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Updated: May 31, 2026
Technique of Conjunctival Biopsy and Direct Immunofluorescence for Diagnosing Mucous Membrane Pemphigoid
Published on: June 17, 2025
From pemphix to desmogleins
Daška Štulhofer Buzina1, Branka Marinović
1Department of Dermatology and Venereology, University Hospital Center Zagreb, School of Medicine, University of Zagreb, Salata 4, 10000 Zagreb, Croatia. daska.stulhofer-buzina@zg.htnet.hr
Abstract:
In the not so distant past, the word pemphigus or pemphix was common for describing various diseases characterized by blistering as well as various disorders that do not originate from a blistering pathology. Patients with these conditions were grouped in "other" skin diseases. Step by step, during the past, we were introduced to these severe conditions. First, we learned from sporadic case reports, then new differentiations were reported according to histology, later immunopathology was developed, and now there are discoveries of new molecules. Immense progress with new approaches to therapy has been achieved, but much improvement is still needed. The modern definition of pemphigus undoubtedly represents a group of rare, intraepidermal autoimmune bullous diseases characterized by intraepidermal blisters and circulating autoantibodies desmogleins against the keratinocytes cell surface.
Insights
Pemphigus, a group of rare autoimmune blistering diseases, involves intraepidermal blisters and autoantibodies targeting keratinocytes. While therapies have advanced, significant improvements in treating these severe skin conditions are still needed.
Area of Science:
- Dermatology
- Immunology
- Pathology
Background:
- Historically, pemphigus was a broad term for blistering conditions and unrelated disorders.
- Patients were often categorized under "other" skin diseases, lacking specific diagnosis.
Observation:
- Progress in understanding pemphigus involved case reports, histology, and immunopathology.
- Recent advancements include the discovery of new molecules involved in the disease process.
Findings:
- Pemphigus is now defined as rare, intraepidermal autoimmune bullous diseases.
- Characterized by intraepidermal blisters and autoantibodies against desmogleins on keratinocyte surfaces.
Implications:
- Despite therapeutic progress, substantial improvements in pemphigus treatment are still required.
- Further research into novel molecules and targeted therapies is essential for better patient outcomes.
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