Resistant dermatomyositis in a rural indigenous Maya woman

Abstract

A 28-year-old indigenous Guatemalan woman presented with 7 months of progressive weakness and numerous dermatological findings. She initially sought care within the free government-run health system and was treated with oral steroids for presumed dermatomyositis. Her symptoms progressed, including severe dysphagia, hypophonia and weakness preventing sitting. She was lost to follow-up in the public system due to financial and cultural barriers. A non-governmental organisation tailored to the needs of Maya patients provided home intravenous pulse dose methylprednisolone in the absence of first-line biologicals. With longitudinal home-based care, she achieved symptom free recovery. The rising burden of chronic non-communicable diseases highlights shortcomings in health systems evident in this case, including lack of provider capacity, limited infrastructure to test for and treat rare diseases and poor continuity of care. We provide potential solutions to help care delivery in low-resource settings adapt to the demans of chronic disease control with particular attention to social determinants of health.

Keywords: dermatology; global health; rheumatology.

Figure 1

Photo of facial dermatological findings at first visit with Maya Health Alliance approximately 7 months after symptom onset. Notable findings include diffuse hyperpigmentation (most notable on forehead), periorbital oedema and erythematous-violaceous eruption over eyelids (red arrows) consistent with heliotrope rash, which is more difficult to appreciate given darker complexion.

Figure 2

Patient profile used by crowdfunding partner Watsi (www.watsi.org) to fund patient’s treatment.