Diagnosis and management of morphoea in children: an overview

Abstract

Summary Paediatric morphoea (localized scleroderma) is an inflammatory sclerosing disorder of the skin and subcutis associated with tissue atrophy. It is thought that the disease develops on the background of genetic predisposition (e.g. mosaicism for the common linear variant) initiated by various trigger factors, and that detected autoantibodies and inflammatory cytokines represent secondary epiphenomena. In contrast to the common belief that morphoea is a benign self‐limiting disorder, long‐term data indicate that its chronicity, relapsing nature and extracutaneous complications lead to significant morbidity, particularly when the disease starts in early childhood. Early recognition may be challenging, and the most important clinical clues are band‐like distribution, atrophy of underlying tissue, skin sclerosis, and localized loss of body/scalp hair, eyelashes or eyebrows. Extracutaneous manifestations occur in up to 20% of patients, with arthritis/arthralgia and neurological symptoms being most frequently observed, followed by ophthalmological complications such as uveitis. Corticosteroids and methotrexate are highly effective as first‐line therapy in morphoea, leading to partial reversal of skin manifestations. However, the development of atrophy is not sufficiently prevented by standard therapy. There is a relapse rate of 25%–48% within the first years after stopping treatment, thus long‐term follow‐up is warranted. Mycophenolate mofetil seems to be a beneficial second‐line therapy, and a new drug, abatacept, also seems to be a promising and well‐tolerated second‐line treatment option. Additionally, autologous fat injections are beneficial and may be used as an adjunct to ongoing therapy.

A Case of Linear Scleroderma in a Young Child: A Diagnosis Easily Missed in Primary Care

“…Certain factors increase the likelihood of relapse, such as the presence of ANA, delayed initiation of therapy, and the subtype of generalized scleroderma. Some patients experience extended periods of inactivity followed by activation, requiring long-term follow-up [ 14 ].…”

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: Localized scleroderma, systemic sclerosis and overlap syndromes

“…Nevertheless, relapses of LS following treatment have been reported in 25%-50% of patients. 16,56,106 Age at onset and extracutaneous involvement of disease is the most important risk factor for recurrent disease, and relapses occurred significantly more often in paediatric (27%) compared to adult (17%) patients with LS. 32 Disease subtype (generalized or mixed type of LS) as well as ANA positivity are other risk factors for recurrences.…”

“…arthritis) and disease relapse. 5,15,16 In the active stage of generalized LS, blood eosinophilia may be observed. 17,18 In patients with linear LS of the extremities with concomitant joint involvement, increased levels of rheumatoid factor may be present and do sometimes correlate with the clinical degree of arthritis activity.…”

“…migraine, headaches and eventually epilepsy). 12,16,[21][22][23][24][25][26] Cranial magnetic resonance imaging (MRI) is recommended to detect potential subcortical calcifications or brain atrophy. 21,[27][28][29] On the other hand, many patients are asymptomatic even if such abnormalities are seen.…”

Eyebrow and Eyelash Alopecia: A Clinical Review

“…Localized scleroderma is a disorder of excessive collagen deposition that can present as unilateral atrophy of the frontoparietal region above the eyebrow. Known as “ en coup de sabre ” (French for “the blow of the sword”) for its resemblance to the scar of a sword wound, linear morphea of the paramedian forehead and scalp can be accompanied by eyebrow depression and hair loss [ 38 ]. In a case review of 50 pediatric patients with localized scleroderma, eyebrow and eyelash loss occurred in 4% and 12% of patients, respectively [ 39 ].…”