Scleredema adultorum associated with IgG-kappa multiple myeloma--a case report and review of the literature.
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Biomedical subjects
Publications and source records attributed to M Sandbank.
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Humoral and cellular immunity were evaluated in 10 patients with Darier's disease. The mean levels of serum immunoglobulins, serum complement, peripheral macrophages, and peripheral B and T lymphocytes were within normal limits. Secretory IgA was present in all patients studied. Skin tests for delayed hypersensitivity revealed complete anergy in one of the eight patients tested. Lymphocyte transformation tests revealed statistically significant enhanced responsiveness to both PHA and ConA mitogens in several concentrations studied. These findings may suggest either the existence of alterations in immunoregulation of lymphocyte subpopulations in Darier's disease or may be limited to alterations in membrane functions of certain lymphocyte subpopulations which can be detected only in vitro. The role that such immunologic aberrations may play in the pathogenesis of Darier's disease is still obscure.
A survey of 115 pemphigus patients who have been followed for 4-24 years was carried out in an attempt to analyze the possible factors influencing the course and prognosis of the disease. At the time of the study, 45 patients were in complete remission, 45 still had lesions, and 25 had died. In 12 cases, the death occurred from causes related to the disease or consequences of treatment. The comprehensive statistical analysis revealed that a good prognosis may be expected for patients who, in the initial stage of the disease, show involvement of the skin only (p less than 0.05) and when systemic steroid therapy is instituted within half a year of appearance of the disease (p less than 0.05). There were no significant differences in the rate of recovery and mortality between the different groups of patients in regard to the initial dose of prednisone. A daily dose of 120 mg of prednisone was sufficient to control the lesions in most of the severe cases and less contributory to death in comparison with previous studies.
In a series of 100 adults with tinea faciei various dermatologic manifestations were mimicked, viz.: discoid lupus erythematosus in 52 patients, lymphocytic infiltration in 15, seborrheic dermatitis in 11, rosacea in 8, contacts dermatitis in 7, polymorphous light eruption in 4 and granuloma faciale in 3. Trichophyton rubrum was isolated in 78% of the respective cases, other isolated organisms being Microsporum canis (13 cases), Trichophyton violaceum in 6, Trichophyton mentagrophytes (asteroides) in 2 and Epidermophyton floccosum in 1 case. In 85% of these patients the nails were also involved by the same agent found in the lesions of the face. On the basis of these observations it is recommended that all adult patients with tinea faciei should undergo a comprehensive mycological investigation to find the primary focus, which may be an infected nail.
Juvenile pemphigus (JP) is a rare disease. Of 274 pemphigus patients who were under our care during the last 26 years, 4 (1.4%) were in the JP category. The course and prognosis of the disease in the 4 patients who have been followed for 4-19 years are presented. Two patients are still in complete remission and off treatment for 4 and 6 years. In view of the literature and our experience, it appears that the clinical manifestations and course of JP are variable, similar to adult pemphigus. However, it seems that JP has a favorable prognosis quoad vitam.
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A 57-year-old patient with rheumatoid arthritis, who was being treated with D-penicillamine, developed a bullous eruption 2 months after the onset of treatment. On the basis of the clinical, histological and immunofluorescent findings, the diagnosis of herpetiform pemphigus was made. The eruptions disappeared within 2 weeks after the discontinuation of D-penicillamine treatment. During the 16-month follow-up period there was no recurrence. It is noteworthy that a patch test with potassium iodide was positive and that testing for HLA antigens revealed HLA B8, A1, A2, and BW39.
A superficial pustular eruption with acute onset has been reported in patients with acne vulgaris receiving systemic antibiotic treatment. In all past cases the causative micro-organism was found to be a gram-negative bacterium. This case report describes a similar clinical picture under the same circumstances, in which the bacterium incriminated was a coagulase-negative Staphylococcus.
For patients treated with high doses of prednisone (over 40 mg/day) laboratory follow-up is important, particularly when the therapeutic response does not correlate with the dose of prednisone prescribed. Taking advantage of the similarity in structure of prednisone to cortisone and of prednisolone to cortisol we evaluated simple, inexpensive methods for the assessment of prednisone and prednisolone intake and bio-availability. Free urinary prednisolone was estimated by radioimmunoassay for cortisol and found to be linearly correlated with the dose of prednisone administered (r = 0.9310). Levels of free prednisolone were 6.5-11% of the dose of prednisone per day. Free and metabolized prednisone and prednisolone were estimated as 17-OHCS in the 24-hr urine collection. Total, conjugated and free 17-OHCS were linearly correlated with the dose of prednisone, the best correlation being with total 17-OHCS (r = 0.9060). About 40% of the prednisone administered was secreted as total 17-OHCS.
Topical application of citral on male rat skin induces hyperplasia of the sebaceous glands. Ultrastructural study showed that this hyperplasia is manifested by an increase in number of the partially differentiated cells of the gland. Citral was found in fat droplets of the mature differentiated and partially differentiated cells. Because citral causes an increase in testosterone level, we conclude that the sebaceous gland hyperplasia is related to androgen activity.
We report on a 75-year-old man showing the very rare association of Leser-Trélat's sign and adenocarcinoma of the sigmoid colon. Our case is the second one reported so far.
We report on a 17-year-old primigravida with pruritic urticarial papules and plaques of pregnancy. Histopathologic examination revealed multiple dermal fibroblasts which almost disappeared after recovery. This unusual histologic feature has not been described in polymorphic eruption of pregnancy (pruritic urticarial papules and plaques and pregnancy) so far.
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We hereby report a case presenting a rare form of secondary syphilis with an unusual nodular eruption accompanied by pruritus and generalized lymphadenopathy, bearing a striking resemblance to lymphoma. The histologic picture was also misleading and was compatible with lymphocytoma cutis. The diagnosis was eventually made after results of serologic tests for syphilis were found to be positive, with an extremely high titer of VDRL, and dark-field microscopy had demonstrated spirochetes in a nodular lesion. Secondary syphilis should thus be considered in the differential diagnosis of nodular lesions resembling lymphoreticular disease. It is also suggested that syphilis be added to the list of diagnoses belonging to the category of pseudolymphoma.