Diffuse systemic sclerosis on a young primipara patient

Authors: Marlon R. Aljam, Benedicto dL. Carpio, Eileen Regalado-Morales, Armelia Lapitan-Torres

Abstract

Introduction: Scleroderma is an autoimmune disease usually affecting women from 30s to 50s due to inflammation, changes in vasculature, and connective tissue-producing cells. Environment and chemical factors could be triggers, however, fetal microchimerism can also initiate scleroderma.

Case Summary: A patient in early twenties had three attempts before giving birth last July 2012. She noted facial and nape hyperpigmentation during her last pregnancy which did not resolve post-partum. On 2013, patient started to notice tightening of skin on both
arms. She was initially diagnosed with scleroderma and was managed with appropriate medications but she was non-compliant. Skin hyperpigmentation continued to spread in a centripetal manner, while skin hardening spread in the opposite direction. The past 2 years saw acceleration of disease process, and Raynaud’s phenomenon, sclerodactyly, weight loss, microstomia, dysphagia, dyspnea, abdominal pain and insomnia became evident. Diagnostics elicited SCL70 antibody at >200 (normal=<15). ESR (109 mm/hr, normal=42 mm/hr), CK-MM (151.22, normal=<115), CK-MB (183.82, normal=30-135) and CK Total (32.60, normal=0-16) were also elevated. These collectively suggest diffuse systemic sclerosis. Currently, her management includes vitamin D3 twice weekly and
beraprost 20 mg/tablet 1 tablet 2 times a day.

Conclusion: The timing of the presentation of initial signs of diffuse systemic sclerosis after childbirth highly suggests fetal microchimerism as a critical factor that may trigger
an autoimmune cascade leading to a full blown development of a rare disease like scleroderma.

 

Citation

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Keywords

Diffuse systemic sclerosis, scleroderma, diffuse scleroderma, primipara,
pregnancy, child-bearing years, young

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