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History
A 30-year-old woman presents with 10-month history of asymptomatic skin lesions developing over her trunk. Initially the lesions were red oval patches, which over many months became hard and the skin white. She does not have any joint problems, there is no family history of skin disease and she is otherwise well.
Examination
There are scattered, oval, indurated shiny white plaques with a purplish, poorly defined border predominantly over her trunk (Fig. 33.1). Some of the lesions have now become more hyperpigmented.
Questions
• What is the diagnosis?
• What are the possible triggers?
• How should the patient be managed?
Medicine
This patient’s skin biopsy confirmed the clinical diagnosis of morphoea. Palpation of the skin is an extremely important part of a dermatological examination. Plaques of mor-phoea feel very firm to the touch and the surface of the skin has lost its normal texture.
The skin feels smooth and often waxy. Histopathology shows a proliferation of collagen in the dermis and subcutis. The cause of this localized dermal scleroderma is unknown.
Women between the ages of 20–50 years are most commonly affected. The lesions themselves are usually asymptomatic. Skin changes are usually localized and initially erythematous/violaceous oval plaques which progress into ivory-coloured, hardening plaques measuring 1–20 cm in diameter. Morphoea may be circumscribed, generalized, linear and occasionally involve underlying structures (pansclerotic).
This patient had the generalized form of the disease with multiple plaques over her trunk.
Linear morphoea, in contrast, normally affects the extremities, particularly over the fron-toparietal scalp/face where a deep-seated form of linear scleroderma ‘en coup de sabre’ can occur. The sclerotic skin grows no hair and the underlying skull bone may become distorted.
Although the aetiology of morphoea is unknown, recognized triggers include local skin injury, post-radiation and viral infections such as measles. A small proportion of patients with classical morphoea developed the disease following tick bites, which transmit Lyme disease due to Borrelia burgdorferi infection. Autoantibodies may be positive including rheumatoid factor, antinuclear and antihistone antibodies.
The clinical course is generally slowly progressive, although some cases can ‘burn out’ and others spontaneously resolve. Extensive sclerotic plaques on the limbs can be associ-ated with stiffness, weakness and loss of mobility.
Management is difficult. However, some success has been with topical vitamin D ana-logues (calcipotriol), intralesional and systemic corticosteroids, methotrexate, ciclosporin and psoralen–UVA/UVB or UVA1.
KEY POINTS
• Morphoea appears as ivory-coloured, hardening plaques.
• It can be circumscribed, generalized, linear and may affect underlying structures.
• The aetiology is unknown, but triggers include local skin injury and viral infections.
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