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Unusually extensive scalp ulcerations manifested in pemphigus erythematosus
Neha Chandan1, Eden P Lake, Lawrence S Chan
1University of Illinois College of Medicine, Chicago, Illinois. nchand8@uic.edu.
Dermatology Online Journal
|February 23, 2018
Summary
Senear-Usher Syndrome, a rare autoimmune skin disorder, presents with overlapping features of pemphigus foliaceus and lupus erythematosus. This case highlights diagnosis via immunofluorescence and ongoing treatment for scalp ulceration.
Area of Science:
- Dermatology
- Autoimmunology
- Pathology
Background:
- Senear-Usher Syndrome (pemphigus erythematosus) is an autoimmune blistering disease.
- It exhibits clinical overlap between pemphigus foliaceus and lupus erythematosus.
- Typical lesion sites include the scalp, face, and upper torso.
Observation:
- A patient presented with progressive scalp ulcers.
- Clinical signs included hyperpigmentation and eroded plaques with hemorrhagic crusts.
- These symptoms indicated a severe manifestation of the condition.
Findings:
- Direct immunofluorescence confirmed pemphigus erythematosus.
- Key findings were intercellular and dermoepidermal junction deposition of immunoglobulin G and complement.
- This diagnostic pattern is crucial for differentiating autoimmune blistering diseases.
Implications:
- Early and accurate diagnosis is vital for effective management.
- Treatment involves systemic steroids and steroid-sparing immunosuppressants.
- Understanding immunofluorescence findings aids in patient management and prognosis.
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