Discussion
The coexistence of PV and EoE in these two cases presents a unique
clinical scenario, with only one case report in the medical literature
presenting a similar association3. highlighting the
complexity and interplay of autoimmune diseases. PV is an autoimmune
blistering disorder primarily affecting the skin and mucous membranes,
while EoE is a chronic immune-mediated esophageal condition
characterized by eosinophilic infiltration and esophageal dysfunction,
predominantly associated with allergic conditions1,2.
Both cases underscore the necessity of a thorough and multidisciplinary
diagnostic approach when dealing with patients presenting with new or
unusual symptoms, particularly in those with known autoimmune
conditions. In Case 1, the patient developed dysphagia and esophageal
impaction, which necessitated a biopsy to distinguish between esophageal
involvement by PV and EoE. In Case 2, the patient initially presented
with symptoms of EoE, which were managed successfully, only to later
develop PV. The diagnosis of EoE in both cases was confirmed by
endoscopic findings and biopsies demonstrating significant eosinophilic
infiltration. The presence of both conditions in the same patients
suggests a possible shared immunopathogenic mechanism, although the
exact pathophysiology remains unclear3. The
simultaneous occurrence of both conditions is exceptional in the medical
literature, and dysphagia in PV patients is often empirically attributed
to mucosal involvement of the esophagus by PV4,5. The
shared pathogenesis of PV and EoE might be linked to the involvement of
desmoglein (DSG) proteins, mainly DSG-16. Our findings
are consistent with a previously reported case by Gue et al. in 2017
documenting a 13-year-old boy with PV and EoE, emphasizing the possible
dysregulation of DSG 1 as a common etiologic factor in both
conditions3. Eosinophils play a central role in EoE
and have also been implicated in the pathogenesis of PV, suggesting a
shared pathogenic mechanism1,2. The significant
improvement in both EoE and PV symptoms in our patient treated with
dupilumab, a monoclonal antibody targeting the IL-4 receptor alpha that
is FDA-approved for the treatment of EoE and used off-label for
recalcitrant PV cases7–9, suggests that Th2-mediated
pathways might be a common underlying mechanism in both
diseases10–12.
Conclusion :
The coexistence of PV and eosinophilic esophagitis EoE in these cases
highlights the complex interplay between autoimmune diseases and
underscores the necessity of a thorough and multidisciplinary diagnostic
approach. Our findings demonstrate that EoE should be considered in PV
patients presenting with dysphagia, as it is essential not to attribute
such symptoms solely to esophageal involvement by PV. The significant
improvement observed in both conditions with the use of dupilumab
suggests a shared Th2-mediated pathway, opening new avenues for
therapeutic intervention. Further research is needed to elucidate the
underlying mechanisms linking PV and EoE.