Ref Number = PITIKA-ASPR0199
Galuh Hardaningsih, Dina Muktiarti, Nia Kurniati
Scleroderma is a complex cutaneous disease which is associated with inflammation, vasculopathy and fibrosis as well as extracutaneous organ involvement. Clinical manifestations depend on diffuse deposition of collagen in the skin and internal organs with vasculat injury and immunologic abnormalities. Treatment ids directed at controlling inflammation and manage specific skin and other organ problems.

To report a Scleroderma case due in a-6 1/2 year old-girl

Case report
A 6½ old girl was sent to Immunolgy Outpatient Clinic for exploration of possible Cutaneous Lupus diagnosis. She was other wise a normal girl up to 2 years before consultation, when she suffered from unspecified bone pain and recurrent prolonged fever. There was gradually changes on the skin around her lower jaw, chin and around her left ear that extended to her left armpit, left hand up to her fingers. There were also similar changes on both lower legs. She has no difficulty of chewing or opening her mouth, but we can see her mandible was small comparing to her facial structure. The discoloration on the skin accompanied by hyperpigmentation surrounding the lesion and the skin appeared smooth and shiny; it was more firm compared to surrounding muscles. The lesion on her leg were more linear, with subcutaneous atrophy without raised boulder specific for cutaneous lupus. We also didn’t find other specific lupus manifestation. Her laboratory evaluation showed us raised leucocyte level, positive ANA serum level at >1/1000 with speckled pattern, Ig-anti dsDNA was negative, normal level of C3 and C4, also negative Rheumatoid Factor. We asked for a skin biopsy, and the results did not support lupus diagnosis. Based on all above data we determined a scleroderma diagnosis. She was given a combination of Methotrexate (weekly 10-15 mg/m2) and methylprednisolone (daily1-2 mg/KgBW). After 2 weeks the fever diminished and we didn’t find new or extending lesion; we stopped the steroid and continue with methotrexate only. The patient still have to do physiotherapy to overcome her tight skin and to ensure maintaining her muscles movement.

The best way to manage scleroderma is to use multiple modalities; from steroid to methotrexate and from drug to rehabilitation therapy. The goal is to achieve highest quality of life
Keywords: Scleroderma, multiple modalities, quality of life
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