriasis Rubra Pilaris, or PRP, describes a group of rare skin disorders that cause widespread reddish-orange colored patches and surface thickening (hardening). Affected areas of skin are commonly itchy, painful, extremely dry and prone to cracking. Depending on the severity of the condition, bordered patches may only cover select areas (over the joints, palms of hands, the soles of feet, head, neck and trunk), or the entire body. Those suffering from Classical Adult Onset PRP (the most common classification) will generally develop a singular patch, or lesion, on their head, neck or upper trunk. After a few weeks, symptoms spread down the body into multiple red, scaly patches. Patches tend to cluster, and sufferers may be able to feel tiny bumps within each cluster, caused by clogged hair follicles. Aside from slightly raised edges on each spot, symptoms may resemble a very large rash, which is why this condition is often initially confused with psoriasis. In less common instances of pityriasis rubra pilaris, symptoms present on the palms and foot-soles, causing skin to look yellow-colored and feel thick and crusty. Severely dry and hardened skin may form cracks, causing hand-usage or walking to be painful. Hand symptoms may also manifest in the nails, creating discoloration, nail bed thickening, and linear black lines.

There is no known cause of PRP, but professionals believe there is a hereditary component. Cases of PRP are classified by the age at which the condition presents, and the persistence of symptoms. Classic Adult Onset PRP accounts for 50% of cases, describing patients who develop symptoms as an adult and typically go into spontaneous remission within 3 years. Atypical Adult Onset PRP (5% of cases) describes patients who first experienced symptoms as an adult, but suffer for 20+ years before symptoms disappear. Classic Juvenile Onset PRP (10% of cases) describes patients who develop symptoms between ages 5 and 10, and experience remission within one year. Circumscribed Juvenile PRP (25% of cases) describes patients who develop symptoms before puberty; these patients may experience a partial remission of symptoms in their late teens, although the symptomatic outcome varies. Atypical Juvenile Onset PRP (5% of cases) describes patients who inherit this condition and experience persistent symptoms from birth. Finally, a small number of cases are associated with HIV. There is no 100% cure for PRP. Treatment is considered successful when it helps to alleviate uncomfortable symptoms and avoid complications. Those who suspect they have symptoms of the condition should consult a medical professional for diagnosis and treatment. Do not attempt to self-diagnose.

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