Figure 1

Clinical findings of four differently aged PN patients. Panel A refers to the US patient (#25); B, C and D to the Turkish patients #16, #17a and #26, respectively. Patient #25, the youngest in our cohort shows an erythroderma characterized by background erythema and islands of relative sparing on face and legs (A1, A2) and distal onycholysis of fingers and toes (A3, A4). Patient #16 face: poikiloderma and carious teeth are well apparent (B1). Poikiloderma is also visible on the trunk and arm (B2). Pachyonychia of the toes is shown (B3). Facial view of patients #17a and #26 demonstrating prominent forehead, saddle nose and long philtrum (C1, D1); poikiloderma is evident on the face and on the ear helix too (C1) and forearm (D2). Plantar hyperkeratosis (C2) and nail thickening (C3) can be seen. Severe malformation of hands and feet with unhealing ulcers and marked nail dystrophy (D2, D3).