PMC

Pansclerotic morphea affecting limbs circumferentially and sparing distal toes and fingers.

Teenage female with typical (A) sclerodactyly and (B) digital pitting and ulcers.

Typical isolated oval lesion on trunk consistent with superficial circumscribed morphea.

Expressionless facies of scleroderma. Pre-pubertal female with decreased oral aperture and ‘pursed lips’.

Dermal atrophy of LS, demonstrated by ‘cliff drop’ lesions in a teenage female with generalized morphea.

Severe example of Parry Romberg Syndrome/ hemifacial atrophy of the right face.

Parry Romberg Syndrome affecting left face before (A) and after (B) subcutaneous fat grafting.

Typical ‘lilac ring’ observed at the border of multiple plaque lesions of the trunk indicating active disease, courtesy of Elena Pope at Children’s Sick Kids, Toronto.

Waxy yellow center of plaque lesion remaining on right side of the neck years after the onset of localized scleroderma.

Linear lesion down trunk (A) and legs (B) of a toddler with sclerodermatous chronic graft versus host disease.

Typical shiny and thick skin of the hands with skin induration traveling up the forearm (past the metacarpal phalangeal joints) in a patient with systemic sclerosis.

Phases of localized scleroderma and associated features. Active disease features, erythema, skin induration/edema and new or enlarging lesions, occur first and give way to disease damage features, dyspigmentation, atrophy and skin thickening.

En coup de sabre (ECDS) lesion affecting scalp and head in a unilateral fashion (A) causing moderate amount of subcutaneous tissue loss and skeletal deformity (B).

Linear scleroderma lesion affecting unilateral right upper extremity (A). This young woman has associated joint contractures of the wrist, metacarpal and phalangeal joints. Joint contractures of the hand are grossly apparent compared to the contralateral side during active extension (B) and flexion (C).

Pediatric scleroderma divided into systemic and localized disease, which is further differentiated into subtypes based on clinical findings of skin involvement. Clinical subsets are related, with particular morbidities as mentioned.
ILD = interstitial lung disease; PAH = pulmonary arterial hypertension; ROM = range of motion