A progressive pigmentary dermatosis with purpuric eruption, usually in males, affecting chiefly the lower extremities, occasionally other parts of the body. Onset at any age with irregular plaques, orange brownish with "cayenne pepper" spots, within and at edges of lesions, erupting most frequent on the legs. Occasionally, slight pruritus. No systemic symptoms. Autosomal dominant trait. Since approximately half the patients have a positive Hess test it must be distinguished from haemorrhagic manifestations. Schamberg's patient was a 15 year old boy.
See also Gougerot-Blum disease, or pigmented purpuric lichenoid dermatitis, under Henri Gougerot, French dermatologist, 1881-1955.
- J. F. Schamberg:
A peculiar progressive pigmentary disease of the skin.
British Journal of Dermatology, Oxford, 1901, 13: 1-5.