A complex inflammatory triad: a rare case of parryromberg syndrome with systemic lupus erythematosus and thyroiditis

Abstract

Parry-Romberg Syndrome (PRS) is a rare disorder characterized by progressive unilateral facial atrophy, traditionally viewed as a localized scleroderma variant. Its rare coexistence with systemic lupus erythematosus (SLE) and autoimmune thyroiditis (AIT) suggests a broader inflammatory etiology, challenging the notion of PRS as a purely localized condition. A 31-year-old woman with a history of AIT presented with progressive right-sided hemifacial atrophy. During pregnancy, she developed systemic symptoms, including fever, dyspnea, generalized rash, and severe anemia, prompting hospitalization. Laboratory and histopathological findings confirmed an overlap of PRS, SLE, and AIT, with pancytopenia and chronic hepatitis. Treatment involved azathioprine, hydroxychloroquine, low-dose corticosteroids, and levothyroxine, with close monitoring of hematologic, inflammatory, and hepatic markers. The patient reported improved quality of life with counselling and medical management. This case highlights PRS as a systemic inflammatory condition, supported by serological, histopathological, and clinical findings. Shared Th17-mediated inflammatory pathways link PRS, SLE, and AIT, guiding targeted immunosuppressive therapies. Pancytopenia and hepatic involvement reflect systemic inflammation, necessitating a broad diagnostic approach. The rarity of PRS limits large-scale studies, but multicenter registries could elucidate its pathophysiology and optimize treatment strategies. PRS may involve systemic inflammation beyond facial atrophy, requiring comprehensive evaluation and tailored immunosuppressive therapies. Multidisciplinary collaboration is essential for managing its complex manifestations. Vigilance for multi-system autoimmune overlap in PRS patients is crucial, along with screening for associated conditions and long-term follow-up to monitor progression and response to therapy. Further research is needed to explore inflammatory mechanisms and develop standardized therapeutic approaches

Bangladesh J Medicine 2026; Vol. 37, No. 2(1) Supplementation: 237-238