Rheumatoid arthritis and Still’s disease

Rheumatoid arthritis (RA) is a connective tissue disorder resulting in an inflammatory arthritis. While it is unusual for RA to present with skin problems, cutaneous features are not uncommon. Still's disease, also known as juvenile rheumatoid arthritis, is characterised by intermittent high fevers associated with a salmon-coloured rash, and arthritis.

This chapter, as set out below, provides a brief overview of the cutaneous manifestations of RA and Still's disease. 

RA - cutaneous features

• The skin becomes atrophic and bruises easily

• The nails may become brittle and split length-wise

• Palmar erythema

• Rheumatoid nodules
• Firm, skin-coloured, non-tender, mobile nodules within the subcutaneous tissue. Most nodules are found on bony prominences, adjacent to joints and other extensor surfaces
• Refer to the related chapter Rheumatoid nodules for more information 

• Vascular lesions
• ​Raynaud's phenomenon 
• Nailfold infarcts 
• Small vessel vasculitis resulting in purpura, and occasionally tissue necrosis 
• Leg ulcers - often have a mixed aetiology and are difficult to treat 

• Interstitial granulomatous dermatitis - firm, non-tender linear or arciform bands of affected skin, typically extending from the anterior and posterior part of the axillae to the flank. A papular variant has been described

• RA has also been associated with Sweet's syndrome and  pyoderma gangrenosum 

Still's disease

• Usually affects children, but occasionally adults 

• Fatigue is common

• Fever - an intermittent high fever occurs in all patients, and tends to spike at a similar time every day

• A rash coinciding with the fever affects many patients. Characteristics of the rash are:
• Distribution - usually arises on the limbs and trunk, but may occur on the face or neck
• Morphology - small, non-pruritic macules or papules, and occasionally large patches up to five cm in diameter. Salmon-pink coloured, and often surrounded by a zone of pallor 

• Other systemic features include:
• Lymphadenopathy and hepatosplenomegaly 
• Haematological changes - leucocytosis, anaemia, raised inflammatory markers, high levels of AST and ALT, negative Rheumatoid factor and ANA
• Pleuritis and / or pericarditis

• In many cases the rash and fever may occur intermittently for months or even years before joint symptoms develop 

• All patients with Still's disease eventually develop chronic arthritis 

• Patients with RA or suspected Still's disease are generally managed by Rheumatologists