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Purpura simplex manifests clinically as macular purpuric and petechial pigmented, golden, annular, or lichenoid lesions. These subtypes have been termed Schamberg's purpura, lichen aureus, Majocchi's purpura, and Gougerot-Blum purpura. Histologically, there is inflammation and hemorrhage without fibrinoid necrosis of vessels. One hundred seventy-four cases were retrospectively reviewed. In some patients, the eruption was related to medications. Treatment was usually of limited benefit. Fifty-eight of 87 patients (67%) who had follow-up data did eventually have clearing of lesions. Because of the clinical and histologic similarities among the subtypes, the inclusive term purpura simplex is favored.

Two unusual cases of purpura simplex raise the problem, as yet unsolved, of the pathogenesis of the purpuric eruption. Together with a previous observation of hemorrhagic purpura made by Roskam, they show the inconstancy and variability of the vascular fragility test. ACKNOWLEDGMENT This paper was translated by Raymond G. Gottschalk, M.D., whose cooperation is gratefully acknowledged.

Clinical observations are presented on 11 members of a family in which hereditary familial purpura simplex and congenital ptosis coexist in 6. These persons represent 4 generations of dominant genetic transmission of both traits.

The characteristic histologic change seen at 2 h in the Rumpel-Leede (Hess) test is, in addition to focal hemorrhage in the upper dermis, a perivascular lymphocyte infiltrate with focal areas of lymphocytic epidermal invasion. The histologic findings, even with the liquefaction degeneration, are similar to those of purpura simplex and indicate that, early in many forms of vasopermeability, the lymphocyte may be a primary inflammatory cell.

The Schoenlein-Henoch syndrome is described on the basis of 44 personally observed cases. It is defined as a condition in which nontraumatic hemorrhage with or without edema may occur in the skin, or subcutaneous tissue, or joints, or viscera, or in any combination of them, and in which the blood platelets are normally abundant. The causes of the syndrome are varied, but streptococcal infection is important, and "anaphylactoid" causes rare. The prognosis is good, and the 4 cases with renal involvement also did well. Schoenlein-Henoch purpura and purpura simplex overlap, but in contrast with the latter, Schoenlein-Henoch purpura is not familial, and is common in males and children.

Werlhof1first published his description of "morbus maculosus haemorrhagicus" in 1775, but knowledge concerning the nature of hemorrhagic disease has been slow in advancement. Since his publication, many attempts have been made to segregate various clinical types of purpura and to define their relationship to a defect in the clotting mechanism. Although efforts to explain the many etiologic factors concerned and the particular defect involved have not been entirely successful, certain aspects have been clarified by recent contributions to hematology. In view of the additional new facts, the present discussion will be devoted to a partial reclassification of the hemorrhagic disorders and to methods of treatment. CLASSIFICATION OF THE HEMORRHAGIC STATES Hornung2in 1734 divided purpura into three groups: purpura simplex, febrile purpura, and purpura scorbutica. Since then from time to time various attempts have been made to subdivide purpura into clinical entities. Among these endeavors may be