Derm Dx: ‘Twisted’ hands

Rheumatoid arthritis (RA) is a chronic inflammatory disorder characterized with insidious onset of symmetric morning stiffness and polyarthritis of the wrists, metatarsophalangeal, proximal interphalangeal and metatarsophalangeal joints. It's a worldwide problem and affects 0.5 to 1% of the population. Although...

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Rheumatoid arthritis (RA) is a chronic inflammatory disorder characterized with insidious onset of symmetric morning stiffness and polyarthritis of the wrists, metatarsophalangeal, proximal interphalangeal and metatarsophalangeal joints. It’s a worldwide problem and affects 0.5 to 1% of the population.

Although the cause of RA is unknown, it is thought that genetics play a role, as there is a strong association for RA with the Human Leukocyte Antigen (HLA)-DR haplotype — a gene found in the major histocompatibility complex (MHC) class II locus.

It is thought that inflammation in RA results from multiple pathways, which eventually lead to tissue destruction and joint deformations, such as ulnar deviation, swan-neck and boutonniere deformities of the hands. The cutaneous manifestations of rheumatoid arthritis will be reviewed here. 

Cutaneous manifestations of RA include the following:

Rheumatoid nodules (RN) are the most common extra-articular manifestation of RA, occurring in 20% of the patients with the disease. RNs are subcutaneous, firm, skin-colored nodules that range in size from <5 mm to several centimeters in diameter.

They can be freely mobile of fixed to surrounding tissue and can be painless or painful — if located in plantar surfaces. The nodules are benign. However, secondary infection or reduced joint mobility (due to location) can occur. Treatment is not necessary if nodules are asymptomatic.

Rheumatoid vasculitis refers to vasculitis that occurs in patients usually with systemic RA, and ranges from mild to severe. In mild forms, the vasculitis occurs in the skin in the form of petechiae, minute digital ulcerations, livedo reticularis and nail-fold telangiectasias.

However, in severe forms it involves internal organs including the heart, lungs, GI, CNS, as well as the skin, which can manifest as painful, punched-out leg ulcers that appear along the lateral malleolus, palpable purpura, digital infarcts and distal gangrene. Ulcers resolve with systemic therapy.

Felty syndrome (FS) is the development of neutropenia and splenomegaly in the setting of RA. Cutaneous manifestations of Felty syndrome include rheumatoid nodules, and lower extremity ulcers. Ulcers in FS are chronic, deep and reside over the skins and ankles. Unfortunately, patients with FS have an increased mortality due to sepsis.

Pyoderma gangrenosum. The ulcerative form — out of the four stages of PG (ulcerative, pustular, bullous and vegetative) — is associated with RA. Patients begin with multiple tender erythematous-to-violaceous papules, which rapidly turn into purulent ulcers. These last several months and resolve with and without treatment.

Interstitial granulomatous dermatitis with arthritis. The lesions associated with this condition are erythematous-to-violaceous, non-pruritic cord-like, annular or linear plaques in the axilla, torso and inner thighs. Patients may describe a burning sensation in the lesions. Flares and remissions are characteristic.

Juvenile rheumatoid arthritis (JRA), like RA, is a chronic disease. It’s characterized by the onset of arthritis with duration of 1.5 months or longer at the age of 16 years. The types of arthritis are classified as pauciarticular, polyarticular and systemic (Still’s) disease. In pauciarticular disease, one to four joints are involved and in polyarticular disease, five or more joints are involved.

A pink macular rash in the face, trunk and limbs, recurrent high fevers, fatigue and joint pain characterize Still’s disease. Once the fever subsides, the rash fades; however, it reappears in subsequent fever spikes. Treatment for JRA will treat Still’s disease.

Treatment & prognosis

In RA, the course and outcome are variable from mild to severe disease. There is no cure for RA, and it is very important to educate the patient and family about the chronic nature of the disease.

The goals of treatment are to reduce patient discomfort and prevent joint damage, loss of function and deformities. Treatment is based on severity of RA disease. Current therapies include steroids, NSAIDs, disease-modifying anti-rheumatic drugs (DMARDs), including methotrexate and biologics (e.g. etanercept).

Even with these drugs, many patients still have active disease and may require other forms of treatment, such as surgery and physical therapy.

Nelly Song is a senior medical student at the University of Texas Medical School at Houston.

Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine also in Houston.