PRPSG Facebook Post: Standard Therapies


PRP tends to follow a natural waxing and waning course, with episodes in which there is periodic worsening (exacerbation) or cessation (remission) of symptoms. As a result, according to many researchers, it may be difficult to evaluate the effectiveness of particular therapies. (1)

The value of treatment is difficult to assess, as the clinical course is so variable for each of the different types of PRP. Patients with classical adult onset PRP, for example, may present with intense and widespread reddening of the skin (erythroderma). Hospital admission for skin care, fluid replacement and other supportive care may be warranted. (2)

From the patient perspective, there are two major objectives in the treatment of pityriasis rubra pilaris:

❏½ relieving symptoms as they present
❏½ achieving long-term remission, if possible

The mantra heard within the PRP community is simple but deafening: “What works for one doesn’t work for all.”

TREATMENT OPTIONS

Management of PRP often involves systemic and topical therapies combined. Topical therapies can help with the symptoms and may be enough for people with mild PRP. Topical treatments are usually combined with systemic therapy for PRP that affects a large part of the body. Most people need systemic therapy to control the condition. (1)

Treatment options will vary based on age, geography and cost to the patient. Moreover, laboratory tests are important to monitor the effects of medications on the body – especially the liver – and to manage and monitor the side effects of drugs.

Some of the medications used to treat PRP can harm a developing fetus and are not recommended for use right before or during pregnancy (1)

People seeking information about specific treatment options for themselves or family members should speak with their health care provider. (1)

Oral retinoids are derivatives of vitamin A that slow the growth and shedding of skin cells. Treatment options include acitretin / Soriatane® and isotretinoin / Accutane®

Oral retinoids (synthetic vitamin A derivatives) are usually preferred as a first-line systemic treatment for PRP. (1)

Immunosuppressants to slow down the body’s immune system. Often used when oral retinoids are ineffective. Treatment options include (oral and injection) include methotrexate and cyclosporine.

Biologicals, with generally fewer side effects, are targeted to reduce inflammation. These are injectable or intravenous (IV) medications that affect the immune system. Treatment options include adalimumab / Humira®, etanercept / Enbrel®, infliximab / Remicade®, ustekinumab / Stelara®, secukinumab / Cosentyx® and apremilast / Otezla®

Other therapies
❏½ Ultraviolet light therapy. This is normally given in combination with psoralen (a drug that makes you less sensitive to the sun) and a retinoid. (3)
❏½ Topical creams that contain urea or lactic acid. These go directly on your skin. (3)
❏½ Oral vitamin A. This may be helpful in some people, but only in very high doses. Retinoids are more effective and more commonly used than vitamin A. (3)
❏½ Traditional Chinese Medicine and other Alternative Medicines with varying degrees of success.

REFERRALS

Depending on the severity, duration and array of signs and symptoms, PRP patients seek the expertise of specialized healthcare professionals:

❏½ Opthamologist – Ectropion and impaired vision
❏½ Podiatrist – Impaired mobility
❏½ Otorhinolaryngologist (ENT specialist) – impaired hearing, removal of cerumen from ear canal.
❏½ Hepatologist – monitor impact of PRP treatment on the liver.
❏½ Psychiatrists/Clinical Psychologist – depression and mental wellness
❏½ PRP patient support resources – (SEE Resources)

(1) Genetic and Rare Diseases Information Center; https://rarediseases.info.nih.gov/diseases/7401/pityriasis-rubra-pilaris#ref_7262 (2017)

(2) DermNet New Zealand; https://www.dermnetnz.org/topics/pityriasis-rubra-pilaris (2017)

(3) Healthline.com; http://www.healthline.com/health/pityriasis-rubra-pilaris#treatment8 (2017)

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2017 NORD PRP Report Revision

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