CD1A Rich Inflammatory Stage of Lichen Sclerosus Et Atrophicans Mimicking Mycosis Fungoides and Extramammary Paget’S Disease

1600-0560

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

We report a case of inflammatory stage vulvar lichen sclerosus et atrophicans (LSA) with histopathologic features simulating both patch stage mycosis fungoides (MF) and extramammary Paget’s disease. The patient is a 79-year-old woman with an erythematous plaque on her vulva. A punch biopsy was performed that revealed mild psoriasiform hyperplasia of the epidermis with spongiosis, marked interface vacuolar alteration, scattered individual necrotic keratinocytes and Civatte bodies. A band-like mixed inflammatory infiltrate was present in the papillary/upper dermis with prominent intraepidermal lymphocytes. Some intraepidermal lymphocytes showed atypical nuclear contours simulating the cells seen in MF. The presence of numerous intraepidermal epithelioid cells with a pagetoid distribution raised the possibility of extramammary Paget’s disease. A panel of immunohistochemical (IHC) stains showed a reactive lichenoid mixed population of lymphocytes with numerous intraepidermal Langerhans cells. This confirmed the diagnosis of the inflammatory stage of lichen sclerosus. The presence of prominent epithelioid Langerhans cells with activated small lymphocytes represents an immune response in LSA and could cause diagnostic confusion with neoplastic conditions. IHC staining can confirm cell type and rule out MF and extramammary Paget’s disease.

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