Synonyms: Dermatitis pigmentaria progressiva, pigmentary progressive dermatosis syndrome, progressive pigmentary dermatosis, progressive pigmentary disease, purpura pigmentosa chronica progressiva, purpura pigmentosa progressiva, purpuric pigmentary dermatosis syndrome.

Associated persons: Paul Blum

Henri Gougerot

Jay Frank Schamberg

Description: 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.

Bibliography:  J. F. Schamberg: A peculiar progressive pigmentary disease of the skin. British Journal of Dermatology, Oxford, 1901, 13: 1-5.

Pigmented purpuric dermatoses have been traditionally divided into 5 clinical entities: Schamberg's purpura (typically presenting as "cayenne pepper" spots on lower extremities), Majocchi's purpura (described as purpura annularis telangiectoides), lichen aureus (characterized by a solitary golden-colored patch with purpura), Gougerot-Blum purpura (typically presenting as lichenoid papules with purpura on lower extremities) and eczematid-like purpura of Doucas and Kapetanakis (characterized by itching and orange-colored pigmentation). These clinical entities are histologically indistinguishable and, probably, represent a spectrum of one disease.