Severe Relapsing Hailey-Hailey Disease Displaying a Durable Complete Response to Hydroxyurea

Area of Science:

• Dermatology
• Genetics
• Hematology

Background:

• Familial benign chronic pemphigus, or Hailey-Hailey disease, is a chronic, autosomal-dominant genetic skin disorder.
• It is characterized by impaired keratinocyte adhesion, leading to blisters and rashes, often on the neck, torso, and extremities.
• Conventional treatments have limited efficacy, particularly for severe or relapsing forms.

Purpose of the Study:

• To report a case of Hailey-Hailey disease that showed a surprising positive response to hydroxyurea treatment.
• To explore the potential of hydroxyurea as a therapeutic option for refractory Hailey-Hailey disease.

Main Methods:

• A 54-year-old male patient with a long history of severe Hailey-Hailey disease was diagnosed with polycythemia vera.
• He was treated with hydroxyurea for polycythemia vera.
• The patient's Hailey-Hailey disease symptoms were monitored during hydroxyurea therapy.

Main Results:

• The patient experienced complete resolution of Hailey-Hailey disease skin lesions after commencing hydroxyurea treatment.
• The skin condition remained in remission for four years while polycythemia was well-controlled.
• Hydroxyurea was well-tolerated with no significant side-effects.

Conclusions:

• Hydroxyurea may represent a novel and effective treatment for severe, chronic, or relapsing Hailey-Hailey disease.
• This finding warrants further investigation in larger clinical studies.
• The study highlights an unexpected therapeutic benefit of a medication commonly used in hematology.