A Rare Case of Azathioprine-Induced Sweet's Syndrome in a Patient with Crohn's Disease.

Sweet's syndrome has been reported in association with inflammatory diseases such as Crohn's disease. It has also been reported in association with several drugs. Here, we report a rare case of Sweet's syndrome induced by azathioprine in a patient with Crohn's disease.

Fatal scleromyxedema: report of a case and review of the literature.

Scleromyxedema is a rare fibromucinous connective tissue that can be associated with systemic changes, such as myopathy, neurologic defects, esophageal dysmotility, paraproteinemia, and restrictive lung disease. We describe a fatal case of scleromyxedema in which neurologic, cardiac, gastrointestinal, and muscle changes were present. At autopsy, mucin was found in the papillary dermis of skin and in coronary and pulmonary vessels, but was absent from the brain, kidneys, heart, gastrointestinal tract, esophagus, liver, thyroid, lymph nodes, bone marrow, and pancreas. Because the pathogenesis of scleromyxedema may not always be attributable to mucin deposition, the role of circulating factors in the development of systemic manifestations warrants further investigation.

In vitro functional reactivities of cutaneous mast cells from patients with mastocytosis.

Cutaneous mast cells from 3 patients with mastocytosis were evaluated for their morphologic characteristics and in vitro functional reactivities to different secretory agonists. By electron microscopy, mastocytosis mast cells appeared larger than normal skin mast cells, frequently had atypical, highly indented or bilobed nuclei, and each contained numerous, elongated cytoplasmic projections. Suspensions of mastocytosis mast cells were obtained from lesional skin biopsy specimens, and their response to both immunologic and nonimmunologic secretagogues was compared with mast cells from normal skin. Lesional skin mast cells had a net histamine release of 12.3% (+/- 1.3 SEM) and 31.1% (+/- 6.0 SEM) following stimulation with the purified human anaphylotoxin C3a and mouse monoclonal antihuman IgE antibodies, respectively. This specific release was similar to the responses observed in normal skin mast cells (11.5% +/- 4.5 SEM and 16.7% +/- 2.1 SEM, respectively). Mast cells from cutaneous lesions of mastocytosis also responded to the nonimmunologic secretagogues, morphine sulfate and calcium ionophore A23187 with a specific histamine release of 15.1% (+/- 1.2 SEM) and 39.8% (+/- 8.7 SEM), respectively. The results of this study demonstrate that mast cells from lesions of mastocytosis are morphologically atypical, but have a histamine content similar to normal skin mast cells and retain their functional reactivities to clinically relevant secretory stimuli.