Follicular atrophoderma in a 6-year-old male child with type-2 pachyonychia congenita

A 6-year-old male child presented with progressive hypertrophy and discoloration of all 20 nails. Further evaluation revealed milia in bilateral concha and face, which were present since infancy. Other findings included atrophoderma over bilateral sides of lateral canthi of eyes and absent lower central incisors. Past medical and family history had remarkable findings such as natal teeth and similar nail changes in his elder sister. Similar nail changes, milia and focal plantar keratoderma were present in his father and elder sister. The patient's clinical presentation and history were compatible with pachyonychia congenita subtype 2.