Pansclerotic morphea in childhood: a case report

Scleroderma can be differentiated into progressive systemic and circumscript forms. The extensive form with lethal outcome is known from case reports of children and adolescents. The present case report concerns a boy who died at 16 years of age. In the 5th year of life, he experienced weight loss and developed multiple, firm, partially atrophic plaques in the skin of the extremities. These plaques gradually became confluent and extended over the whole torso and head. Plaque ulceration resulted in massive mutilations to the body. Later the patient's cachexia worsened and he developed keratose, moderately differentiated squamous cell carcinoma of the right leg. The prognosis of pansclerotic morphea for children is worse than for adults. No successful therapy is known.