Generalised Panniculitis as a Post-COVID-19 Presentation in a Child with Aicardi-Goutières Syndrome

ABSTRACT

A 5-year-old girl was admitted with a 3-week history of cutaneous changes affecting her face and upper limbs. There was recent exposure to COVID-19 with an affected family member at home, but our patient did not have a history of respiratory illness or fever. She was born to consanguineous parents and had a presumed diagnosis of Aicardi-Goutières Syndrome (AGS), which had been confirmed in her older sibling with identical phenotype. Physical examination revealed dusky areas of induration involving the face, bilateral forearms and hands with associated oedema. With throat swabs negative for SARS-CoV-2 and no clear cause for the rash, the most likely explanation was thought to be a post-COVID-19 phenomenon. Our suspicion was confirmed by the presence of SARS-Cov-2 IgG antibodies. Histological examination demonstrated panniculitis with a predominantly lymphocytic infiltrate. As the skin changes continued to extend, the patient was commenced on prednisolone 20 mg once daily with good response. AGS is a hereditary early-onset encephalopathy and characterised by upregulation of type-1-interferon (IFN) activity, a key player in the host immune response against viral infections (1). We suggest that our patient developed an entirely unique, COVID-19 triggered, prolonged auto-inflammatory, type-1-IFN driven response, resulting from a previously unreported combination of AGS and COVID-19 infection, leading to her generalised panniculitis. Recent studies have implicated type-1-IFN signalling in the pathogenesis of COVID-19 associated perniosis and a favourable outcome (2). Upregulation of this pathway was clearly protective in our patient. References 1. Crow Y, Manel N. Aicardi-Goutières syndrome and the type I interferonopathies. Nat Rev Immunol 2015 15, 429-440. 2. Magro C, Mulvey JJ, Laurence J, et al. The differing pathophysiologies that underlie COVID-19 associated perniosis and thrombotic retiform purpura A case series [published online ahead of print, 2020 Jul 22]. Br J Dermatol. 2020.