S. Smithson, D. Orchard, S. Robertson, P. Bekhor, L. Scardamaglia
Mid-dermal elastolysis (MDE) is an uncommon condition of elastic tissue, of unknown aetiology. First described in 1977, it is characterised clinically by areas of wrinkled skin, and histologically by the absence of elastic ﬁbres in the mid-dermis.
A previously healthy 15 year old presented with a 5-month history of violaceous cutaneous plaques, with associated laxity of the skin, involving the bilateral groins and axillae. It was rapidly progressive, with associated malaise and nausea. Connective tissue and autoimmune screen was negative. Histopathology, with Verhoeff-Van Gieson stain, revealed a dermal inﬁltration by histiocytes and multinucleated giant cells, associated with elastophagocytosis and a reduction in elastic ﬁbres, particularly in the mid reticular dermis, in-keeping with the appearance of MDE.
The patient was commenced on methotrexate, with limited improvement. The disease progressed, with weight loss and increasing lethargy. The cutaneous laxity had a notable impact on the emotional well-being of the patient and psychological support was required. Over a course of 6 months, multiple immunosuppressive therapies were trialed in an attempt to the halt disease progression, including prednisolone, hydroxychloroquine and dapsone. Prednisolone was the only helpful agent, but carried a high side effect burden and could not be weaned below 15 mg. Due to the need for a steroid sparing agent mycophenolate mofetil was trialed. This offered good cutaneous disease control, and improved the overall symptoms, enabling a return to pre-morbid function. Due to the rarity of this disorder, no evidence-based treatment data exists for MDE. In our case, mycophenolate mofetil was successful.