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Friday, 10 November 2017 22:52

Neutrophilic dermatosis of the dorsal hands

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D Micallef, M Bonnici, *D Pisani, D Mintoff, *O Woods, M J Boffa; Department of Dermatology, Sir Paul Boffa Hospital, Malta & *Department of Pathology, Mater Dei Hospital, Malta

Neutrophilic dermatosis of the dorsal hands (NDDH) is an uncommon localised variant of Sweet syndrome characterised by tender erythematous plaques, pustules and bullae on the dorsae of the hands. In this presentation we report three new cases of NDDH in Malta. All patients were female, aged 60-82 years and presented with ulcerated violaceous plaques on the dorsae of the hands. The diagnosis was confirmed histologically in each case. Neutrophil leucocytosis was noted in all cases and two had raised inflammatory markers. None of the cases were associated with inflammatory diseases or malignancy and all resolved with corticosteroids. We performed a comprehensive literature review and found 94 cases of NDDH reported on PubMed. The mean patient age was 59 years and 60.5% were females. 84.0% of cases had reported bilateral involvement and other sites were affected in 31.9%. Underlying disease was found in a significant proportion, notably 13 cases of recent infection, 11 cases of haematological disorders (myeloproliferative disorders, myelodysplasia, or malignancies), 9 cases of active solid organ tumours and 3 patients with inflammatory bowel disease. Systemic and/or topical corticosteroids were employed in the treatment of 77.7% of cases while dapsone and colchicine were the commonest steroid-sparing agents used. All treatment regimens led to improvement which was often rapid and complete. While NDDH is uncommon, the fact that three cases were encountered locally over five years raises the possibility that the condition might actually be commoner than thought and may be misdiagnosed. Awareness of NDDH is important since a correct diagnosis would trigger a search for underlying diseases and proper treatment with corticosteroids and/or steroid-sparing agents.

 

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