Inherited knee disorders in the Medici family.

Reconstructing a medical condition which was existent centuries ago is limited by the lack of contemporaneous evidence-based descriptions in the accounts given by physicians and other observers. Despite these limitations modern paleopathological evidence, supplemented by techniques of historical investigation, have led to the conclusion that males in the Medici family typically suffered from a complex clinical entity with a triple pathology of stenotic spinal ankylosis, recurrent peripheral joint disease and erythematous skin disease; the Medici Syndrome. Examination of the knee joint is illustrative of recurrent joint disease both in the primary and secondary lines of the family. Pictorial and sculptural representations, if used cautiously, can assist in this retrospective process. The six cases presented here illustrate the involvement of the knee joint where the joint destruction ultimately led to an ankylosis.

The Medici syndrome: a medico-historical puzzle.

The historical significance of the Medici family of Florence is widely recognised, but the diseases which afflicted leading members of this family have only been scientifically studied in recent decades. Paleopathological findings on exhumed skeletons, supplemented by medical descriptions in historical documents, have permitted a retrospective diagnosis of a triple pathological syndrome in the senior branch of the Medici family. Peripheral joint and spinal conditions, with the presence of skin disease, are identified in several generations of the family in the 15th century and are presented as the 'Medici syndrome'. Radiological findings are compared with macro- and microscopical descriptions in the diagnosis of the peripheral joint disease and spinal ankylosis/stenosis within the syndrome.

Erythema migrans: a chronicle.

Arvid Afzelius first described and named erythema migrans (EM), a clinical entity that he assumed to be caused by an agent transmitted by the bite of a tick (Ixodes reduvius). Certain neurologic, cutaneous, and other syndromes observed in Europe were recognized in the 1920s and 1930s to be disabling sequelae of EM. In the 1940s and 1950s the effectiveness of penicillin as therapy for EM was demonstrated. In 1968 the first patient with EM and neurologic sequelae in North America benefited from treatment with penicillin. In 1975, an epidemic arthropathy appeared in the area of Lyme, Connecticut. Despite resemblance to EM (the initial appearance of cutaneous lesions), the complex was called Lyme disease because of the occurrence of cardiac, neurologic, and arthritic sequelae. The vector of Lyme disease, Ixodes dammini--a tick that harbors agents that cause Lyme disease and babesiosis--was identified and characterized in 1979. The spirochete that causes Lyme disease was designated Borrelia burgdorferi. The North American and European species of spirochete and the clinical syndromes to which they are related are described.

Erythema annulare centrifugum.

If one reviews the literature on the subject of erythema annulare centrifugum, it becomes quite obvious there is considerable confusion about the clinical presentation and histopathologic findings. This confusion is exemplified by the various quotes from dermatologic texts and scientific publications. Darier, who originally described the disease, described an annular, indurated, erythematous lesion without a scale that histologically was characterized by a superficial and deep lymphohistiocytic infiltrate and normal epidermis. Ackerman suggested that there are two types of gyrate erythema, a superficial type showing a scale, and the deep type as described by Darier. After reviewing the literature and studying patients with gyrate erythemas, it seems that there are two distinct types best termed the superficial and deep forms of gyrate erythema. Dermatologists use the term erythema annulare centrifugum to denote both of these forms. Perhaps that term should be discarded.

Cutaneous photobiology: past, present and future.

The history and origin of the science of photobiology are reviewed. Interest in the biologic effects of light gradually increased, beginning with the discovery of ultraviolet and infrared radiation early in the 19th century. The basis of experimental photobiology was laid by the studies of Raab and Tappeiner on photodynamic action and the early uses of phototherapy by Finsen and Dorno. The discovery of the association of porphyrins with some light-related skin diseases and of the capability of chemical agents such as coal tar and bergamot to induce phototoxic contact dermatitis resulted in a flurry of clinical investigations leading to better understanding of the processes of phototoxicity and photoallergy. The early epidemiologic studies of Unna and Dubreuilh relating solar radiation exposure to the formation of actinic keratoses and non-melanoma skin cancer were experimentally confirmed in animals by Findlay, Roffo, and Blum. In the most recent quarter century (1950-1975), cellular and molecular photobiology has been refined. The studies on photochemistry of nucleic acid and of damage and repair mechanisms in DNA have set the stage for understanding the basic processes of biologic effects of light and promise the development of useful applications of specifically directed phototherapy and prevention of such light-induced diseases as skin cancer.