Optimal management strategies for paediatric morphea – insights from a case series

INTRODUCTION

Juvenile localised scleroderma (morphea) refers to a number of different conditions characterised by skin thickening with increased collagen deposition. The prevalence of all types of morphea ranges from 0.4 to 2.7 per 100,000 people, and it is more common in Whites and females [1]. Several types of localised scleroderma are distinguished: plaque, generalised, bullous, linear, and deep morphea [2]. Linear scleroderma, with a prevalence of 65%, seems to be the most common type in children [3].
The spectrum of skin lesions in morphea is wide – from single plaques, through linear streaks of sclerosis, to serious cases such as facial hemiatrophy in Parry-Romberg syndrome [2]. The aetiology of the condition remains unclear, but different factors, such as trauma, vascular abnormalities, genetic factors, autoimmune processes, and infection, are taken into consideration [2]. Even though many treatment options are available, there is a lack of united guidelines regarding recommended therapies in children.

CASE REPORT

Seven children were hospitalised at the Dermatology Department because of linear morphea. All of them were girls aged 5–12 years. In 2 of them, lesions were located on the forehead, while others presented lesions mainly on their limbs. All were treated with a combination of methotrexate (MTX) and systemic corticosteroid – in 2 cases the corticosteroid therapy preceded MTX administration. In all cases, the doses of prednisone were gradually decreased after MTX administration and improvement of clinical condition (inactive stage of the disease); any side effects or increase in morphea activity were reported. In addition, all patients used topical agents on the affected area – in the first phase local glucocorticoids were administered, which were later substituted by tacrolimus. In each patient we achieved suppression of progression of disease activity and gradual improvement of skin condition (Figures 1, 2).
Each patient had regular controls of blood parameters and clinical condition. We observed improvement of skin lesions in all patients. The first effects were seen already on the first control appointments, which took place 1–2 months after the beginning of therapy. First reported effects were mainly ceasing the development and enlargement of the present lesions and lack of new ones. Later, the reduction of erythema, increased mobility, and reduced thickness of the skin appeared. We use the modified localised scleroderma severity index to monitor the effectiveness of the used therapy.
Only one patient reported side effects – stretch marks and nausea. When laboratory parameters are taken into consideration, we observed intermittently slightly increased levels of serum creatinine (up to 0.93, N 0.39–0.73 mg/dl) and total bilirubin (up to 1.55 mg/dl, N 0.20–1.00 mg/dl) in only one patient, but they were present before MTX administration and did not worsen during the therapy. Other patients presented no significant deviations in morphology, creatinine level, and liver function tests before and after introducing the treatment.
In one case MTX therapy was discontinued because of lack of compliance. Consequently, we observed a significant progression of disease activity and skin lesions. After reintroducing MTX to the therapy, the condition of the skin improved again. The therapy is being continued without any side effects or alterations in laboratory tests.
All information about our patients is presented in Table 1.

DISCUSSION

Morphea, or localised scleroderma, is a rare chronic inflammatory disease affecting the skin and subcutaneous tissue [4]. It manifests equally in adults and children, with incidence peaks typically occurring the ages of 7–11 years in children and 40–50 years in adults [5, 6]. Approximately 15% of cases occur in children under 10 years old [7]. Our case report series, consistent with literature findings, included only female patients, highlighting a female predominance in morphea [4].
The aetiopathogenesis of morphea remains unclear, with genetic predisposition, autoimmune dysregulation, and environmental factors implicated [4]. Linear localised scleroderma, the most common clinical type in the paediatric population, affects 51–65% of children with morphea, with peak morbidity observed around ages 7–8 years [3, 7]. Two of our patients exhibited forehead lesions, a manifestation of rare subtypes within linear scleroderma such as en coup de sabre and progressive facial hemiatrophy (PFH). En coup de sabre typically begins at the upper eyebrow ridge and extends to the scalp, potentially leading to cicatricial alopecia, while PFH involves severe atrophy affecting soft and osseous tissues, oral structures, and teeth, often accompanied by craniofacial deformities and neurological complications [6, 8]. Due to these potential complications, it is recommended that head magnetic resonance imaging (MRI) and ophthalmological evaluations in affected patients be conducted.
In children, linear scleroderma tends to present a more severe clinical course compared to adults, often resulting in significant atrophy of skin, fat tissue, fascia, and muscle, leading to functional impairment and disability [9]. Systemic therapy introduced early during the active stage of juvenile linear scleroderma is crucial to halt disease progression, prevent new lesion formation, and improve existing ones [10]. Although various topical and systemic therapies are available (Table 2), including MTX monotherapy and combinations with glucocorticosteroids like methylprednisolone or prednisone, evidence supporting their efficacy in children remains limited [11]. Our case series employed the combination of MTX and oral prednisone, demonstrating satisfactory disease control and gradual regression of skin lesions [5, 6, 10, 12–14]. Notably, side effects were minimal, with only one patient experiencing stretch marks and nausea.
Assessment of treatment efficacy typically reveals reduction in skin sclerosis within 8–12 weeks post-therapy initiation [5]. Further research is needed to establish optimal treatment protocols tailored to the specific needs of paediatric patients with morphea.

CONCLUSIONS

Juvenile localised scleroderma represents a rare and inflammatory condition necessitating tailored therapeutic strategies. In cases where sclerotic lesions involve the face and head, conducting comprehensive evaluations such as head MRI and ophthalmological assessment is imperative to exclude associated underlying pathologies. Our study underscores the efficacy of combining MTX with oral prednisone as a viable treatment modality for linear localised scleroderma in paediatric patients. Nevertheless, there remains a critical need for further rigorous cross-sectional studies to generate evidence of high certainty that can inform the development of standardised treatment guidelines for juvenile localised scleroderma.

DISCLOSURES

1. The study was funded by the Medical University of Łódź, project no. 503/1-064-01/503-51-001-19-00.
2. Assistance with the article: None.
3. Financial support and sponsorship: None.
4. Conflicts of interest: None.

REFERENCES