ImmunoDeficiency Resource (IDR)

Patients have psoriasiform hyperkeratosis of the palms, soles and the dorsal surfaces of the hands and feet. Scaly, psoriasiform lesions over the knees, elbows, and interphalangeal joints Psoriasiform lesions can also be seen on the limbs. Diffuse transgredient palmoplantar keratoderma, typically developing within the first 3 years of life. Punctiform accentuation, particularly along the palmoplantar creases. This keratoderma associates, as early as infancy, intense gingivitis with alveolar bone lysis and early loss of baby teeth. During childhood, this phenomenon of periodontal disease recurs with rapid loss of adult teeth. Unless treated, periodontosis results in severe gingivitis and loss of teeth by age 5 years. Patients exhibit increased susceptibility to cutaneous (furunculosis, skin abscesses, hidradenitis suppurativa) and systemic infections. Anomalies of chemotaxis and phagocytosis by polymorphonuclear leukocytes have been observed. Patients may have malodorous hyperhidrosis.