Early invasive vulvar squamous cell carcinoma arising in a woman with vulvar pemphigus vulgaris and systemic lupus erythematosus

Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucous membranes. Genital involvement occurs when most other common sites are concurrently affected or are in remission. Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many parts of the body and the skin with occasional bullous lesions. Pemphigus vulgaris and SLE may be associated, albeit rarely. Here, we report the first case of a woman affected with SLE presenting with early invasive squamous cell carcinoma (SCC) arising from Pemphigus Vulgaris of the vulva

BEHÇET’S DISEASE

Behçet’s disease (BD) which is classified among vasculitides is a systemic disease with various manifestations. Its clinical course is characterized by attacks and remissions. Till now, two nationwide surveys of BD from Iran and Japan and 4 major case series from Turkey, Korea, Morocco and England have been reported. Clinical picture of BD is dominated by mucous membrane manifestations, including oral aphthosis - seen in 96.8% of patients in Iran, 98.2% in Japan, 100% in Turkey, 97.5% in Korea, 100% in Morocco and 100% in England- and genital aphthosis which is seen less frequently- 65.3% in Iran, 73.2% in Japan, 88.2% in Turkey, 56.7% in Korea, 83.5% in Morocco and 89% in England. Skin aphthosis is not frequent but it is the most characteristic lesion of BD. Ocular manifestations include anterior uveitis, posterior uveitis and retinal vasculitis. Joint manifestations include arthralgia, monoarthritis, oligo/poly arthritis, and ankylosing spondylitis. Other manifestations include neurological, gastrointestinal and cardiopulmonary manifestations, vascular involvement, orchitis and epididymitis. Erythrocyte sedimentation rate is usually elevated. Urinary abnormalities are infrequent and transient. Positive pathergy test has been reported in 57.4% of patients in Iran, 44% in Japan, 57% in Turkey, 40% in Korea, 68% in Morocco and 32% in England. Lesions usually heal without sequela, except for lesions of eyes, brain and vascular system. The major cause of morbidity is the ocular lesion, which could lead to severe loss of vision or blindness

CORRELATION OF PEMPHIGUS VULGARIS ANTIBODY TITERS BY INDIRECT IMMUNOFLUORESCENCE WITH ACTIVITY OF DISEASE BASED ON PEMPHIGUS AREA AND ACTIVITY SCORE (PAAS)

Indirect immunofluorescence (IIF) has been used to identify and measure autoantibody levels in pemphigus vulgaris but data about relationship between clinical severity of disease and antibody titers by IIF have been conflicting. We conducted this cross-sectional study to correlate the severity of oral and/or cutaneous involvement in patients with pemphigus vulgaris based on Pemphigus Area and Activity Score with IIF titers. Sixty-one new pemphigus vulgaris patients were included in this study. Human prepuce was used as substrate for IIF and assessment of disease severity was based on Pemphigus Area and Activity Score. The mean±SD age was 44.04±30.46 years, with a range of 18 to 79 years. IIF was positive in 56 (91.8%) patients. There was a significant relationship between total disease score and IIF titers ((P<0.001). Also a significant relationship was found between skin score (P=0.04) and mucosal score (P=0.04) with IIF titers. Our results show that there is a significant relationship between disease activity based on Pemphigus Area and Activity Score and antibody titers by IIF. Further studies are recommended to determine the usefulness of this technique for monitoring disease

Dermatoscopic and mucoscopic features of lesions in patients with Behcet's disease

Objective: Behcet's disease (BD), as a vasculitis, can affect small and large vessels. As dermatoscopy has been shown to improve the accuracy in diagnosis of various skin lesions especially vascular patterns, we set this study to find if there is any characteristic pattern in the dermatoscopy of Behcet's mucocutaneous lesions.Methods: This prospective cross-sectional observational study designed to evaluate dermatoscopic features of Behcet's mucocutaneous lesions. Fifty six consecutive patients presenting at the outpatient clinic of the BD Research Unit were included. If present, for each patient one oral, one skin and one genital lesion were evaluated by dermatoscopy. When indicated, pathergy test was performed according to a standard protocol and the results were evaluated by dermatoscopy.Results: A total of 40 oral, 8 genital, 14 skin lesions and 14 pathergy tests were evaluated by dermatoscopy. While vascular component was the most prominent feature in oral aphthae, this component was less prominent in genital lesions. Dot vessels were the most common form of vessels in both oral and skin lesions. All the oral lesions were characterized by a central white structureless area. Skin lesions were characterized by a red structureless background. In pathergy tests, negative pricks showed absence of specific features while positive pricks were characterized by a structureless background in pink, purple or red. No obvious vascular component was detected in any of the pricks.Conclusion: It seems that these findings have no specific clues for the diagnosis of BD, but our study is the first study in this field and the findings may give way to further investigations

IN TIME VARIATION OF PATHERGY PHENOMENON IN BEHCET'S DISEASE

The pathergy phenomenon (PP) is one of the hallmarks of Behcet's Disease (BD). It has been reported to be positive from 17.5% to 83% of cases in different studies. PP is used as a diagnosis criterion in the Dilsen criteria, the Japan criteria, the International criteria, and the Iran criteria. It is therefore important to know wether PP is a fix manifestation of the disease or a cyclic manifestation like mucocutaneous disorders. To evaluate the charaterist'tc of P'P over a period of time, 77 patients were selected randomly (rcgaardless of their disease activity or their disease manifestations), to have a second pathergy test, lite pathcrgy test was performed by needle puncture on the skin of the forearm. Three methods were used: 1- needle prick with a 21 - gauge needle. 2- needle prick with a 25  gauge needle. 3- needle prick with a 25-guage needle and the injection of I drop of a sterile normal saline solution. Tlie result was evaluated 24 hours Liter. Tlie shortest interval between two tests-was one month and the longest 13 years. Die mean interval was 35.4 months, the standard deviation was 36.6 Tlurty-nine patients had a negative PI' at their first evaluation. At the second evaluation, 27 remained negative while 12 changed to positive. Thirty-eight patients had a positive PP at their first evaluation. At the second evaluation, 19 remained positive while 19 changed to negative. Although there is a difference between the two groups, it is not statistically significant (x=3.813 , p=0.05). Our data demonstrates that the pathergy phenomenon is waxing and waning, like the majority of other manifestations of Behcet's Disease. TJierefore, it may be repeated for diagnosis purposes