Childhood bullous pemphigoid developed after the first vaccination.

Bullous pemphigoid (BP) typically affects the elderly. There are at least 40 reported cases of BP in childhood, 10 reported cases at 1 year of age or younger. The antigen of this autoimmune disease is localized to the hemidesmosome. Neoplasia, recurrent trauma, some systemic diseases, and psoriasis were previously reported as possible triggering factors of bullous pemphigoid in some cases. In the last 5 years, 10 adult and 2 infantile BP cases with a close relation of vaccination have been reported. Anti-influenza vaccine, tetanus toxoid booster, and tetracoq vaccine were the possible causes of these cases. We report herein a 3.5-month-old BP case in whom the lesions developed 24 hours after the first tetracoq vaccine. We suggest that vaccination may be the triggering factor of BP of any age by stimulating the immune system with an unexplained mechanism.

Juvenile Behçet's disease among 1784 Turkish Behçet's patients.

BACKGROUND: Behçet's disease is a chronic, relapsing disease, about which information on its clinical course in juveniles is only available from small groups of patients. MATERIALS AND METHODS: Patients suffering from Behçet's disease who had their first lesion at or before the age of 16 were evaluated in terms of: age at onset, mucocutaneous signs, and findings related to systemic involvement. Ninety-five patients, evaluated as having juvenile Behçet's disease (JBD), were detected among 1784 Turkish Behçet's patients. The mean age of these 95 patients (51 boys or men, 44 girls or women) who had JBD was 26.8 +/- 7.1 years. RESULTS: The difference between sexes in terms of age at onset, development period of second lesions, and systemic involvement was not found to be significant in JBD. Patients were divided into two groups, one showing severe disease (N = 27) and the other mild disease (N = 68). There was no significant difference between the two groups with respect to age, age at onset, and sex distribution. The interval between the development of the first and second lesions was shorter in the patient group with severe disease (P < 0.001) and the development of second lesion was most frequently seen in the first 5 years (P < 0.05). Systemic involvement developed also in a shorter time in the group with the severe disease (P < 0.01) and was most frequently encountered during the first 5 years (P < 0.05). Conversely, patients with the mild disease developed systemic involvement more frequently after 6 years or later. CONCLUSIONS: Severe Behçet's disease in children and juveniles shows no age or sex predilection, but leads to an earlier recurrence and more severe systemic signs than the mild form.

Interferon alfa-2a in the treatment of Behçet's disease.

BACKGROUND: There is no definite treatment for Behçet's disease. New drugs are being evaluated in cases unresponsive to conventional treatment modalities. OBJECTIVE: In this study we investigated the efficacy of interferon alfa-2a on skin lesions in 18 Behçet's disease patients who had predominantly mucocutaneous involvement. METHODS: Eighteen patients with Behçet's disease were treated with interferon alfa-2a at 3 million IU/day in the first week (three times a week), 6 million IU/day in the second week (three times a week), 9 million IU/day in the third week and thereafter (three times a week). Interferon alfa-2a was administered subcutaneously for a total of 12 weeks. RESULTS: At the end of the treatment the efficacy of the drug was found to be good in 9 patients and very good in 7 patients. Interferon alfa-2a is particularly effective in skin manifestations such as genital ulceration and erythema-nodosum-like lesions. It was also found to be effective in systemic manifestations such as fever, diarrhea and eye involvement. CONCLUSION: Our results indicate that interferon alfa-2a is a promising drug in the treatment of Behçet's disease.

Mucocutaneous criteria for the diagnosis of Behçet's disease: an analysis of clinicopathologic data from multiple international centers.

BACKGROUND: Although four of five of the new international criteria for the diagnosis of Behçet's disease relate to mucocutaneous lesions, disagreement exists as to the exact nature of cutaneous lesions (e.g., vessel-based vs follicular). OBJECTIVE: Our purpose was to review clinical data, clinical photographs, and skin biopsy specimens from multiple medical centers throughout the world to monitor current practice in the implementation of mucocutaneous diagnostic criteria for Behçet's disease. METHODS: Ten medical centers responded to a request to collaborate by sending clinical data, photographs of cutaneous lesions, and biopsy specimens from 22 patients. RESULTS: Of specimens from 22 patients, 14 revealed a histopathologic pattern of neutrophils containing perivascular and interstitial inflammation, whereas specimens from three patients revealed only mononuclear cells in a vessel-based pattern. Biopsy specimens from three patients revealed primarily folliculocentric inflammation and an additional two specimens were from erythema nodosum-like lesions. CONCLUSION: Perivascular inflammation was the predominant histopathologic finding in specimens of cutaneous lesions in this clinical series. Folliculocentric lesions could not be predicted on the basis of review of clinical photographs. Histopathologic assessment of cutaneous lesions is crucial if the proposal is accepted that exclusion of folliculocentric lesions is important to ensure accurate implementation of diagnostic criteria in patients with suspected Behçet's disease.

The association of HLA-B5 antigen with specific manifestations of Behçet's disease.

The HLA-B5 phenotype was investigated in 235 patients with Behçet's disease and in 100 healthy persons. HLA-B5 was more frequent in the patients (77 vs. 30% p < 0.001, relative risk = 7.8). The frequency of HLA-B5 was higher in patients with genital ulceration than without (82.3 vs. 63%, p < 0.01). Patients with thrombophlebitis showed a less frequent positivity of HLA-B5 antigen than the patients without thrombophlebitis (50 vs. 79.2%, p < 0.02). These results suggest that HLA-B5-related genes not only affect the development of Behçet's disease but also the occurrence of its clinical manifestations.

A new kind of skin lesion in Behçet's disease: extragenital ulcerations.

A new skin lesion was encountered in 29 of 970 Behçet's patients. The lesions resembled oral aphthae clinically, were recurrent and left a scar tissue like genital ulcers but were located extragenitally. Skin biopsies could be done in only 4 cases and they all showed vasculitis.