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Systemic dermatology

Connective tissue diseases key points test

Which of the following statements about discoid lupus erythematosus are correct? # It is rare in males (<5%) #! It presents as red dry plaques on light-exposed areas #! The plaques heal with scarring # Hydrocortisone cream is generally effective Explanation: Discoid lupus erythematosus (LE) is more common in females than in males with a ratio of 2:1. It results in red scaling plaques on light-exposed areas especially the face and scalp. The plaques heal with scarring. Treatment requires potent or ultrapotent topical steroids and often systemic medication. Which of the following are suitable treatments for cutaneous lupus erythematosus? #! Clobetasol propionate cream # Topical diclofenac gel #! Hydroxychloroquine # Doxycycline Explanation: Treatment of cutaneous LE includes sun protection, potent topical steroids, antimalarials and a variety of immunosuppressive treatments. Which of the following features suggest systemic lupus erythematosus? #! Photosensitivity #! Diffuse alopecia #! Leucopaenia # Positive anti-Ro antibodies Explanation: Systemic LE is characterised by a macular facial rash, photosensitivity, patchy hair loss, palmar erythema and periungual telangiectasia. Systemic symptoms include fever, fatigue, arthritis, CNS, renal, cardiac and pulmonary disease. Positive autoantibodies usually include ANA and anti-double-stranded DNA. Anti-Ro antibodies are characteristic of subacute cutaneous LE. Dermatopathological features of lupus erythematosus include: * Spongiosis *! Lichenoid tissue reaction * Intraepidermal immunoglobulins * Parakeratosis and keratinocyte hyperplasia Explanation: Dermatopathological findings in LE include a lichenoid tissue reaction and positive direct immunofluorescence with immunoglobulins at the dermo-epidermal junction. Scleroderma is seen in which of the following conditions? # Acute graft versus host disease #! Morphoea # Polymyositis #! CREST syndrome Explanation: Scleroderma is seen in morphoea, Raynaud’s disease, systemic sclerosis, mixed connective tissue disease, scleroedema and chronic graft versus host disease. Systemic sclerosis is characterised by which of the following features? #! Calcinosis # Muscle weakness # Ivory white oval sclerotic plaques #! Raynaud’s phenomenon Explanation: Systemic sclerosis is characterised by progressive accumulation of collagen, fibrosis and loss of mobility of the skin and other organs. The CREST variant ( Calcinosis, Raynaud’s, oEsophageal , S clerodactyly, Telangiectasia) may have less severe systemic manifestations. Ivory white plaques are not due to systemic sclerosis but describe morphoea. Muscle weakness is characteristic of dermatomyositis. Chronic graft-versus-host disease after bone marrow transplantation: #! Arises more than 100 days after transplantation # Affects 75% of survivors # In 30% cases results in toxic epidermal necrolysis #! May result in scleroderma and lichenoid plaques Explanation: 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. Toxic epidermal necrolysis may arise in acute GVHD Dermatomyositis is characterised by which of the following features? #! Association with malignancy in those over 55 #! Red-purple rash affecting the face, chest and hands # Peripheral myopathy # Raynaud’s phenomenon Explanation: Dermatomyositis 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 Skin lesions associated with rheumatoid arthritis include: #! Rheumatoid nodules # Erythema marginatum #! Pyoderma gangrenosum #! Vasculitic leg ulcers Explanation: Skin lesions associated with rheumatoid disease include rheumatoid nodules, vasculitis, nail fold telangiectases, palmar erythema and ragged cuticles, pyoderma gangrenosum, Felty’s syndrome and venous disease.