Generalized Scleroderma in Children

Pediatric morphea (localized scleroderma): Review of 136 patients

“…We were able to uncover at least 20 previously described pediatric patients whose initial morphea evolved into lung, heart, esophageal, renal, or cutaneous manifestations of progressive systemic sclerosis. 11,12,17,20,[28][29][30][31][32][33][34] Some of these patients have been described without individual details; those 14 patients (including ours) with further information are presented in Table V. In an international collaborative study, 750 pediatric patients with morphea were described by primarily rheumatologists at 70 centers.…”

The Dermatological Aspects of Collagen Diseases

“…In contrast hereto, the course of the illness in the diffuse form is progressive, in scleroderma for example lasting many years, particularly if there has been a long interval between the Raynaud-like attacks and t hi' development of the scle rosis (23,48,49), otherwise death may occur after 2-4 years, in diffuse scleroderma even after a shorter period.…”

“…2a, b) the main lesions are to be found on the fingers, hands, and face, the Slorck, The Dermatological Aspects of Collagen Diseases 211 feel arc rarely affected. The lesions arc as a rule combined with Raynaud-like artcriospasms, w hich either precede the lesions, may occur simultaneously or follow later on (48,49,58). Practically al ways the fingers show the characteristic deformation of the nails and atrophy of the terminal phalanx with ulcerations resembling rat biles; furthermore a stiffness due to edema, a sclerosis of the joint capsulae and contracture develop.…”

“…Combined with scleroderma diffusa, one often observes a sclerosis and corresponding clinical disturbances of the oesophagus and stomach, fibrosis of the lungs and of the joints (96), also pathologic changes in the heart and the kidneys (86,114,115,124). Particularly often vasomotor disturbances resembling a Raynaud symptom are seen in combination with sclerodactylia (23,48,49,86,97).…”

“…The prognosis with and without treatment may therefore be quoted as fair in the case of circumscribed scleroderma, good in morphea and bad in the diffuse type of scleroderma (18,19,20,41,49,114,115).…”