Juvenile localized scleroderma, also known as morphea, is the more frequent subtype
of scleroderma in childhood. It comprises a group of distinct conditions which involve
the skin and subcutaneous tissues. They range from very small plaques of fibrosis
involving only the skin, to diseases which may cause significant functional and cosmetic
deformity.
The most widely used classification divides JLS into five general types: circumscribed
morphea (CM), linear scleroderma, generalized morphea (GM), pansclerotic morphea and
the new mixed subtype where a combination of two or more of the previous subtypes
is present.
Circumscribed morphea (CM) is characterized by oval or round circumscribed areas of
induration surrounded by a violaceous halo.
When there are four or more plaques with individual plaques that are larger than 3
cm and they become confluent involving at least two out of seven anatomic sites (head-neck,
right upper extremity, left upper extremity, right lower extremity, left lower extremity,
anterior trunk, posterior trunk) it is called Generalized Morphea (GM).
Linear scleroderma, the most common subtype in children and adolescents, is characterized
by one or more linear streaks that can extend through the dermis, subcutaneous tissue,
and muscle to the underlying bone, causing significant deformities. The upper or lower
extremities can be affected but also the face or scalp, as in the en coup de sabre
variety (ECDS). The Parry Romberg syndrome (PRS), characterized by hemifacial atrophy
of the skin and tissue below the forehead, with mild or absent involvement of the
superficial skin is considered the severe end of the spectrum of ECDS and for this
reason is included in subtype of linear scleroderma
Pansclerotic morphea, an extremely rare but severe subtype, is characterized by generalized
full-thickness involvement of the skin of the trunk, extremities, face and scalp with
sparing of the fingertips and toes.
A recent multinational study reported that almost one fourth of the patients present
extra-cutaneous manifestations such as arthritis, neurological findings, associated
autoimmune conditions or ocular abnormalities. Antinuclear antibodies (ANA) are present
in more than 40% of patients with JLS.
The management of JLS is challenging and the detection of disease activity and progression
remains a fundamental problem. Clinical examination is subjective, classical skin
scoring methods, utilized in the assessment of systemic sclerosis, cannot be applied.
Among the new tools which have been proposed for the assessment of the skin lesions,
infrared thermography (IRT), computerized skin score (CSS), ultrasound (US) and magnetic
resonance imaging (MRI) are those most frequently used.
Over the years, many treatments have tried for localized scleroderma. Circumscribed
morphea generally is of cosmetic concern only, and therefore treatments with potentially
significant toxicity are not justified.
When there is a significant risk for disability, such as in linear and deep subtypes,
systemic treatment methotrexate (MTX) in combination with corticosteroids should be
considered.
Disclosure of interest
None declared.