Schamberg's Disease

SCHAMBERG'S DISEASE (morbus Schamberg), or progressive pigmentary dermatosis, is a rare skin disease, first described in 1901 by Schamberg; the first case in the USSR was published in 1928 by Shvartsman. Schamberg's disease occurs more often in young men and is localized mainly on the skin of the lower legs and feet, in individual cases on the upper extremities, buttocks, and trunk. The disease begins with closely spaced reddish punctate petechial spots, resembling the smallest grains of cayenne pepper and sometimes having a somewhat telangiectatic appearance. While new eruptions continue to appear gradually at the periphery of the lesions, the old punctate hemorrhages slowly disappear, leaving a fairly persistent reddish-brown pigmentation, and sometimes slight superficial atrophy. Subjectively, the patient sometimes experiences slight itching. The evolution and involution of an individual lesion can last for years. Histologically: the epidermis is little changed; in the papillary and subpapillary layers of the skin proper, and sometimes deeper, there is significant infiltration, consisting mainly of lymphocytes, and to a lesser extent of polynuclears, epithelioid and plasma cells. Characteristic is the accumulation in the areas of the infiltrate of fine-grained, intracellularly and extracellularly located brown iron-containing hemosiderin pigment. Changes in the vessels are minimal: they consist of slight dilation, swelling of the endothelium, and insignificant formation of new capillaries. Diagnosis. Schamberg's disease clinically sometimes differs little from Majocchi's disease (see); in typical cases of the latter, telangiectasias come to the fore, the eruptions have the shape of rings, and histologically there is sharp dilation of vessels, endarteritis, ruptures, etc. Schamberg's disease differs from the so-called yellow ochre dermatitis (dermite jaune d'ocre) in that the latter develops (on the lower legs and feet) as a result of local or general circulatory disorders, and usually dilated veins are present. Etiology is unknown. The positive Koebner phenomenon obtained in Schamberg's disease—an eruption similar to the existing one appears at the site of superficial trauma to healthy skin—allows one to assume an inferiority of the vascular walls; in individual cases, changes in the blood (thrombopenia, a decrease in the number of erythrocytes, etc.) have also been noted. The prognosis regarding the duration of the disease is always doubtful. In treatment, attention should be paid to the general condition of the patient, and the blood should always be examined. For local treatment in cases with a small number of lesions, freezing with carbonic acid snow (30 seconds with slight pressure) can be recommended.