Pityriasis Rubra Pilaris Clinical Presentation

Updated: Sep 11, 2020
  • Author: Philip D Shenefelt, MD, MS; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

The familial form of pityriasis rubra pilaris has a gradual onset, whereas the acquired form has an acute onset. Various triggers have been described, including vaccination. [15]  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 (see the images below). The lesions may expand and coalesce to cover the entire body.

Plantar keratoderma with an orange hue on the sole Plantar keratoderma with an orange hue on the soles.
Palmar keratoderma with an orange hue on the palms Palmar keratoderma with an orange hue on the palms.
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Physical Examination

Physical findings can be grouped by skin, nails, mucous membranes, and eyes.

Skin

Pityriasis rubra pilaris is characterized by orange-red or salmon-colored scaly plaques with sharp borders, which may expand to involve the entire body (see the images below). 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 (see the images below). This pattern may be referred to as nutmeg grater papules. 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.

Reddish orange plaques on the trunk. Reddish orange plaques on the trunk.
Follicular hyperkeratosis seen on the dorsal aspec Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.

Nails

Nail changes include distal yellow-brown discoloration, subungual hyperkeratosis, longitudinal ridging, nail plate thickening, and splinter hemorrhages. Nail pitting is not typical.

Mucous membranes

Patients may complain of pain and irritation in the mouth. Mucous membrane changes include a diffuse whitish appearance of the buccal mucosa, lacy whitish plaques, grayish-white papules and plaques, erythema, or possible erosions. [16]

Eyes

Patients with extensive disease may develop ectropion. Patients have also reported blurred vision and dryness.

Griffiths classification

Type I is classic adult pityriasis rubra pilaris. This is the most common form of pityriasis rubra pilaris, accounting for more than 50% of all cases of pityriasis rubra pilaris. Onset is acute, and the features are classic, including erythroderma with islands of sparing, palmoplantar keratoderma, and follicular hyperkeratosis. This type of pityriasis rubra pilaris has the best prognosis. Reportedly, about 80% of patients have remission in an average of 3 years. One reported case resolved spontaneously after 20 years. [17] Scarring alopecia has been reported in this setting. [18]

Type II is atypical adult pityriasis rubra pilaris. This form accounts for about 5% of all cases of pityriasis rubra pilaris. It is characterized by ichthyosiform lesions, areas of eczematous change, alopecia, and long duration (often 20 y or more).

Type III is classic juvenile pityriasis rubra pilaris. This form accounts for about 10% of all cases of pityriasis rubra pilaris. It is very similar to type I; however, its onset is within the first 2 years of life. Remission can occur sooner than with type I, within an average of 1 year.

Type IV is circumscribed juvenile pityriasis rubra pilaris. This form accounts for about 25% of all cases of pityriasis rubra pilaris. It occurs in prepubertal children and is characterized by sharply demarcated areas of follicular hyperkeratosis and erythema of the knees and the elbows. The long-term outcome is unclear, with some reports of improvement in the late teenaged years. This form of pityriasis rubra pilaris rarely progresses.

Type V is atypical juvenile pityriasis rubra pilaris. This form accounts for about 5% of all cases of pityriasis rubra pilaris. Most cases of familial pityriasis rubra pilaris belong to this group. It has an early onset and runs a chronic course. It is characterized by prominent follicular hyperkeratosis, sclerodermalike changes on the palms and the soles, and infrequent erythema.

Type VI is HIV-associated pityriasis rubra pilaris. Patients with HIV may have nodulocystic and pustular acneiform lesions. Elongated follicular plugs or lichen spinulosus–type lesions have also been reported to be present. Patients' conditions tend to be resistant to standard treatments, but they may respond to antiretroviral therapies.

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Complications

Pityriasis rubra pilaris 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 pityriasis rubra pilaris. It is characterized by generalized erythema and scales, hair loss, and onycholysis. Systemic symptoms include malaise, fatigue, anorexia, fever, and chills. Patients with erythroderma may develop lymphadenopathy, hepatomegaly, splenomegaly, and electrolyte abnormalities due to increased transepidermal water loss. Cardiac failure may occur in patients with preexisting heart conditions.

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