Pityriasis Rubra Pilaris: A Clinical Review

David Jerard Pincus

Disclosures

Dermatology Nursing. 2005;17(6):448-451. 

In This Article

Clinical Findings

The initial clinical findings of PRP may be redness and scaling of the face and scalp. Skin on the palms and soles may become quite thickened, painful, and orange in color. The patient may experience debilitating hyperkeratosis. The skin becomes progressively redder, and painful fissuring of the palms and soles may occur. While an exfoliative erythroderma may ensue, characteristic "skip" areas of non-involvement are usually seen. Rarely patients may complain of fever, chills, and malaise (Arnold & Buechner, 2004). The most common affected sites are the palmoplantar surfaces, elbows, and knees (75%-80%); dorsa of the hands and feet (60%); and the face (40%) (Vijayalakshmi & Mallika, 2003).

The most characteristic clinical feature is a follicular verrucous papule about 1 mm in diameter with a central keratotic plug. A yellow-orange ring surrounds the lesion (Vijayalakshmi & Mallika, 2003). The second most important diagnostic element is a palmoplantar keratoderma, which is salmon colored and edematous. The Achilles tendon area is frequently involved. As stated previously, there is a cephalic rash. The hair and teeth are not involved but occasionally psoriasiform nail changes may occur. White lacy plaques may be observed on the buccal mucosa (Vijayalakshmi & Mallika, 2003).

The histological changes seen in PRP are consistent but not specific. There is acanthosis with blunting of rete pegs and suprabasal perinuclear vacuolation in isolated cells. Liquefactive degeneration of the basal layer is most likely to be seen overlying the dermal papillae. The granular cell layer is attenuated in some areas and thickened in others. Mild superficial vasodilatation of capillaries with a slight to moderate lymphohistiocytic perivascular infiltrate is found (White, 2003). Examination of a follicular papule is most helpful in the diagnosis. Parakeratosis of the follicular ostea associated with follicular hyperkeratosis is seen. Hypertrophy of the errector pillae is a helpful diagnostic feature when present. A dermal perivascular mononuclear cell infiltrate is present and there are occasional mononuclear cells (see Figures 1 & 2) (Arnold & Buechner, 2004).

There is psoriasiform hyperplasia of the epidermis with alternating areas of orthokeratosis and parakeratosis and plugs of the follicular infundibulum.

A sparse, superficial, perivascular lymphocytic infiltrate is also noted.

The presence of PRP has occasionally been associated with immunodeficient states, such as HIV. In the juvenile forms of this disease, PRP may be associated with T-helper cell impairment, hypogammaglobulinemia, and de creased IgA. Underlying diseases are unusual, but PRP may also be associated with HIV, hypothyroidism, myasthenia gravis, celiac disease, and acute stem cell leukemia.

Patients with pityriasis rubra pilaris can be classified into five types, according to Griffith's Classification (Griffiths, 1975), which differ from each other on the basis of clinical features, age of onset, and prognosis (see Table 1 ).

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