| Items |
ComedonalDarier’s disease |
Classic Darier’s disease |
| Incidence |
Extremely rare |
Rare |
| Demographics |
| Age of onset |
Ranges from 10s to 66 |
2nd decade usually |
| Sex |
Predominantly in males |
Equal in both sexes |
| Geographic distribution |
Asia, East & Middle East |
Worldwide |
| Aetiology |
| Defective Gene |
ATP2A2 |
ATP2A2 |
| Reported mutation types |
Deletion [only one report] |
Missense & splicing [many reports] & others e.g. deletions |
| Mode of inheritance |
Autosomal dominant with a possible X-linked inheritance |
Autosomal dominant |
| Clinical Manifestations |
| Comedonal lesions (Open & / or Closed) |
Must be present |
Absent |
| Facial ice-pick scars |
May be present |
Absent |
| Dirty warty papules / plaques |
May be absent |
Must be present |
| Palmar pits / keratoses |
May be present |
May be present |
| Nail changes |
May be present |
May be present |
| Mucosal lesions |
May be present |
May be present |
| Associations |
| Neurological disorders |
None reported |
Higher liability |
| Susceptibility to infections |
None reported |
Increased susceptibility |
| Histopathological Findings |
| Acantholyticdyskeratosis |
Invariably present |
Invariably present |
| Follicular involvement |
Present & prominent |
Present |
| Villi & papillary projections |
Markedly elongated |
Elongated |
| Course |
| Chronicity |
Chronic |
Chronic |
| Remissions |
Not reported |
Spontaneous remission rarely reported |
| Exacerbations by sun, heat & lithium |
Absent |
Present |
