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Scleroderma

Scleroderma in childhood is rare and variable in clinical presentations. 

Localized scleroderma is the most common and can present at any age, with the appearance of a patch of abnormal skin (called morphoea) and which when untreated, generally follows a course of active expanding disease, fibrosis and eventual softening with some “remission”.

The functional and cosmetic impact can be profound, as the lesions may interfere with growth of a limb and subcutaneous tissues (of the face or a limb). Hemi-facial atrophy (Parry Romberg syndrome) and En Coup de Sabre are classical examples of localised scleroderma.

Management includes aggressive treatments (corticosteroids and methotrexate, as well as other immunosuppressives) with the aim of limiting severe disfigurement and disability.

The photograph below shows morphoea (on the abdomen) - this is the commonest form of scleroderma in children

The photograph below shows linear scleroderma with pigmented indurated shiny lesions 

The photograph belows shows linear scleroderma with pigmented indurated shiny lesions and the right foot is smaller than the left

The photograph below shows linear scleroderma with pigmented lesions over the trunk.

 The photograph below shows linear scleroderma with typical 'waxy' pigmented lesions over the foot.

The photograph below shows facial involvement with scleroderma (en coup de sabre) 

The photograph below shows hemi-facial atrophy (Parry Romberg)

Systemic scleroderma is extremely rare in children and includes progressive diffuse fibrous changes of the skin and fibrous changes involving internal organs — most commonly lungs, gastrointestinal tract, heart and kidneys — with a significant mortality.

The radiograph below is a barium swallow and shows a dilated oesophagus in systemic sclerosis.  

Systemic scleroderma is slowly progressive, has a guarded prognosis and requires potent immunosuppression. 

The photograph below shows typical 'salt and pepper' pigmentation in systemic sclerosis 

The photograph below shows sclerodactyly (tapered fingers) in systemic sclerosis

 

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