Skin signs of rheumatic disease

Author(s): Jenny Chung, House officer, Auckland City Hospital, New Zealand. DermNet New Zealand Editor in Chief: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy editor: Gus Mitchell, November 2017.

What is rheumatic disease?

Many autoimmune connective tissue diseases and vascular conditions in rheumatology have cutaneous manifestations.

Rheumatic diseases described on this page are:

Cutaneous lupus erythematosus

About 25% of patients with systemic lupus erythematosus (SLE) initially present with skin involvement. It is important to correctly classify cutaneous lupus erythematosus (CLE), as it helps determine the underlying type and severity of SLE.

Skin manifestations of lupus erythematosus (LE) are commonly divided into LE– specific and LE–non–specific disease. Note that four of the nine American College of Rheumatology criteria for SLE are skin signs (malar/butterfly rash, discoid plaques, photosensitivity, and oral ulcers).

LE–specific disease

Acute CLE

Subacute cutaneous LE (SCLE)

Chronic CLE 

Drug-induced LE

Rarer types of LE

LE–non–specific disease

Cutaneous vascular disease

Other symptoms

Dermatomyositis

Dermatomyositis, also referred to as idiopathic inflammatory myopathies, is a heterogenous group of autoimmune disorders affecting the skin and/or musculature. Skin signs are critical in diagnosing and classifying dermatomyositis.

The hallmark skin signs of dermatomyositis help differentiate it from CLE.

Classic skin signs of dermatomysitis are:

Other skin signs of dermatomyositis

Systemic sclerosis

Systemic sclerosis is a multi-system form of scleroderma with hallmark skin signs. It is associated with a high mortality and morbidity rate. Clinical features of systemic sclerosis are diverse affecting multiple organs in the body. Two important skin signs are Raynaud phenomenon and skin sclerosis; these help classify the disease into the following subsets.

Localised systemic sclerosis

CREST syndrome

Visceral disease may begin after a long period of Raynaud disease.

Diffuse systemic sclerosis

Overlapping systemic sclerosis

Patients have features of scleroderma with another autoimmune rheumatic disease, such as dermatomyositis or polymyositis, SLESjögren disease, vasculitis, or polyarteritis.

Scleroedema

Scleroedema is a rare disease caused by excessive mucin and collagen production. it is associated with preceding infection, diabetes and paraproteinaemia. 

Scleromyxoedema

Scleromyxoedema is a rare disease caused by excessive mucin production and fibrocyte hyperplasia. It is associated with paraproteinaemia and skin signs.

Sjögren syndrome

Sjögren syndrome is a systemic autoimmune disorder that primarily reduces the function of sweat glands and mucosal mucous glands causing sicca symptoms. It is much more common in women than in men. 

Mucosal signs of Sjögren syndrome

Skin signs of Sjögren syndrome

Rheumatoid arthritis

Rheumatoid arthritis (RA) is a systemic autoimmune inflammatory disorder that causes debilitating symmetrical polyarthritis and other  manifestations. One of the seven criteria for RA is the presence of rheumatoid nodules. Skin signs can be divided into RA-specific and nonspecific signs.

RA-specific skin signs

Other signs of vasculitis associated with RA include:

RA-nonspecific skin signs

Other skin signs arising in rheumatoid arthritis include;

Systemic-onset juvenile arthritis

Systemic–onset juvenile arthritis is a rheumatic disease characterized by high fevers, skin signs (in 95%) and arthritis. Lymphadenopathy, myalgia and abdominal pain may be present. Key signs include:

Relapsing polychondritis

Relapsing polychondritis is a rare multisystem autoimmune disease, often associated with an autoimmune or haematological condition. Skin signs in relapsing polychondritis are nonspecific; they are not diagnostic criteria nor are they associated with disease severity.

Hallmark signs of relapsing polychondritis are:

Other skin signs include:

Psoriatic arthritis

Psoriatic arthritis is a form of spondyloarthropathy that can be associated with psoriasis. Psoriasis typically presents with red scaly plaques on scalp and extensor surfaces as well as nail dystrophy. There are subtypes of psoriasis with specific skin signs.

Chronic plaque psoriasis (psoriasis vulgaris)

Guttate psoriasis

Nail psoriasis

Psoriatic erythroderma

Pustular psoriasis

Vasculitis

Systemic vasculitis is a heterogenous group of disorders with inflammation of blood vessel walls. The skin and blood vessels have a close anatomical and physiological relationship, hence skin signs are common in vasculitis

These skin signs prompt an investigation for any systemic involvement of vasculitis. However, the skin signs alone are not sufficient for diagnosis of a specific condition.

Small vessel vasculitis

Small vessel vasculitis present with palpable purpura, usually more pronounced on gravity-dependent areas, with associated oedema.

ANCA-associated vasculitides

Cryoglobulinaemic vasculitis

Urticarial vasculitis

Henöch–Schonlein purpura

Erythema elevatum diutinum

Degos disease/malignant atrophic papulosis

Medium vessel vasculitis

Polyarteritis nodosa

Buerger disease

Kawasaki disease

Large vessel vasculitis

Takayasu arteritis

Giant cell arteritis

Cogan syndrome

Behçet disease

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