Pierre Y. Venencie

Scleredema: A review of thirty-three cases

A review of thirty-three cases of scleredema, with particular reference to clinical and histologic findings, revealed that the disease was often of insidious onset and the course was usually prolonged. A preceding respiratory tract infection was uncommon and usually did not indicate a short course. The patients were divided into two groups according to the presence or absence of diabetes. Diabetes, when present, was typically the late-onset, insulin-dependent type and difficult to control. In many of these patients, the onset of scleredema was so subtle that it went unnoticed by the patient until pointed out by an examining physician.

Recombinant interferon alfa-2b treatment of necrobiotic xanthogranuloma with paraproteinemia

nocarcinomas and are characterized by a high metastatic potential, especially to bones, liver, and lungs.1o Dissemination occurs via three mechanisms: (1) local, (2) lymphatic, and (3) hematogenous,13 often via the vertebral venous system. The route of cutaneous metastases is controversial, but arterial or venous embolizationlO> 23 and dissemination via lymphatic vessels24 and perineural lymphatics2’ have been suggested.13

Multiple cutaneous leiomyomata and erythrocytosis with demonstration of erythropoietic activity in the cutaneous leiomyomata.

We report the case of a 41‐year‐old woman with multiple cutaneous and uterine leiomyomata. The erythropoietic activity demonstrated in a cutaneous leiomyoma might account in part for her secondary erythrocytosis.

Monoclonal gammopathy and mycosis fungoides: Report of four cases and review of the literature

Of four patients with mycosis fungoides and monoclonal gammopathy, one died of multiple myeloma that developed 4 years after the mycosis fungoides. The other three had monoclonal gammopathy of undetermined significance. This association does not appear to occur with a high frequency in patients with mycosis fungoides than in the general population. However, it demonstrates that B cell proliferation can occur in patients with primary cutaneous T cell disease.

Monoclonal Gammopathy in Sézary Syndrome: Report of Three Cases and Review of the Literature

Three patients with Sézary syndrome had monoclonal gammopathy: two had gammopathy of unknown significance and one had myeloma. The frequency of the association of Sézary syndrome and monoclonal gammopathy seems to be lower than previously suggested. However, it does emphasize that the presence of T-cell cutaneous disease does not preclude B-cell clonal proliferation.

Necrobiotic xanthogranuloma with myeloma. A case report

Necrobiotic xanthogranuloma is a newly recognized cutaneous manifestation associated with paraproteinemia. Necrobiotic xanthogranuloma is distinctive clinically by yellowish‐red nodular lesions, often ulcerated, with predilection for the periorbital area and ocular involvement. Histologically, it is characterized by the combination of inflammatory xanthogranulomas together with areas of necrobiosis. These skin lesions are associated with a dysglobulinemia and preceded the development of an IgG lambda myeloma in a previous patient. Hyperlipidemia may be found in some patients. A case of necrobiotic xanthogranuloma is reported, occurring in a 46‐year‐old man and associated with an IgG kappa myeloma.

Keratoderma and spastic paralysis.

Punctate keratoderma and spastic paralysis occurred in three generations of a family. Several members had keratoderma of the palms and soles or spastic paralysis or both. The family history was consistent with autosomal‐dominant inheritance. The age at onset and the rate of progression of symptoms were variable. The concurrence of these lesions can be interpreted to mean either that the keratoderma and the paraplegia are the pleiotropic effects of the same mutant gene or, less likely, that they are the manifestations of two different autosomal mutations segregating in this family. We are not aware of a similar syndrome having been previously reported.