Bullous disorders as a manifestation of immune reconstitution inflammatory syndrome: A series of three cases

Abstract

Bullous disorders such as pemphigus vulgaris, bullous pemphigoid after the initiation of highly active antiretroviral therapy in certain human immunodeficiency virus reactive individuals have been described in this case series as a manifestation of an immune reconstitution inflammatory syndrome. This phenomenon should be suspected in individuals who present with bullous lesions within 3-8 weeks after initiation of therapy despite of improved immunological response. Strong clinical suspicion, through clinical examination, appropriate laboratory investigation such as CD4 T-cell count, histopathological examinations with H and E stain, direct immunofluorescence test are required for diagnosis.

Case of bullous pemphigoid positive for full‐length BP180 ELISA accompanied by immunological reconstitution due to antiretroviral therapy for human immunodeficiency virus infection

“…Several theories have been proposed to explain the development of autoimmunity with HIV infection, including molecular mimicry to a self-antigen, persistent antigen stimulation, and restoration of the immune system with ART. 1 According to the previous reports proposing a clinical staging system to categorize autoimmune disorders in HIV and their relationships to total CD4 counts and viral loads, 1,2 the present case, in which bullous lesions had appeared accompanied by the restoration of CD4 counts after ART (Figure 1c), could be categorized as stage IV (i.e., CD4 >500 with low viral load) and was suggested to be caused by immune reconstitution by ART, as in the reported case, 3 although there was no obvious evidence. Interestingly, the present case improved spontaneously without any treatment, suggesting that the autoimmune disease due to immune reconstitution by ART might disappear depending on the normalization of the immune response.…”

Exuberant and refractory bullous pemphigoid in HIV patient: a case report

“…The depletion of CD4 T cells triggers an intensified activation of all immune system components 7 . Factors such as molecular mimicry to self-antigens, persistent antigenic stimulation, immune reconstitution under antiretroviral therapy (ART), and dysregulation in the interaction between T and B cells may justify this binomial 1,7,8 . The intricate web of physiopathological factors can pose a significant challenge in the treatment of autoimmune diseases in individuals with HIV, as illustrated in the current case.…”

Case Report: Autoimmune Pemphigus Vulgaris in a Patient Treated With Cemiplimab for Multiple Locally Advanced Cutaneous Squamous Cell Carcinoma

“…As a confirmation for the above comments, cases of bullous disorders, such as PV and BP, have been reported in the literature after HAART therapy as a manifestation of IRIS, caused by a paradoxical production of auto antibodies against intercellular substances (desmoglein) and dermo-epidermal junction, respectively. The pathogenic mechanism may occur due to aberrant T−cell signalling to B cells, and secretion of immunoglobulin due to the sudden increase in CD4 T−cell count following initiation of HAART ( 20 ).…”