Case 2

Diagnosis

Behcet’s disease

 

Progress

The patient was referred to medical department with oral prednisolone commenced.  After 8 weeks, her oral and genital ulcers had healed. The patient was then switched to azathioprine.

 

Discussion

Causes of vulvar ulcers are diverse and can be divided into infectious and noninfectious categories. Sexually transmitted infections include genital herpes simplex virus (HSV) infection, syphilis, chancroid, granuloma inguinale, and lymphgranuloma venereum. Secondary bacterial infection or fungi can also cause genital ulcers. Non-infectious etiologies include psoriasis, Behcet’s disease, Wegener granulomatosis, and fixed drug eruption ⁽¹⁾. Malignancies may also present as non-healing genital ulcer.

 

Behcet’s disease is a rare cause of genital ulceration. It is a chronic relapsing multisystem vasculitic disorder with unknown aetiology. The diagnosis of Behcet’s disease is based on clinical criteria and the International diagnostic criteria for Behcet’s disease include oral aphthosis, skin manifestations, vascular lesions, pathergy phenomenon, genital aphthosis, and ocular lesions ⁽²

^,3). Our patient had fulfilled the criteria of recurrent oral and genital ulcerations plus the cutaneous manifestation, and hence, a diagnosis of Behcet’s disease was made.

 

Genital ulcers of Behcet’s disease typically start as a tender nodule, it will then become deep and painful, and eventually heal with scarring. Searching for scars on genital skin, even in the absence of active, clinical disease is an important part of the examination. Ulcers typically are found on the labia majora, but can occur anywhere on the vulva, perineum, or perianal skin. They can also occur intra-vaginally, potentially leading to fistula formation with the urethra or bladder.

 

Although literatures have not studied on the effectiveness of colposcopy and biopsy in the diagnosis of Behcet’s disease, colposcopy definitely has a role in a more detailed evaluation of the genital tract, characteristics of the ulcer and to rule out the possibility of malignancy. There are also case reports and studies of people using colposcopy in aiding them in the examination of genital ulcers in Behcet’s disease.

 

Upon biopsy taking, care should be taken to obtain a full-thickness punch or incisional specimen that includes skin from the periphery of the ulcer, rather than from the center of the lesion. Histopathological results in Behcet’s ulcer are typically nonspecific with chronic active inflammation and necrosis without vasculitis as demonstrated in the above slides. Special staining may be used to the biopsies to facilitate the diagnosis of specific infection.

 

Papanicolaou smear in our patient showed ASCUS, but she had a normal cervix on colposcopy and biopsy results showed cervicitis only. In a prospective study by Suna and collesgues looking at 152 women, of whom 78 are patients with Behcet’s disease, it was noticed that abnormal cervical cytology, acetowhite epithelium and iodine-negative epithelium on colposcopy were more common in Behcet’s disease patients than the control. However, the rate of abnormal histopathology was similar between both groups. The majority of the ASCUS results revealed a normal finding after cervical histopathology in patients with Behcet’s disease. This was postulated to be caused by the benign inflammatory changes in the cervical epithelium ⁽²⁾.

 

In general, malignancy is rare in Behcet’s ulcers. In a recent case report by Hata and collesgues, a squamous cell carcinoma in a chronic genital ulcer has been reported in a female patient with Behcet’s disease ⁽⁴⁾. For these reasons, it might be expected that the disease may lead to abnormal cervical or vaginal epithelial changes. However, there is no study to confirm its causal relationship yet ⁽²⁾.

 

As demonstrated in this case, diagnosis for recurrent genital ulcers can be difficult, however once the information of oral ulcer was obtained, the differential diagnosis of Bechet’s became more evident. Therefore, the most important aspect of the evaluation of a patient with vulvar aphthae is a complete history and physical examination, with particular attention to signs or symptoms of an underlying associated systemic condition.