Vesiculobullous Darier Disease Symptomatically Responsive to Cetirizine

Area of Science:

• Dermatology
• Genodermatoses
• Immunology

Background:

• Darier disease is an autosomal dominant genodermatosis.
• It is characterized by abnormal keratinization, leading to hyperkeratotic papules and plaques.
• A rare vesiculobullous variant exists, posing treatment challenges.

Observation:

• A 55-year-old woman with treatment-resistant Darier disease experienced a rare vesiculobullous presentation.
• Standard treatments including topical tacrolimus and glucocorticoids were ineffective.
• A punch biopsy revealed a perivascular infiltrate of eosinophils.

Findings:

• Cetirizine, an antihistamine with eosinophil-antagonizing properties, was administered.
• The patient showed marked improvement in burning sensation with increased cetirizine dosage.
• This suggests a potential role for eosinophils in the pathogenesis of vesiculobullous Darier disease.

Implications:

• Eosinophils may contribute to blister formation in vesiculobullous Darier disease.
• Major basic protein from eosinophils might disrupt desmosomes, leading to blisters.
• Cetirizine offers a potential therapeutic avenue for this rare variant by targeting eosinophils.