Erythema Elevatum Diutinum with a Prolonged Bullous Phase

Area of Science:

• Dermatology
• Immunopathology

Background:

• Erythema elevatum diutinum (EED) is a rare, chronic vasculitis characterized by persistent papulo-nodular and plaque lesions.
• The condition typically affects extensor surfaces and joints, presenting a diagnostic challenge due to its chronicity.

Observation:

• A 70-year-old male presented with chronic, persistent papulo-nodular and plaque lesions on the skin overlying joints and extensors.
• A distinctive preceding phase of a prolonged vesicular eruption was noted.
• Clinical and pathological findings were consistent with erythema elevatum diutinum.

Findings:

• Skin testing with Streptokinase-Streptodornase (SK/SD) elicited a highly reactive response.
• Abnormal neutrophilic chemotaxis was observed.
• The patient exhibited a dramatic clinical response to dapsone therapy.

Implications:

• This case highlights the potential diagnostic utility of preceding vesicular eruptions in EED.
• Abnormal neutrophilic chemotaxis may play a role in the pathogenesis of EED.
• Dapsone appears to be a highly effective treatment for EED, warranting further investigation.