Case 30 Clinical information supplied

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Case 30Clinical information supplied

• 25 year old male patient with Crohns disease for
  4 years.
• Six week history of oral ulceration with
  cobblestoning of oral mucosa and pustules
  (confluent ) on the lips.
• Associated with confluent pustular vegetative
  plaque which is mildly exudative in the groin
  bilaterally and perineum.
• Nil axillary
• History of eye inflammation.
• Increased eosinophils on FBC. Recently on
  Infliximab until 8/52

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Ancillary investigations

• PAS neg
• IMF - neg

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Pyostomatitis vegetans

• Admitted to dermatology ward
• Oral steroids and steroid mouth wash
• Improved
• Had a similar flare 2012.
• Crohns symptoms deteriorating until started
  steroids
• ? Need to review Crohns treatment
• Currently on mesalazine
• Adalumimab and AZA failed

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Pyostomatits vegetans

• Pyodermatitis-pyostomatitis vegetans is a rare
  but characteristic pustular eruption of the mouth
  and skin folds consistently associated with
  inflammatory bowel diseases such as ulcerative
  colitis and Crohns
• The skin disease is pyodermatitis vegetans and
  the mucosal disease is pyostomatitis vegetans.

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Pyostomatitis vegetans

• The inflammatory bowel disease usually precedes
  the development of the skin and/or mouth problem
  by months or years. Sometimes however the bowel
  problem is only diagnosed when it is looked for
  after the skin/mouth diagnosis.
• Age group 20-50 years.
• It is more common in males than females (31).

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Pyostomatitis vegetans

• The typical features of pyodermatitis vegetans
  include
• redness
• pustules
• crusting
• large raised (vegetating) plaques
• Brown post-inflammatory pigmentation is
  prominent.
• In the mouth, pyostomatitis vegetans consists of
  redness with multiple yellow or white pustules.
  The pustules (microabscesses) easily rupture, to
  form superficial ulcers (erosions) which have
  been likened to snail trails.
• Signs in the lining of the mouth in pyostomatitis
  vegetans include
• swelling and thickening
• cobblestoned appearance
• raised growths (vegetations)

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Pyostomatitis vegetans

• On routine pathology, pustules (microabscesses)
  either within or just below the epidermis will be
  seen, typically with many eosinophils (early
  lesions) and/or neutrophils (late lesions).
  Granulomas do not occur. Direct immunofluoresence
  is negative or only weakly positive.
• On blood tests, indirect immunofluorescence is
  negative, i.e., there are no skin antibodies
  circulating. The blood count almost always
  reveals an increased number of eosinophils. Liver
  abnormalities have been reported so these should
  also be tested for routinely.
• The diagnosis of pyodermatitis-pyostomatitis
  vegetans is therefore made on the combination of
• clinical presentation
• histological features
• negative direct and indirect immunofluorescence
• eosinophilia on blood test
• exclusion of infection.

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