Pyostomatitis vegetans K12.15

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Pluriorificially localized stomatitis; Pyo-(Rhino-Blepharo-)Stomatitis vegetans

History
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Hallopeau, 1889

Definition
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Rare, non-infectious, inflammatory disease of the oral mucosa, in a few cases also of the genito anal region. There are often associations with inflammatory bowel diseases. Some authors regard it as a minimal variant of pemphigus vegetans, type Hallopeau. Other authors evaluate the disease as a variant of Pyoderma gangraenosum.

Etiopathogenesis
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Unsolved. In almost all cases there are associations with intestinal diseases ( ulcerative colitis, regional enteritis) or immune defects.

Manifestation
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Occurs mainly between the ages of 20 and 60. Clear emphasis on the male sex.

Localization
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Possible in the area of the entire oral cavity, especially the labial and buccal mucosa are affected. The back of the tongue is usually left out.

Clinical features
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Start with miliary pustules, confluence and ulceration on erythematous ground. Frequently the pustules are arranged in lines and serpentines, later a patch-like picture of the entire oral cavity with a layer of mucous membranes. Sometimes mild pain, sometimes severe pain.

Very often the clinical picture is correlated with chronic inflammatory bowel diseases such as Crohn's disease or ulcerative colitis.

Histology
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Acanthosis, parakeratosis, focal acantholysis. Neutrophilic, more rarely eosinophilic microabscesses. Mixed cell infiltrate in the lamina propria. No formation of granulomas.

Direct Immunofluorescence
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No or unspecific deposits.

Indirect immunofluorescence
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No circulating antibodies.

Differential diagnosis
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Therapy
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Therapy of a possibly underlying inflammatory bowel disease. Frequently, the mucous membrane changes heal only under systemic, low-dose steroid medication, possibly also under azathioprine.

External therapy
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Astringent stomatological products such as tormentillae astringents, chlorhexidine solution or dexpanthenol solution R255 R045 R066. In case of very painful erosions, solutions with anaesthetic additives such as Dolo-Dobendan solution, Acoin solution, periodontal ointment or Dynexan gel are suitable. Alternatively Ciclosporin A-adhesive paste(Ciclosporin A-adhesive paste 2.5%) can be tried.

Internal therapy
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Glucocorticoids such as prednisone (e.g. Decortin H) in medium-high dosage, initially 40-60 mg/day, gradual dose reduction according to clinic. Alternative therapy trial with Infliximab (e.g. Remicade) 5 mg/kg bw in week 1, 2 and 6 and Methotrexate 25 mg/week overlapping and as maintenance therapy.

Literature
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  1. Ballo FS et al (1989) Pyostomatitis vegetans. J Am Acad Dermatol 21: 381-387
  2. Bens G et al (2003) Successfull treatment with infiliximab and methotrexate of pyostomatitis vegetans associated with Crohn`s disease. Br J Dermatol 149: 181-184
  3. Hallopeau H (1889) Sur une nouvelle forme de dermatite pustuleuse chronique en foyers à progression excentrique. In: Congrès International de Dermatologie et de Syphiligraphie tenu à Paris (Ie session). Masson, Paris, S. 344-362
  4. Hallopeau H (1898) "Pyodermite végétante", its relation to dermatitis herpetiformis and pemphigus vegetans. Arch Dermatol Syphil 43: 289-306
  5. Hallopeau H (1898) Second Mittheilung on "Pyodermite végétante" (suppurative form of Neumann's disease). Arch Dermatol Syphil 45: 323-328
  6. Lobkowicz F et al (1991) Pyostomatitis vegetans. dermatologist 42: 92-95

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020