Small skin blood vessel occlusions by cryoglobulin aggregates in ulcerative lesions in IgM-IgG cryglobulinemia

The skin biopsy specimens from six patients with primary mixed IgM-IgG cryoglo-bulinemia were examined by immunofluorescence, light and electron microscopy. The biopsy taken from the involved skin of one patient with leg ulcers revealed small blood vessel occlusions by cryoglobulin aggregates. Since a similar finding was not observed in the biopsy material taken from the other five patients who had no ulcerative skin lesions, it seems that the cryoglobulin aggregates play a role in the development of the skin ulcerations in primary mixed IgM-IgG cryoglobulinemia.     

Residual skin cancer after preoperative biopsy: evaluation by Mohs micrographic surgery

BACKGROUND: Some patients are referred for Mohs surgery with no or minimal clinical evidence of skin cancer at the biopsy site. OBJECTIVE: To determine the incidence of residual skin cancer at biopsy sites during Mohs micrographic surgery. METHODS: We evaluated all patients that underwent Mohs surgery for basal cell and squamous cell carcinomata in one year. The study was carried out prospectively. Debulking was done using a no. 15 blade at the clinical borders of the tumor or biopsy site. All specimens were sectioned at the middle and cut to the periphery at 20- microm intervals. RESULTS: Seven hundred and forty-one patients underwent operations. In 390 patients, a biopsy was performed prior to surgery. A total of 351 patients were biopsied as prestaged (chemocheck) during surgery. Ninety-nine (25%) of the preoperatively biopsied patients showed no residual tumor in the debulking specimen or the first layer. Of these 99 patients, 84 had basal cell carcinoma and 15 had superficial or in situ squamous carcinoma. CONCLUSIONS: In this study, preoperative biopsy for diagnosis of skin cancer of the face was curative in 25% of patients, despite pathologic diagnosis of incompletely excised tumor. However, as the majority of preoperatively biopsied patients showed residual tumor, Mohs micrographic surgery is indicated in all patients with incomplete removal of skin cancer of the head and neck.     

Nonsecretory IgD-Kappa Multiple Myeloma in a Patient with Gaucher's Disease

A patient with Gaucher's disease and non-secretory multiple myeloma is described. The presence of typical Gaucher and myeloma cells was confirmed by transmission and scanning electron microscopy. The immunological studies established the diagnosis of ‘non-secretory’ type of multiple myeloma.     

Familial cryoglobulinemia and C4 deficiency

The coexistence of cryoglobulinemia and C4 deficiency was found in one family. Twelve subjects were examined, most of them being asymptomatic. Cryoglobulins were found in 9 members and its type was defined in 6 of them, 3 having mixed IgM-IgG and 3 having monoclonal IgM cryoglobulins. The concentration of the third component of the complement system was normal in 10 subjects, while the concentration of the fourth one was found to be below the lower limit of normal in 9 subjects. Only C4 deficiency--and not cryoglobulinemia--was HLA-linked. There was no evidence of HBs antigenemia. The results of this study are discussed in the light of the pertinent literature.     

Levan-induced glomerulitis in rabbits: a possible role for direct complement activation in situ

Since high-molecular-weight levan is known to reduce capillary permeability to large molecules, an experiment was designed to investigate whether this agent may attenuate the glomerulonephritis associated with acute serum sickness in rabbits. The study, in fact, demonstrated an enhancing effect of levan, which caused increased glomerular proliferative changes and leucocyte infiltration and, possibly, increased IgG deposition in this experimental model. In addition, rabbits injected only with levan also demonstrated mild glomerulitis and C3 deposition. In one of four rabbits examined, this was accompanied by a marked fall in the serum level of total haemolysing complement. Levan was demonstrated to cause activation of complement when incubated with normal rabbit serum in vitro. We believe that these findings are best explained on the basis of complement activation in situ by levan in the glomeruli, probably via the alternative pathway, with the resulting inflammatory response. In the case of BSA-injected rabbits, this response is believed to be additive to that of the classical immune complex-mediated complement activation.     

The clinicopathologic features of cryoglobulinemic nephropathy

Renal biopsy specimens from eight patients with cryoglobulinemic nephropathy were examined by light, electron, and immunofluorescence microscopy. The light microscopic features were not diagnostic. Characteristic ultrastructural findings were observed in all except one specimen and consisted of glomerular electron-dense deposits, which had a coarse, granular appearance at low-power magnifications and revealed tubular, annular and in a few cases, also fibrillar structures at high-power magnifications. Subendothelial deposits were a constant finding. The presence of intravascular thrombi (deposits), osmiophilic bodies, and crystals within the cytoplasm of the glomerular cells are also helpful for the diagnosis of this entity. Because of these specific findings, cryoglobulinemic nephropathy should be recognized as a clinicopathologic entity.     

Systemic lupus erythematosus and lymphoma. A family study

After 3 years of treatment with azathioprine and prednisone, immunoblastic lymphoma appeared in a patient with systemic lupus erythematosus. An increased incidence of immunological aberrations and malignancies was found in her family and their relation to the pathogenesis of this patient's diseases is discussed.     

Possible role of fibrinogen in the aggregation of white blood cells

In order to verify whether leukocyte aggregation correlated with aggregation of other cellular elements during inflammation, we examined the state of leukocyte adhesiveness/aggregation (LAA) in the peripheral blood and red cell aggregation. Correlation was found to be significant as was the correlation between LAA and fibrinogen, and with the fibrin/fibrinogen degradation products concentration during various inflammatory states. In vitro leukocyte aggregation was decreased when the cells were suspended in autologous heat defibrinogenated plasma as compared to cells suspended in autologous native plasma. Heat aggregated fibrinogen but not native fibrinogen caused leukocyte aggregation in vitro. Finally, Arvin defibrinogenation in rabbits reduced the state of LAA in endotoxinemic rabbits. Integrating all this information, we assume that fibrinogen participates not only in the aggregation phenomena of red cells and platelets, but also in those of leukocytes.     

Nonsecretory IgD-kappa multiple myeloma in a patient with Gaucher's disease.

A patient with Gaucher's disease and non-secretory multiple myeloma is described. The presence of typical Gaucher and myeloma cells was confirmed by transmission and scanning electron microscopy. The immunological studies established the diagnosis of 'non-secretory' type of multiple myeloma.     

An unusual pattern of arthritis dermatitis syndrome

A 27-year-old woman presented with skin lesions and arthritis of the right knee of two days' duration. Her medical history was noncontributory and synovial fluid aspirated from the affected knee and a biopsy of a skin lesion were negative for gonococci. However, after methylene blue staining of cervical and urethral smears, intracellular and extracellular diplococci were demonstrated. Immunofluorescence showed deposits of immunoglobulin complexes of the skin section within the wall of blood vessels and dermal-epidermal junction. Treatment with ampicillin, 4 gm a day, resulted in resolution of the lesions and arthritis in two days. The need for cervical and urethral smears and for better, as yet unavailable tools for the diagnosis of the arthritis dermatitis syndrome, is emphasized.