Pyostomatitis vegetans K12.15

Hallopeau, 1889

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.

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

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

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.

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.

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

No or unspecific deposits.

No circulating antibodies.

Pemphigus vegetans, type Hallopeau: histologic and immunohistologic clarification

Amicrobial intertriginous pustulosis: intertriginous localization preferentially in women, usually in the context of an autoimmune disease.

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.

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.

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.

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, pp. 344-362
4. Hallopeau H (1898) "Pyodermite végétante", its relations to dermatitis herpetiformis and pemphigus vegetans. Arch Dermatol Syphil 43: 289-306
5. Hallopeau H (1898) Second communication 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
7. Nusbaum KB et al (2020) Pyostomatitis vegetans with extensive tongue involvement. Int J Dermatol 59:e263-e265.