DP10 A relapsing–remitting rash with associated neurological symptoms: a rare presentation of ​­neuro-Sweet syndrome

Abstract

Abstract A 46-year-old woman presented with an 8-week history of an asymptomatic eruption on her upper extremities and back. She had a history of ulcerative colitis, asthma and hyposplenism. There had been no changes to her regular medications. Over the next 3 years, she developed a relapsing and remitting course of headaches, pyrexia and feeling unwell with the associated rash. She further developed deafness and vertigo and was admitted with headaches, blurred vision and paraesthesia of the fingers. Physical examination revealed multiple erythematous, asymptomatic, annular plaques present on her upper extremities and back with no oral, genital or ophthalmological lesions. Skin biopsy from the lesion on the upper back showed a neutrophilic dermatosis with mildly acanthotic epidermis, superficial dermal collection of acute inflammatory cells, dermal oedema and endothelial swelling, with no evidence of vasculitis. This was considered in keeping with Sweet syndrome. Magnetic resonance imaging of the brain revealed meningoencephalitis and focal cerebritis. A lumbar puncture performed for infection was negative. Following this, a diagnosis of neuro-Sweet syndrome (NSS) was made. She has responded well to oral steroids and is currently well controlled on dapsone. To date, extensive malignancy screens have been negative. Sweet syndrome, or acute febrile neutrophilic dermatosis, is characterized by fever, malaise, leucocytosis and painful distinctive skin lesions and may be associated with malignancies, drugs and infection. Skin biopsy shows dense dermal infiltration of neutrophils without any histological signs of vasculitis. Sweet syndrome is known to involve multiple organs; however, involvement of the central nervous system (CNS) is rare. When the CNS is involved, it is referred to as NSS and is defined as the coexistence of characteristic skin lesions of Sweet syndrome with encephalitis or meningitis (Suh HB, Kim HJ. Recurrent neuro-Sweet disease associated with preceding upper respiratory infection: a case study. iMRI 2018; 22:187–93). Although the condition is usually steroid responsive, disease recurrences have been reported in 50% of cases in the literature (Hisanaga K, Iwasaki Y, Itoyama Y, Neuro-Sweet Study Group. Neuro-Sweet disease: clinical manifestations and criteria for diagnosis. Neurology 2005; 64:1756–61). It is important to note that the clinical features and imaging results in NSS are similar to those of neuro-Behçet disease (NBD), and it is important to distinguish between the two as CNS involvement in NSS is often transient, whereas NBD often follows a more progressive course. We report a rare case of NSS and highlight that NSS should be considered by dermatologists in patients presenting with recurrent skin lesions of Sweet syndrome and associated neurological symptoms.