Abstract :
A13-year-old boy presented with telangiectatic erythema on his face and hemorrhagic ulcers on his lips. His medical history was significant for repeated infections, mostly of the respiratory tract. Physical examination revealed a short stature (131 cm), low weight (20 kg), thin face, beak-like nose, telangiectatic erythema on the face, high-arched palate and hemorrhagic crusted ulcers on the lips. A few café-au-lait spots and two hypopigmented small patches were seen on his abdomen. Histologic features of the skin biopsy specimen are shown in the Figures.