Sustained Successful Treatment of Two Phenotypically Different Cases of Genital Papular Acantholytic Dyskeratosis

Genital papular acantholytic dyskeratosis (GPAD) is a rare, recurrent condition. It is characterised by skin coloured or whitish papules which may coalesce to form plaques affecting the genitocrural or anogenital skin in women and men. Histological features include hyperkeratosis, focal parakeratosis with acantholytic and dyskeratotic cells. It is recognised as a distinct clinicopathological entity localised to genital skin, but there are increasing suggestions of genetic links with Hailey-Hailey disease and Darier's disease. Heat, friction, and hormonal influence may play a role in aetiology. We present two patients with different phenotypical presentations who have had effective treatment sustaining remission. Corticosteroids are widely reported as an ineffective treatment, but our first patient had a rapid response to oral prednisolone without recurrence. The second case supports the evidence that surgical excision is an effective option for localised disease. Both patients presented via circuitous routes. Diagnosis is often delayed and likely underestimated. Our first patient developed the condition after surgical menopause, which may add to evidence of hormonal influence. We highlight that GPAD is an important differential to consider for vulval, anogenital, or genitocrural papules.