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Severe perianal ulceration

BMJ 2010; 340 doi: http://dx.doi.org/10.1136/bmj.c2157 (Published 26 May 2010) Cite this as: BMJ 2010;340:c2157
  1. Naomi Kate Webber, specialist registrar, dermatology1,
  2. Alistair Robson, consultant dermatopathologist1,
  3. Eithne Macmahon, consultant virologist2,
  4. Ann-Marie Powell, consultant dermatologist1
  1. 1St John’s Institute of Dermatology, Guy’s and St Thomas’ Foundation Trust, London SE1 7EH
  2. 2Department of Virology, Guy’s and St Thomas’ Foundation Trust
  1. Correspondence to: N K Webber naomi.webber{at}doctors.org.uk

    A 34 year old woman presented to the dermatologists with a five month history of severe perianal and vulval ulceration associated with debilitating pain. She had no vaginal discharge. She was referred by the gastroenterologists who were investigating her for recurrent episodic diarrhoea. Her medical history included end stage renal failure, deep vein thrombosis, and cerebrovascular accident secondary to systemic lupus erythematosus. Her treatments included prednisolone 7.5 mg once daily, mycophenolate mofetil 500 mg twice daily, warfarin, and haemodialysis. She had not been sexually active in recent years.

    Examination showed extensive discrete areas of perianal ulceration extending to the posterior labia majora that were painful to touch (fig 1). She had no abnormality of the hair, nails, oral mucosa, or skin elsewhere.

    Fig 1 Perianal ulceration extending to the posterior labia majora

    In addition to profound renal impairment and positive lupus serology, blood tests showed haemoglobin of 7.9 g/l (normal range 130-180) and lymphocyte count of 0.5×109/l (1.2-3.5). HIV-1 and HIV-2 antigen and antibody tests were negative.

    Questions

    • 1 What are the differential diagnoses of perianal ulceration and the likely diagnosis in this patient?

    • 2 What investigations would you carry out to confirm the diagnosis?

    • 3 How would you treat this condition?

    Answers

    1 What are the differential diagnoses and the likely diagnosis in this patient?

    Short answer

    Differential diagnoses are herpes simplex infection (HSV) of the genitals, tuberculosis, and infection with atypical mycobacteria. Other possibilities include inflammatory dermatoses such as metastatic Crohn’s disease; pyoderma gangrenosum; vasculitis; lichen planus; Behçet’s syndrome; autoimmune blistering disease, such as pemphigus vulgaris; and drug reactions. The history of lymphopenia and immunocompromise make HSV the most likely …

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