An unusual case of diffuse focal osteolysis with disseminated osteoclastosis.

We report the unusual case of a 31-year-old woman referred for inflammatory pain of the long bones associated with diffuse focal osteolysis, elevated erythrocyte sedimentation rate (ESR) and hypercalcemia. Corticosteroid therapy produced dramatic symptomatic improvement, and biological parameters returned to normal. Biochemical markers of bone remodeling, particularly those of resorption, were strikingly elevated. Parathyroid hormone, parathyroid hormone-related protein, nephrogenous cyclic adenosine monophosphate and calcitriol levels were normal. Investigations for infectious disease and malignancy were negative. The histology of the lesions consisted of cortical osteolysis caused by intense osteoclastic resorption, with several foci of osteogenesis, without malignant cells. The patient died 9 months after the onset of symptoms, following a "malignant" course: severe hypercalcemia, extension of lytic bone lesions and spontaneous fractures. To our knowledge, such a peculiar picture with quite unusual radiologic findings, disseminated focal cortical lesions and a progressive course, without evidence for a tumoral process, has never been described. This case suggests either the presence of an unknown systemic osteoclast-activating factor from an occult tumor or a primary malignant hyperosteoclastosis.

[Systemic scleroderma and sarcoidosis: 3 new cases]. / Sclérodermie systémique et sarcoïdose: trois nouveaux cas.

We observed 3 patients with successive scleroderma (SS) and (what is considered to be) sarcoidosis (SA). The diagnosis SS included in the 3 patients: Raynaud's syndrome with pulpal necrosis and capillaritis, sclerodactylia and acro-osteolysis, multiple joint pain and FAN+. Also observed were: esophagus involvement (n = 3), pulmonary artery hypertension (n = 1), telangiectasia (n = 2) and anti-Scl 70 (n = 2). Initially, all patients had restrictive pulmonary disease. SS was diagnosed 5 to 9 years prior to SA in 2 patients. Diagnosis of SA was based on the following arguments: Loëfgren's syndrome with erythema nodosa (n = 1), parotiditis (n = 2), sicca syndrome (n = 2), myalgia (n = 2), joint involvement (n = 2), non-infectious pluropericarditis (n = 2), epitheloid and giant cell granulomas without caseous necrosis (lung = 3, liver = 1, lymph nodes = 1, salivary glands = 1, synovia = 1), negative search for bacilli, elevated conversion enzyme (n = 1) and, in each case, by the lack of any other cause. One patient died from lung cancer and another from respiratory failure. Nome of the patients had primary biliary cirhosis. This rare association between SS and SA demonstrates the confluent limits of certain systemic diseases and raises a difficult problem to differentiate pulmonary involvement in these diseases. The gravity of this localization and the poor sensitivy to corticosteroids.