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Psoriasis: Evolving treatment for a complex disease

Cleveland Clinic Journal of Medicine. 2012 June;79(6):413-423 | 10.3949/ccjm.79a.11133
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ABSTRACTThe cutaneous manifestations of psoriasis can vary in morphology and severity, and therapy should be tailored accordingly. Biologic agents are important new options for treating patients with the most severe forms of the disease. All physicians should be aware that severe psoriasis may increase cardiovascular morbidity and the risk of death, and preventive strategies for patients with severe disease should be considered.

KEY POINTS

  • Studies in the past 10 years have uncovered a link between psoriasis, metabolic syndrome, and cardiovascular disease. Interestingly, the risk grows less with age; patients at greatest risk are young men with severe psoriasis.
  • The most common presentation of psoriasis is plaque psoriasis. However, there are several other clinical variations of psoriasis, each of which has a distinct response to treatment and may be associated with significant systemic symptoms.
  • Tumor necrosis factor inhibitors should be considered first-line in the treatment of psoriatic arthritis.
  • Phototherapy and systemic medications including methotrexate, acitretin (Soriatane), cyclosporine (Gengraf, Neoral, Sandimmune), and biologic agents are the most effective treatments for moderate-to-severe psoriasis.

PSORIASIS HAS SEVERAL CLINICAL PHENOTYPES

Psoriasis has several clinical variants, each with a distinct clinical course and response to treatment.27 Moreover, many patients present with more than one variant.

Plaque psoriasis

Figure 1. Well-demarcated erythematous, scaly plaques characteristic of plaque psoriasis on the elbow.
Plaque psoriasis (Figure 1) accounts for more than 80% of cases. It is characterized by well-demarcated, scaly, pink-to-red plaques of various sizes with a relatively symmetric distribution. Involvement of the extensor surfaces such as the elbows and knees and of the scalp, trunk, and intergluteal cleft is common.

Plaques can persist for several months to years, even in the same location, and only about 5% of patients report complete remission for up to 5 years.

Inverse psoriasis

Photo courtesy of Joseph C. English III, MD.
Figure 2. Patient with inverse psoriasis of the axilla.
Involvement of the skin folds, including the axillary, genital, perineal, intergluteal, and inframammary regions with pink-to-red plaques with minimal scale is the main clinical feature of inverse psoriasis (Figure 2). Absence of satellite pustules clinically distinguishes it from candidiasis.

Guttate psoriasis

Photo courtesy of Laura K. Ferris, MD, PhD.
Figure 3. Guttate psoriasis with characteristic erythematous, scaly papules and small plaques on the back.
Guttate psoriasis (named for its droplet-shaped lesions) presents abruptly with 1-mm to 10-mm pink papules with associated fine scale over the trunk and extremities (Figure 3). This variant occurs in fewer than 2% of patients with psoriasis, who are usually younger than 30 years. It is often preceded 2 to 3 weeks earlier by an upper respiratory tract infection with group A beta-hemolytic streptococci.

Erythrodermic psoriasis

Approximately 1% to 2.25% of all patients with psoriasis develop this severe form, affecting more than 75% of the body surface area. It presents as generalized erythema, which is the most prominent feature, and it is often associated with superficial desquamation, hair loss, nail dystrophy, and systemic symptoms such as fever, chills, malaise, or high-output cardiac failure. There may be a history of preceding characteristic psoriatic plaques, recent withdrawal of treatment (usually corticosteroids), phototoxicity, or infection.

Conversely, approximately 25% of all patients with erythroderma have underlying psoriasis.28

Pustular psoriasis

Photo courtesy of Joseph C. English III, MD.
Figure 4. Erythematous plaques studded with pustules and red-brown macules on the acral surface of the foot in palmoplantar pustulosis.
Pustular psoriasis (Figure 4) is uncommon. The predominant lesions are large collections of neutrophils in the stratum corneum that clinically present as sterile pustules. The pustules may be localized within or at the edge of existing psoriatic plaques or may present as a generalized eruption.

There are several forms of pustular psoriasis, including generalized pustular psoriasis, annular pustular psoriasis, impetigo herpetiformis (pustular psoriasis of pregnancy), and palmoplantar pustulosis. However, there is some evidence to suggest that palmoplantar pustulosis may be distinct from psoriasis.29

Several triggers have been identified, including pregnancy, rapid tapering of medications, hypocalcemia, infection, or topical irritants.

Generalized pustular psoriasis, annular pustular psoriasis, and impetigo herpetiformis often present in association with fever and other systemic symptoms and, if left untreated, can result in life-threatening complications including bacterial superinfection, sepsis, dehydration, and, in rare cases, acute respiratory distress secondary to aseptic pneumonitis.30

Placental insufficiency resulting in stillbirth or neonatal death and other fetal abnormalities can occur in severe pustular psoriasis of pregnancy.31

Psoriatic arthritis

Psoriatic arthritis is a seronegative inflammatory spondyloarthropathy that can result in erosive arthritis in up to 57% of cases and functional disability in up to 19%.32 Although rare in the general population, it affects approximately 6% to 10% of psoriasis patients and up to 40% of patients with severe psoriasis.33 In 70% of cases, psoriasis precedes the onset of arthritis by about 10 years, and approximately 10% to 15% of patients simultaneously present with psoriasis and arthritis or develop arthritis before skin involvement.5,34

Patients complain of joint discomfort that is most prominent after periods of prolonged rest. Patterns of involvement are extremely variable but have been reported as an asymmetric oligoarthritis (involving four or fewer joints) or polyarthritis (involving more than four joints) in most patients. A rheumatoid arthritis-like presentation with a symmetric polyarthropathy involving the small and medium-sized joints has also been reported, making it difficult to clinically distinguish this from rheumatoid arthritis.

A distal interphalangeal-predominant pattern is reported in 5% to 10% of patients. Axial disease resembling ankylosing spondylitis occurs only in 5% of patients. Arthritis mutilans, characterized by severe, rapidly progressive joint inflammation, joint destruction, and deformity, occurs rarely. Enthesitis, ie, inflammation at the point of attachment of tendons or ligaments to bone, is present in up to 42% of patients.5,35

Nail disease

Photo courtesy of Joseph C. English III, MD.
Figure 5. Nail pitting and onycholysis with surrounding psoriatic plaques along the perionychium and proximal nail fold.
Nail psoriasis occurs in 35% to 50% of patients and can be seen with all forms of psoriasis.1 Involvement of the nail matrix can result in nail pitting and leukonychia. Oil spots, subungual hyperkeratosis, and distal onycholysis are the result of disease involvement of the nail bed (Figure 5). Up to 90% of patients with psoriatic arthritis have nail changes, especially patients with enthesitis.36

Disease severity also varies

Disease severity also differs among patients. An estimated 80% of patients have mild to moderate disease and 20% have moderate to severe disease, which includes disease involving more than 5% of the body surface or involvement of the face, hands, feet, or genitalia.1

The Psoriasis Area and Severity Index (PASI) is an objective measure used in clinical trials. It incorporates the amount of redness, scaling, and induration of each psoriatic lesion over the body surface involved. A 75% improvement in the PASI score (PASI-75) is regarded as clinically significant.37