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extramammary Paget's disease

Etiology: - associated with underlying carcinoma in 25-50% of cases a) gastrointestinal carcinoma b) urogenital carcinoma Epidemiology: - 8% of all malignant skin cancers (presumably excluding BCC) Pathology: - dermatosis is itself a malignancy - not contiguous with underlying carcinoma * histopathology image [3] Genetics: - associated with defects in LITAF gene Clinical manifestations: 1) morphologically similar eruption to that of Paget's disease of the breast 2) erythematous, scaling weeping eczematous eruption 3) generally occurs in anogenital region * images [4] Complications: - can be fatal after metastasis to internal organs Management: - excision or destruction of dermatosis

Related

Paget's disease of the bone; osteitis deformans Paget's disease of the breast or nipple

General

paraneoplastic dermatosis

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 170-71
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17. American College of Physicians, Philadelphia 2009, 2012, 2015
  3. Sandhu N, Elston DM (histopathology image) Medscape: Extramammary Paget Disease http://emedicine.medscape.com/article/1100397-overview
  4. DermNet NZ. Extramammary Paget disease (images) http://www.dermnetnz.org/site-age-specific/extra-mammary-paget.html

Databases & Images

OMIM 167300 images related to extramammary Paget's disease