Three case reports of post immunization and post viral Bullous Pemphigoid: looking for the right trigger.

BACKGROUND: Bullous pemphigoid (BP) is a blistering skin disorder infrequent in infancy and rarely reported in medical literature. CASE PRESENTATION: Here we describe three cases of BP which were referred to our department in the last 15 years. Two of them developed an eruption of bullous lesions just a few days after vaccination for diphtheria, tetanus, pertussis, poliomyelitis, hepatitis B and Haemophilus influenzae B. The third patient developed the same blistering lesions shortly after herpetic stomatitis. In all three cases, clinical diagnosis was confirmed by histological examination which showed subepidermal bullae with a dermal inflammatory infiltrate, and direct immunofluorescence of perilesional skin showed linear IgG and C3 deposits along the basement membrane zone. Immunoblot assay was positive for BP antigen 180. Treatment with oral prednisone was instituted and the lesions resolved in two out of three patients; the third one was treated with an immunosuppressive agent (tacrolimus) and corticosteroid and subsequently with intravenous immunoglobulin and plasmapheresis, due to an underlying complex autoimmune disease. CONCLUSION: Although the mechanism of induction of BP is still unclear, the close relationship between trigger events (immunization or viral infection) and onset of the disease arises a possible association.

Searching for foreign antigens as possible triggering factors of autoimmunity: Torque Teno virus DNA prevalence is elevated in sera of patients with bullous pemphigoid.

OBJECTIVE: The Torque Teno virus (TTV), a member of virus genus Anellovirus has been shown to be commonly present in humans, yet without detectable pathogenicity. Recent studies imply that TTV may contribute to provoke autoimmune progresses in systemic lupus erythematosus and idiopathic inflammatory myopathies. We aimed to study the presence of TTV in a group of patients with autoimmune bullous diseases with a further goal to identify long-lasting foreign antigen, such as TTV as possible triggers of skin-specific autoimmunity. PATIENTS AND METHODS: We performed in silico research to study similarities between known TTV sequences and antigens of bullous pemphigoid (BP), pemphigus vulgaris (PV) and dermatitis herpetiformis (DH). Basic Local Alignment Search Tool results showed matching regions for the major BP antigens BP180 and BP230, PV antigen desmoglein 3 and DH antigen transglutaminase 3 and disclosed overlapping, antigen-predicted sequences only for BP180 regions. We also assessed the prevalence of TTV in these disorders and compared them with the results from two healthy blood donor groups (group 1: sex- and age-matched for the general bullous group, n = 95; group 2: sex- and age-matched for BP, n = 50). Furthermore, we assayed lymphocytes from four TTV DNA and BP180 NC16A blot-positive BP patients and three controls in a standard lymphocyte transformation test with a TTV peptide from the conserved ORF(Open Reading Frame)1/N22 region. RESULTS: We found that the detection rate of TTV was comparable with that in healthy controls in the group of PV (19/33); whereas detection rates in DH showed a slight, but not significant tendency for elevation (17/20). Contrary, the TTV prevalence in BP patients was significantly elevated (group 1: 36/40 vs group 2: 31/50, P < 0.032). Lymphocytes from all four virus-positive BP patients heavily reacted to TTV peptide while two of the three healthy controls have shown not to recognize the viral sequences. Only the TTV carrier healthy control had a minor reaction at lowest peptide concentration. The combined in silico, polymerse chain reaction and in vitro cell assay data of the present study indicate that a TTV persistence may contribute to the pathogenesis of BP.

Prevalence of human herpesvirus 8 infection measured by antibodies to a latent nuclear antigen in patients with various dermatologic diseases.

BACKGROUND: Human herpesvirus 8 (HHV-8) has been detected in all epidemiological forms of Kaposi sarcoma (KS). The role of HHV-8 in dermatologic diseases other than KS is controversial. Some studies based on polymerase chain reaction findings suggest an association between HHV-8 and epithelial tumors of the skin, lymphoproliferative disorders, or pemphigus. OBJECTIVE: To assess the prevalence of antibodies against a latent nuclear antigen of HHV-8 in patients with various dermatologic diseases. DESIGN: An indirect immunofluorescence assay was used to search for HHV-8 antibodies. SETTING: Ambulatory or hospitalized patients from a university hospital associated with a research laboratory. PATIENTS: Eighty-three patients with various non-KS dermatologic diseases and 16 patients with KS who were seronegative for the human immunodeficiency virus. Controls were 100 healthy subjects living in the same area. RESULTS: Antibodies to HHV-8 were found in 100% (16/16) of the patients with KS and 3.6% (3/83) of the patients with non-KS dermatologic diseases: 1 patient with pemphigus vulgaris, 1 with discoid lupus erythematosus, and 1 with bullous pemphigoid. The prevalence of antibodies to HHV-8 in controls was 2% (2/100) and was not significantly different than the prevalence in patients with dermatologic diseases other than KS (P =.28). CONCLUSIONS: Our serologic study confirms the higher prevalence of HHV-8 antibodies in patients with KS and demonstrates that contrary to other human herpesviruses, HHV-8 is not a ubiquitous virus in France. We could not determine any causal association between HHV-8 and pemphigus or lymphoproliferative disorders of the skin.

Bullous pemphigoid complicating human orf.

We report five cases of human orf complicated by bullous pemphigoid. This is a previously unrecorded complication of orf. Knowledge of the association allows for better management in the affected patient.