Pyostomatitis vegetans K12.15

H. Hallopeau, 1889

A very rare, noninfectious, inflammatory disease of the oral mucosa; in a few cases, it may also affect the genito-anal region. It is frequently associated with inflammatory bowel diseases (ulcerative colitis, Crohn’s disease). The entity has not been definitively established.

Some authors consider pyostomatitis vegetans to be a “mild form” of pemphigus vegetans, Hallopeau type.

Other authors classify the disease as a variant of pyoderma gangrenosum.

Still others regard it as a distinct systemic disease. 

Unclear. In nearly all cases (this should be a mandatory finding for confirming this diagnosis!), there are associations with chronic intestinal diseases (ulcerative colitis, regional enteritis). Immunodeficiencies appear to be frequently associated with this condition. 

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 deposits or nonspecific deposits (important differential diagnosis forpemphigus vegetans).

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.

Treatment of an underlying inflammatory bowel disease (e.g., ulcerative colitis).

Often, mucosal changes resolve only with low-dose systemic steroid therapy, and in some cases, with azathioprine as well. 

There is no clear consensusin the literature regarding the treatment protocol. The most important systemic medications have been corticosteroids (prednisolone, beclomethasone), anti-inflammatory drugs (sulfasalazine, mesalazine), monoclonal antibodies (infliximab, adalimumab), immunosuppressants (azathioprine, methotrexate), antibiotics (dapsone), or a combination of these active ingredients.

The medications most commonly used topically or as mouthwashes included betamethasone, dexamethasone, clobetasol propionate, triamcinolone acetonide, mometasone furoate, and hydrocortisone.

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.

A 17-year-old male patient had been experiencing clinical symptoms of dentitis on his skin and oral mucosa for 10 years. Clinically, the condition was characterized by plaques that were initially localized but later became widespread and recurrent. Over time, these lesions increased in both severity and extent, developing into ulcerated, crusted plaques. The skin lesions eventually spread to several areas of the body, including the face, chest, both axillae, upper back, buttocks, gluteal fold, and genitals (see Fig.).

In addition to the skin symptoms, the patient suffered from several systemic symptoms. These included decreased appetite, sleep disturbances, and gastrointestinal complaints, particularly constipation and painful bowel movements. He reported fresh rectal bleeding during bowel movements and changes in bowel habits. There were no urinary tract symptoms, joint pain, significant weight loss, vomiting, shortness of breath, or chest discomfort. His medical history revealed no chronic conditions such as tuberculosis, asthma, or diabetes mellitus.

Numerous verrucous, painless plaques were present on various parts of the body, including the cheeks, chest, armpits, upper back, buttocks, and gluteal cleft. The lesions on the buttocks were strikingly large, irregularly shaped, crusted, and appeared chronically inflamed. Nodular rashes were present on the upper chest, while the back showed atrophic scars and healed papular lesions. A crusted lesion was noted above the chin, and indented, non-irritating scars were visible on both cheeks. The face and back were marked by cystic acne scars. In addition, the arms and legs exhibited well-defined, red, ring-shaped lesions suggestive of a superficial fungal infection or an inflammatory dermatosis.

A general physical examination revealed an adolescent of average build who appeared clinically pale, raising suspicion of anemia. No lymphadenopathy. Vital signs were within normal limits. The patient was febrile at presentation. A rectal examination confirmed the patient’s complaint of painful bowel movements with fresh blood; however, it was noted that the blood was not mixed with the stool.

Laboratory: The hematological analysis revealed microcytic hypochromic anemia as well as a decreased mean corpuscular volume; mild lymphopenia; and a slightly reduced serum albumin level. Serum IgE: 1375 IU/mL. Serum ferritin level elevated at 248 ng/mL (consistent with a persistent inflammatory process).

Histology: Hyperkeratosis, parakeratosis, and widening of the rete ridges. Evidence of dermal fibrosis with lymphocytic infiltrate; no acantholysis, no eosinophilic microabscesses. (Case report from: Chrcanovic BR et al. 2024) 

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2. Bens G et al. (2003) Successful treatment with infliximab and methotrexate of pyostomatitis vegetans associated with Crohn’s disease. Br J Dermatol 149: 181-184
3. Chrcanovic BR et al. (2024) Pyodermatitis-pyostomatitis vegetans: a case report and systematic review focusing on oral involvement. Oral Maxillofac Surg 28:1405–1414.
4. Clark L et al. (2016). Pyostomatitis vegetans (PSV)–pyodermatitis vegetans (PDV): A clinicopathologic study of 7 cases at a tertiary referral center. Journal of the American Academy of Dermatology 75: 578–584
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