Netherton syndrome is a rare, severe genetic disorder of cornification without specific treatment. We describe two cases who demonstrated marked cutaneous improvement with secukinumab and suggest a role for IL-17 therapy in treating this condition.
Lymphocytic thrombophilic arteritis and livedoid vasculopathy may both present with livedo racemosa and ulceration. We present 6 cases with features of both conditions, raising the possibility that they are either closely linked or are part of a spectrum of the same condition.
A man in his 70s with background vascular disease presented with 7 months of painful non-resolving lower leg ulcers with eschar and petechiae, left lower ear lobe ulceration and dusky inflammation of the right ear. He demonstrated good bilateral pedal pulses and no peripheral oedema. No lymphadenopathy was palpated.Biopsy suggested leucocytoclastic vasculitis on chronic stasis changes. Blood investigations showed elevated rheumatoid factor and mixed polyclonal IgG and monoclonal IgM cryoglobulins. He was diagnosed with mixed cryoglobulinaemia, and consequent conducted flow cytometry revealed CD5 +marginal zone lymphoma with elevated serum free light chains and kappa/lambda ratio.One-month following rituximab and chlorambucil therapy, the patient’s pain had much improved, ear ulcers had healed and several leg ulcers had reduced in width and depth. The petechial eruption had also resolved.
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