Diffuse cutaneous systemic sclerosis: A case report

ABSTRACT

BACKGROUND: Systemic sclerosis (SSc) is a rare, connective tissue  disease  with  multisystem involvement.This is due to immunological processes,vascular endothelial cell injury  and extensive activation of fibrolast that commonly affects the skin and other internal organs such as the esophagus, lungs, heart, and kidneys. SSc has one of the highest mortality among  the  autoimmune  rheumatic  diseases,  hence  the  emphasis  on  the  early  recognition  and  management  to  prevent significant progression of the disease. CASE A 22-year-old  female  presented  with  a  one-year history of multiple hard and hypopigmented patches on the face, neck, trunk and upper extremities. Further examination revealed mask-like facies, microstomia, frenulum sclerosis, Raynaud's phenomenon, pitted scars on the digital pulp of hands  and  sclerodactyly.Baseline  blood  chemistry,chest radiograph and electrocardiography  were  all  negative  for systemic involvement. Autoantibodies were positive for dsDNA, SS-A/Ro and Scl-70. Skin biopsy revealed sclerosing dermatitis, which was consistent with SSc. OUTCOME: The  patient  was  initially  started  with  oral prednisone 0.5 mg/kg/day and was increased to 0.75 mg/kg/day for eight weeks. Prednisone was slowly tapered to 5.0 mg/day and methotrexate 15.0 mg/week was included in  the  management for eight weeks which resulted in decreased joint pains, halted the progression of skin induration, decreased in pruritus and palmar edema. CONCLUSION: The characteristic dermatological findings of SSc are not only important signs to dermatologists, but these serves as diagnostic clues for clinicians from other disciplines as well. In our case, the presence of the autoantibody Scl-70 indicated the potential risk of pulmonary fibrosis and pulmonary arterial hypertension that accounts with high mortality.Hence,physicians should be aware of the possible risk of organ damage,even when asymptomatic because there is a high risk of disease progression. The importance of early recognition and a multidisciplinary approach lead to the good outcome in this case.