Pyodermaiis-pyostomaiis vegetans What is pyodermaiis-pyostomaiis vegetans? Pyodermaiis-pyostomaiis vegetans is a rare but characterisic pustular erupion of the mouth and skin folds consistently associated with inlammatory bowel diseases such as ulceraive coliis and less commonly Crohn disease.
The skin disease is pyodermaiis vegetans and the mucosal disease is pyostomaiis vegetans. Who gets pyodermaiis-pyostomaiis vegetans? Pyodermaiis-pyostomaiis vegetans is almost always seen in paients with ulceraive coliis or Crohn disease. The inlammatory bowel disease usually precedes the development of the skin and/or mouth problem by months or years. Someimes however the bowel problem is only diagnosed when it is looked for ater the skin/mouth diagnosis.
This condiion usually develops in the age group 20-50 years although it has been rarely reported in children. It is more common in males than females (3:1). Clinical features of pyodermaiis-pyostomaiis vegetans The characterisic lesion in pyodermaiis-pyostomaiis vegetans is the pustule.
On the skin, pyodermaiis vegetans presents as an asymmetrical rash most commonly in skin folds (a form of intertrigo), such as the armpits and groin, and the scalp. Less commonly the trunk, face and ingers/toes may be involved.
The typical features of pyodermaiis vegetans include:
redness pustules crusing large raised (vegetaing) plaques Brown post-inlammatory pigmentaion is prominent.
In the mouth, pyostomaiis vegetans consists of redness with muliple yellow or white pustules. The pustules (microabscesses) easily rupture, to form supericial ulcers (erosions) which have been likened to snail trails.
Signs in the lining of the mouth in pyostomaiis vegetans include:
swelling and thickening cobblestoned appearance raised growths (vegetaions)
All sites within the mouth and throat can be afected, except the tongue and loor of the mouth are rarely involved. Vegetaions tend to develop in areas of redness and occur most commonly on the gums and palate. On the inside of the lips and cheeks, swelling can cause the surface to become folded. Pain is variable and may be surprisingly mild. The lymph glands under the chin may be enlarged.
Other mucosal sites have been less commonly reported to be afected including the vulva, nose and eyelids.
The erupion can develop and progress very quickly, over days.
It is possible to have just the skin erupion or just the mouth erupion; both sites are not always afected. Usually the mouth lesions occur irst, or at the same ime as the skin is afected.
The bowel disease may be asymptomaic or cause such mild symptoms that it has not been previously diagnosed. In paients already known to have inlammatory bowel disease, the development of pyodermaiis-pyostomaiis vegetans may coincide with a lare in the bowel disease. How is pyodermaiis-pyostomaiis vegetans diagnosed? Skin and/or mucosal biopsies are required for rouine histology and direct immunoluorescence (anibody test) to disinguish pyodermaiis-pyostomaiis vegetans from pemphigus vulgaris, Hallopeau type (pemphigus vegetans). On rouine 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 immunoluoresence is negaive or only weakly posiive.
On blood tests, indirect immunoluorescence is negaive, i.e., there are no skin anibodies circulaing. The blood count almost always reveals an increased number of eosinophils. Liver abnormaliies have been reported so these should also be tested for rouinely.
Microbiological swabs for bacteria, viruses (herpes simplex), yeasts (candida) and fungi will show only normal lora. Swabs are important to perform to exclude infecion especially when immunosuppressive therapy is planned.
The diagnosis of pyodermaiis-pyostomaiis vegetans is therefore made on the combinaion of:
clinical presentaion histological features negaive direct and indirect immunoluorescence eosinophilia on blood test, and exclusion of infecion.
When a diagnosis of pyodermaiis-pyostomaiis vegetans is made, further invesigaion for associated inlammatory bowel disease should be performed in paients not already known to have this. This will usually involve at least a colonoscopy and bowel biopsy. Delayed diagnosis of typical inlammatory bowel disease up to two years ater the mouth condiion has been reported and therefore coninued monitoring should be considered in those cases where endoscopy is iniially reported to be normal. Treatment of pyodermaiis-pyostomaiis vegetans Treatment is mainly aimed at controlling the bowel disease as then the skin and mouth lesions will also resolve.
In those rare cases where no bowel disease is found, topical treatment opions may include:
anisepic mouthwashes topical steroids tacrolimus ointment.
Systemic treatments may include:
oral coricosteroids azathioprine dapsone ciclosporin sulfasalazine, mesalazine, sulfamethoxypyridazine oral or intramuscular methotrexate
inliximab infusions
Nutriional supplements may also be required because the bowel disease may cause malabsorpion. In one report, zinc supplements lead to improvement of the mouth lesions.
In severe cases, colectomy for the bowel disease has resulted in resoluion of the mouth and skin lesions.
Vegetans Pyodermatitis-pyostomatitis Apa itu pyodermatitis-pyostomatitis vegetans? Vegetans Pyodermatitis-pyostomatitis adalah jarang tetapi karakteristik letusan berjerawat lipatan kulit dan mulut secara konsisten terkait dengan penyakit inflamasi usus seperti kolitis ulserativa dan kurang sering Crohn penyakit. Penyakit kulit pyodermatitis vegetans dan penyakit mukosa pyostomatitis vegetans. Siapa yang mendapat vegetans pyodermatitis-pyostomatitis? Vegetans Pyodermatitis-pyostomatitis hampir selalu terlihat pada pasien dengan penyakit Crohn atau kolitis ulserativa. Penyakit inflamasi usus biasanya mendahului perkembangan masalah kulit dan/atau mulut oleh bulan atau tahun. Kadang-kadang namun masalah usus adalah hanya didiagnosis ketika mencari setelah diagnosis kulit mulut. Kondisi ini biasanya berkembang dalam kelompok usia 20-50 tahun meskipun telah jarang dilaporkan pada anak-anak. Hal ini lebih umum pada laki-laki daripada perempuan (3:1). Fitur klinis dari pyodermatitis-pyostomatitis vegetans Lesi khas di pyodermatitis-pyostomatitis vegetans adalah pustule. Pada kulit, pyodermatitis vegetans menyajikan sebagai ruam asimetris paling sering di (bentuk intertrigo), lipatan kulit seperti ketiak, selangkangan dan kulit kepala. Kurang sering batang, wajah dan jari jari-jari mungkin terlibat. Fitur khas dari pyodermatitis vegetans termasuk: kemerahan pustula pengerasan kulit besar mengangkat plak (vegetating) Pigmentasi pasca inflamasi cokelat terkemuka. Dalam mulut, pyostomatitis vegetans terdiri dari kemerahan dengan beberapa pustula kuning atau putih. Pustula (microabscesses) dengan mudah pecah, untuk membentuk dangkal borok (erosi) yang telah disamakan dengan jejak siput. Tanda-tanda di dinding mulut dalam pyostomatitis vegetans meliputi: pembengkakan dan penebalan penampilan cobblestoned
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