Connective tissue diseases
- Recognise cutaneous connective tissue diseases.
The so-called connective tissue diseases are the result of environmental stimuli (such as drugs, infection or exposure to ultraviolet radiation) in genetically predisposed individuals. They are associated with a range of circulating autoantibodies.
- All varieties of lupus erythematosus (LE) are more common in females than in males.
- Discoid LE is the most common presentation and results in red scaling plaques on light-exposed areas especially the face and scalp. The plaques heal with scarring.
- Subacute cutaneous LE is characterised by more widespread photosensitive annular plaques.
- Positive anti-Ro antibodies are characteristic of subacute cutaneous LE.
- Treatment of cutaneous LE includes sun protection, potent topical steroids, antimalarials and a variety of immunosuppressive treatments.
Localised discoid LE
Severe discoid LE without systemic features
Subacute cutaneous LE
- Systemic lupus (SLE) is characterised by a “butterfly” macular facial rash, photosensitivity, patchy hair loss, palmar erythema and periungual telangiectasia.
- Systemic symptoms include fever (>90%), fatigue (>90%), arthritis, CNS, renal, cardiac and pulmonary disease.
- Drug-induced lupus may be due to a variety of antibiotics, antihypertensive and anti-inflammatory medications.
Butterfly rash in SLE
Discoid lupus in patient with SLE
Lupus chilblains (SLE)
© R Suhonen
- Dermatopathological findings in SLE include a lichenoid tissue reaction and a band of immunoglobulins at the dermo-epidermal junction (detected by direct immunofluorescence of clinically involved skin).
Low power view shows dense perivascular and periadnexal inflammatory infiltrate
Medium-powered view shows interface changes and lymphocytic infiltrate
High-power view of vacuolar degeneration of basal layer
Systemic lupus erythematosus
Investigations should include:
- CBC (anaemia, leucopaenia, thrombocytopaenia suggest SLE)
- ESR (reflects activity of SLE)
- Urinalysis (proteinuria and casts in renal lupus)
- Auto-antibodies including ANA (positive in >90% of SLE), anti-double-stranded DNA
- Lupus anticoagulant
- Complement profile
Scleroderma (tight skin) is seen in the following conditions:
- Raynaud’s disease (Raynaud’s without known underlying disease)
- Systemic sclerosis
- Mixed connective tissue disease
- Chronic graft versus host disease
- Morphoea is characterised by ivory white oval sclerotic plaques with an inflamed edge that eventually result in post-inflammatory pigmentation and dermal or subcutaneous atrophy.
- A linear variety can involve underlying muscle and bone. If it affects the face and /or scalp, it is also known as en coup de sabre.
© R Suhonen
En coup de sabre
- Systemic sclerosis is characterised by progressive accumulation of collagen, fibrosis and loss of mobility of the skin and other organs such as the respiratory and gastrointestinal tracts.
- The CREST variant (Calcinosis, Raynaud’s, oEsophageal, Sclerodactyly, Telangiectasia) may have less severe systemic manifestations.
- Treatment is mainly symptomatic at this time.
Severe systemic sclerosis
Chronic graft-versus-host disease (GVHD)
- Chronic GVHD arises more than 100 days after bone marrow transplantation and affects 25% of survivors
- It may result in generalised scleroderma, lichen planus-like papules and lichenoid histology.
- Dermatomyositis can be seen in children and adults.
- It is sometimes associated with malignancy, especially in those over 55 years of age.
- It presents with proximal muscle weakness, elevated muscle enzymes and a rash affecting the face, chest, arms and hands
- Dermatomyositis of the eyelids is known as heliotrope rash.
- Violaceous plaques over the knuckles are known as Gottron’s papules.
- Rheumatoid nodules are subcutaneous necrobiotic granulomas
- Vasculitis may result in palpable purpura and painful ulceration
- Nail fold telangiectases, palmar erythema and ragged cuticles are common in all connective tissue diseases
- Other associated dermatoses include non-vasculitic leg ulcers due to pyoderma gangrenosum, Felty’s syndrome and venous disease
- Sjögren’s syndrome (dry eyes, dry mouth) may cause generalised pruritus, vaginal xerosis and dyspareunia, vasculitis and systemic symptoms.
Rheumatoid neutrophilic dermatosis
- Autoimmune reaction to Group A streptococcal pharyngitis
- Clinical criteria for diagnosis are fever, migratory polyarthritis, carditis and Sydenham’s chorea.
- Erythema marginatum is characterised by erythematous, gyrate, non-scaly annular plaques or macules that migrate centrifugally.