Morphea, also known as localized scleroderma, is a rare fibrosing disorder of the skin and underlying tissues Morphea is differentiated from systemic sclerosis based on the absence of sclerodactyly, Raynaud phenomenon, and nailfold capillary changes Patients with morphea commonly have systemic symptoms, such as malaise, fatigue, arthralgias, and myalgias, as well as positive autoantibody serologies However, involvement of morphea is almost uniformly limited to those tissues derived from the mesoderm The underlying pathogenesis of morphea is incompletely understood at this time, but ultimately results in an imbalance of collagen production and destruction

https://www.jaad.org/article/S0190-9622(10)00746-2/pdf

Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis

Background The relationship between en coup de sabre morphea and Parry-Romberg syndrome is unclear, and not much is known regarding their demographic and clinical characteristics or the efficacy of the treatments that are used. Objective The purpose of this study was to describe demographic data, clinical features, and medications used in a large cohort of patients with en coup de sabre morphea and Parry-Romberg syndrome. Methods A retrospective review of patients diagnosed with en coup de sabre morphea or Parry-Romberg syndrome at the Mayo Clinic from 1984 to 2004 was conducted. Demographic data, examination findings, and treatments were noted. Results We identified 54 patients who met criteria for inclusion in the study. Twenty-six patients (48%) had en coup de sabre morphea, 13 (24%) had Parry-Romberg syndrome, and 15 (28%) had both. Disease was present bilaterally in 7.4% of patients. Thirteen percent of all patients in the study group had seizures. Of patients who received treatment, most were treated with antimalarial agents (57.1%) or methotrexate (28.6%). Limitations The study design was that of a retrospective review at a tertiary care center with referral bias. There were also limitations present because of the inherent nature of the diseases studied. Conclusion En coup de sabre morphea and Parry-Romberg syndrome frequently coexist and are likely both variants of morphea. Bilateral disease is more common than previously reported. The efficacy of antimalarials and methotrexate in the treatment of these diseases remains unclear.

En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients

Objective
Morphea is associated with systemic manifestations and autoimmunity, but the prevalence of these findings remains unclear. With the goal of characterizing the frequency of these associations the prevalence of extra-cutaneous manifestations and autoimmunity in adult and pediatric morphea subjects was ascertained.

https://jamanetwork.com/journals/jamadermatology/articlepdf/712042/dst80031_545_550.pdf

Distinct Autoimmune Syndromes in Morphea: A Review of 245 Adult and Pediatric Cases

Background Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement. Objectives We sought to determine the clinical features of morphea in a large pediatric cohort. Methods We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006. Results Most children showed linear morphea, with a disproportionately high number of Caucasian and female patients. Two patients with rapidly progressing generalized or extensive linear morphea and arthralgias developed restrictive pulmonary disease. Initial oral corticosteroid treatment and long-term methotrexate administration stabilized and/or led to disease improvement in most patients with aggressive disease. Limitations Retrospective analysis, relatively small sample size, and risk of a selected referral population to the single site are limitations. Conclusions These data suggest an increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms. Visceral manifestations rarely occur; the presence of progressive problematic cutaneous disease and arthralgias should trigger closer patient monitoring.

Pediatric morphea (localized scleroderma): review of 136 patients.

Background Localized scleroderma (LS) or morphoea is often considered to be a benign self-limiting condition confined to the skin and subcutaneous tissue. However, the course of the disease is unpredictable and severe functional and cosmetic disability may result. Drug treatment with systemic corticosteroids in combination with methotrexate has been reported to be beneficial in LS, but data in children is limited. Objectives To evaluate the efficacy and tolerability of systemic corticosteroids in combination with methotrexate in children with LS. Methods Treatment and outcome of 34 patients with LS were retrospectively analysed. Pulsed intravenous methylprednisolone was given, followed by oral prednisolone on a reducing regimen and maintenance treatment with methotrexate. We assessed treatment outcome clinically and by thermography and monitored adverse events. Results From the onset of treatment, the disease stopped progressing in 94% of the patients. All patients demonstrated significant clinical improvement within a mean time of 5.7 +/- 3.9 months. Mean duration of follow-up over the treatment period and beyond was 2.9 +/- 2.0 years. In 16 (47%) patients therapy was discontinued when the disease was considered to be inactive clinically; however, seven (44%) of the 16 developed a relapse, necessitating repeat treatment. At last follow-up (range 0.2-7.0 years), 24 (71%) of the 34 patients had completely inactive disease. Observed adverse events were moderate and transient and no patient had to stop therapy. Conclusions These data suggest that systemic corticosteroids and methotrexate in combination are beneficial and well tolerated in the treatment of children with LS. Because of the risk of relapse after discontinuing therapy, long-term monitoring is mandatory.

/pdf/evaluation-of-methotrexate-and-corticosteroids-for-the-3xi9chxsnw.pdf

Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children.

Objective. Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. 
Methods. A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. 
Results. Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. 
Conclusion. This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.

/pdf/juvenile-localized-scleroderma-clinical-and-epidemiological-5b5nezmvdw.pdf

S. K. Feitosa de Oliveira

Papers

Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study