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Summary
Pemphigus, a fatal skin disease, often leads to death from infection, particularly Staphylococcus aureus septicemia originating from skin lesions. Long-term corticosteroid use masked inflammation, contributing significantly to mortality.
Area of Science:
- Dermatology
- Pathology
- Immunology
Background:
- Pemphigus is a severe, often fatal, autoimmune blistering skin disease.
- Understanding mortality factors in pemphigus is crucial for patient management.
Purpose of the Study:
- To investigate the causes of death and contributing factors in patients with pemphigus.
- To analyze clinical and autopsy data from deceased pemphigus patients.
Main Methods:
- Retrospective review of patient records and autopsy findings.
- Histological and immunofluorescent confirmation of pemphigus diagnosis.
- Analysis of infection sources, common pathogens, and serum protein levels.
Main Results:
- Infection was the primary cause of death, with septicemia (Staphylococcus aureus) in 9 of 13 cases.
- Pneumonia was prevalent, and skin lesions were common infection sources.
- Low serum protein and albumin levels were observed in most patients.
- Corticosteroid therapy masked inflammatory signs, complicating diagnosis and treatment.
Conclusions:
- Infection, especially Staphylococcus aureus septicemia, is a major cause of mortality in pemphigus.
- Long-term high-dose corticosteroid therapy is a significant contributing factor to pemphigus mortality.
- Judicious use of corticosteroids is recommended to mitigate risks.