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Nodular or keloidal morphea, also known as nodular scleroderma, is a rare form of localized sclerosis (SSc) or morphea. In this paper, we reported a case of this rare entity with a review of the literature. [ABSTRACT FROM AUTHOR]

Background: Paediatric localized scleroderma is a severe inflammatory disorder associated with tissue atrophy, often leading to disability. Assessing disease activity and response to treatment has always been challenging and remains an important difficulty in clinical practice. Objectives: To investigate prospectively the efficacy of systemic treatment with corticosteroids and methotrexate in children with localized scleroderma and the validity of infrared thermography, laser Doppler flowmetry and high‐frequency ultrasound in assessing disease activity. Methods: Children with localized scleroderma were prospectively treated with corticosteroids (initially pulsed IV methylprednisolone 30 mg/kg/day, maximum 500 mg/day and/or oral prednisolone 0.5–1 mg/kg/day) and methotrexate (15 mg/m2 weekly). Treatment response was evaluated using a clinical activity score. Skin temperature, blood flow, dermal thickness and dermal echogenicity of clinically active skin lesions were determined in relation to the unaffected contralateral site at baseline and after 3, 6, 12 and 18 months. Patient charts were later reviewed for long‐term follow‐up. Results: Twenty‐two patients were included [age 6.0 (0.2–14.4] years; female‐to‐male ratio 3.4 : 1) All responded well to therapy. Disease reversibility was demonstrated in the majority of children with partial resolution of skin sclerosis and regrowth of hair. Laser Doppler flowmetry and high‐frequency ultrasound findings correlated with disease activity at baseline. Thermography had no added value in this cohort. The recurrence rate was 36% in the follow‐up period. Conclusions: Corticosteroids and methotrexate are highly effective as first‐line therapy in paediatric localized scleroderma, leading to partial reversal of skin manifestations. However, the recurrence rate is substantial and affected children require long‐term follow‐up. Laser Doppler flowmetry and high‐frequency ultrasound correlate with disease activity in the acute phase and may assist decision‐making in these patients. [ABSTRACT FROM AUTHOR]

Progress in the understanding of the aetiopathogenesis of scleroderma (systemic sclerosis) has been painfully slow and this has greatly impeded the application of specific disease modifying treatments. This article provides a brief historical overview of scleroderma (including the important Australian contribution of Dr Alfred Barnett and colleagues – see accompanying article in this issue on pages 513–18), highlights some recent pathogenic developments and summarises some exciting new therapies. Cautious optimism can now be offered to scleroderma sufferers. [ABSTRACT FROM AUTHOR]