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Keywords:

  • acantholysis;
  • reflectance confocal microscopy;
  • familial benign chronic pemphigus;
  • Hailey–Hailey disease;
  • pemphigus

Background/purpose

Reflectance confocal microscopy (RCM) is a non-invasive method for high-resolution, in vivo imaging of the epidermis and upper dermis. The purpose of the study was to evaluate the potential usefulness of RCM as a non-invasive diagnostic tool for Hailey–Hailey disease (familial benign chronic pemphigus).

Methods

Four patients with Hailey–Hailey disease were examined by RCM. Subsequently, punch biopsies were taken to compare RCM images with corresponding histopathologic findings.

Results

On RCM images, the most sticking feature was acantholysis at the level of the granular and spinous layer, resembling a ‘dilapidated brick wall’. We suggest the term ‘dilapidated brick wall RCM sign’ to describe this feature and to distinguish from the corresponding histopathology finding. Other RCM features included: epidermal disarray, intraepidermal clefts, inflammatory cells in the epidermis and in the superficial dermis. These RCM abnormalities correlated with analogous histopathology findings.

Conclusion

Reflectance confocal microscopy is a promising non-invasive diagnostic tool for Hailey–Hailey disease. The method may also be considered useful for choosing the best site for biopsy, which may aid pathology evaluation and spare time needed to establish the diagnosis.