Mucocutaneous lesions of Beh�et�s disease

Alpsoy, Erkan

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Mucocutaneous lesions of Beh�et�s disease [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2003; 37(2): 92-99

Mucocutaneous lesions of Beh�et�s disease

Erkan Alpsoy¹

Beh�et's disease is a chronic, relapsing, multi-systemic inflammatory process with the clinical features of mucocutaneous lesions and ocular, articular as well as further miscellaneous organ involvement. Basic pathologic process of the disease is vasculitis. Because of the lack of a universally recognized pathognomonic laboratory test, the diagnosis of the disease is primarily based on clinical criteria. Various criteria have been used, and all of them rely heavily on mucocutaneous manifestations in particular oral and genital ulcers, cutaneous vasculitic lesions and the pathergy test. Recurrent and painful ulcers of the oral and genital mucosa in patients with Beh�et's disease are identical to aphthae in appearance, but they tend to be more frequent and occur in crops. The cutaneous lesions of the disease are varied and include erythema nodosum-like lesions, papulopustular lesions, superficial thrombophlebitis, extragenital ulcerations, reactivity of the skin to injection (pathergy reaction) and other cutaneous vasculitic lesions e.g. Sweet's syndrome-like, pyoderma gangrenosum-like, erythema multiforme-like lesions, palpable purpura, subungual infarctions, hemorrhagic bullae, furuncles, and abscesses. Mucocutaneous lesions constitute the hallmark of Beh�et's disease. The high frequency of oral and genital ulcers and cutaneous lesions at any stage in the course of the disease, or as onset signs confirm the importance of these clinical features for the diagnosis. Oral and genital ulcers, cutaneous lesions and ocular lesions together with arthropathy are the most frequent features of the disease, and erythema nodosum-like lesions and papulopustular lesions are the most commonly observed cutaneous lesions in all countries. Oral ulcers represent the onset feature of the disease in the majority of the patients worldwide. Genital ulcers and cutaneous lesions, especially erythema nodosum-like lesions, may also occur as onset lesions. In the majority of patients, mucocutaneous lesions appear before potentially severe organ involvement occur. Therefore, familiarity with the cutaneous spectrum is mandatory for early diagnosis which would prevent the patients from most of the hazardous results of Beh�et�s disease.

Keywords: Beh�et's disease, mucocutaneous lesions, review

Beh�et hastal��n�n deri ve mukoza belirtileri

Erkan Alpsoy¹
Akdeniz �niversitesi T�p Fak�ltesi Deri ve Z�hrevi Hastal�klar Anabilim Dal�

Beh�et hastal��, deri ve mukoza belirtilerinin yan�nda g�z ve eklemler ba�ta olmak �zere �ok say�da organ� tutabilme �zelli�i g�steren sistemik bir hastal�kt�r. Temel patolojisi vask�lit olan hastal�k, tekrarlayan ataklarla kronik bir seyir izlemektedir. Kesin tan� koydurucu laboratuvar bulgular� olmad��ndan tan� klinik bulgulara dayanmaktad�r. Bu ama�la kullan�lan de�i�ik tan� kriterlerinin ortak �zelli�i, hastal��n tan�s�n�n deri ve mukoza belirtileri ve �zellikle de oral ve genital �lserler, deri belirtileri ve paterji testi pozitifli�inin �zerine kurgulanmas�d�r. A�r�l� ve yineleyici �zellikteki oral ve genital �lserler, klinik olarak rek�rren aft�z stomatit ile benzer olmakla birlikte daha s�k tekrarlama ve �ok say�da olma e�ilimi g�sterir. Deri belirtileri de�i�ken olup, en s�k eritema nodozum benzeri lezyonlar, pap�lop�st�ler lezyonlar, y�zeyel tromboflebit, ekstragenital �lser, paterji reaksiyonu ve di�er vask�litik deri belirtileri (Sweet sendromu benzeri, pyoderma gangrenozum benzeri, eritema multiforme benzeri lezyonlar, palpabl purpurik lezyonlar, subungual infarktlar ve hemorajik b�ller, fronk�l ve apseler) g�zlenir. Deri ve mukoza belirtileri Beh�et hastal��n� karakterize eden en �nemli bulgulardand�r. Hastal��n ba�lang�c�nda ya da herhangi bir d�neminde en s�k saptanan ve hastal��n tan�s�nda da son derece �nemli olan belirtilerdir. Oral ve genital �lserler, deri belirtileri, g�z belirtileri ve eklem bulgular� hastal��n g�r�ld�� t�m �lkelerde en s�k saptanan semptomlard�r. Eritema nodozum benzeri lezyonlar ve pap�lop�st�ler lezyonlar ise hastal��n en s�k g�zlenen deri belirtileridir. Hastal��n en s�k ba�lang�� semptomu hemen t�m d�nyada oral �lser olarak bildirilmi�tir. Yine genital �lser ve deri belirtilerinden �zellikle eritema nodozum benzeri lezyonlar ba�lang�� semptomu olarak saptanabilmektedir. Hastalar�n �o�unda, deri ve mukoza belirtileri ciddi organ tutulumundan �nce ortaya ��kmaktad�r. Bu nedenle hastal��n deri ve mukoza belirtilerinin iyi bilinmesi erken tan� ve geli�ebilecek ciddi komplikasyonlar�n �nlenebilmesi a��s�ndan b�y�k �nem ta��maktad�r.

Anahtar Kelimeler: Beh�et hastal��, deri ve mukoza belirtileri, derleme

Erkan Alpsoy. Mucocutaneous lesions of Beh�et�s disease. Turkderm-Turk Arch Dermatol Venereol. 2003; 37(2): 92-99

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