Cervical spine involvement in lipoid dermato-arthritis.

Abstract

Lipoid dermato-arthritis is a recently recognized, rare disease of unknown etiology, characterized by cutaneous nodules and chronic polyarthritis. An abnormality of lipid metabolism (1) has been implicated. Reticulohistiocytoma, reticulohistiocytosis, and reticulohistiocytic granuloma are synonymous terms. The histologic features of the nodules in the skin and synovial tissues are diagnostic and consist of infiltrates of characteristic histiocytes and multinucleated giant cells, possessing abundant eosinophilic, finely granular or homogeneous cytoplasm. Solitary or multiple nodules may occur without arthritis but the latter has been present in the majority of patients with multiple cutaneous lesions (2). The peripheral joints often show progressive changes with widespread destruction of the cartilage and subchondral bone, resembling severe rheumatoid arthritis (2, 3). The present case is reported to illustrate and emphasize the involvement of the cervical spine.2 The present experiments were carried out at the CP-5 research reactor to compare the results of earlier acute studies with those at lower dose levels. They were undertaken to evaluate the effects of a series of brief fractionated exposures ranging from the 30-day acute dose of both fission neutrons and Co60 gamma rays, through the subacute area, to low total-dose levels. Such information will be useful in planning experiments in which animals will be exposed for the length of their lives in the low-level neutron room of the new “Janus” reactor. This reactor, now being built in Argonne's Division of Biological and Medical Research, should be in operation in the near future. Case Report J. D., a 38-year-old white woman, noticed pruritic “bumps” on the arms, elbows, hands, face, and neck in 1951. Xanthelasma of the eyelids also appeared at that time. Painful arthritis developed in the left knee several months later, and soon the other knee and both ankles were affected. Within one year the hips, elbows, wrists, and hands were similarly involved. The arthritis progressed so rapidly that the patient was confined to a wheel chair in 1953. Shortening of the fingers occurred over a period of four years with gradual decrease of pain in the hands. The cutaneous lesions persisted. Dryness and coarseness of the skin and hair suggested hypothyroidism, for which she was treated with desiccated thyroid for several years. The patient was first seen at The University of Michigan Medical Center in 1958 with complaints of severe, aching pain in the joints of the extremities and generalized body tenderness. Urinary frequency, nocturia, and polydipsia had been present for two years. The blood pressure was 140/98. The skin of the face and dorsum of the hands was coarsened, erythematous, and scaling. Slight periorbital edema and bilateral xanthelasmas of the eyelids were noted. Flesh-colored, subcutaneous nodules, up to 5 mm. in diameter, were seen along the nasal margins and in the hands, and were sparsely scattered over the entire body. The thyroid gland was not enlarged on palpation. The heart, lungs, and abdomen were normal. The elbows, shoulders, hips, knees, and ankles were tender and swollen, due to thickening of the articular soft tissues and increased joint fluid. Severe shortening and hyper-mobility of the fingers were noted, with redundancy and telescoping of the skin (“opera glass hands”). Several joints of the feet were also involved. The cranial nerves and deep tendon reflexes were normal and pathologic reflexes were not elicited.