DIAGNOSTIC SIGNIFICANCE OF THE IMMUNOFLUORESCENT PATTERN IN DERMATITIS HERPETIFORMIS

ABSTRACT: Despite significant clinical, histologic, immunopathologic and other differences between typical cases of dermatitis herpetiformis (DH) and bullous pemphigoid (BP), the differential diagnosis may be difficult. Our direct immunofluorescent studies in 59 cases of DH and 134 cases of BP indicate the diagnostic value of immunopathologic studies.     

INTRAMUSCULAR LOW DOSE ALPHA-2B INTERFERON AND ETRETINATE FOR TREATMENT OF MYCOSIS FUNGOIDES

Background. Mycosis fungoides is a lymphoma of cutaneous origin characterized by a proliferation of cells with a T phenotype. Methods. In this pilot study, 13 men with mycosis fungoides in various stages were treated with alpha-2b interferon and etretinate. Results. In ten of them, such a therapy proved to be effective (7 complete responses, 3 partial responses), sometimes with prolonged remissions (up to 20 months, and still persistent) after suspension of the drugs. Conclusions. We chose low-dose interferon administration in order to prevent side effects, which are said to be dose-dependent. In our experience this is not true, but this drawback seems to be overcome by the very good, sometimes spectacular, response to this combination therapy, particularly in low stage forms of the disease. This fact, compared with results provided by other groups, prompts us to plan new research protocols based on associations of retinoids with different interferon types (or even associations of different interferons), because we believe they will have an important place in treatment of cutaneous T-cell lymphoma in future.     

DIAGNOSTIC EVALUATION OF THE LUPUS BAND TEST IN DISCOID AND SYSTEMIC LUPUS ERYTHEMATOSUS

Background. The usefulness of the lupus band test (lbt ) in the diagnosis of cutaneous lupus erythematosus remains controversial. The study was done to determine the sensitivity, specificity, and predictive value of the lbt in discoid lupus erythematosus (dle ) and systemic lupus erythematosus (sle ). Methods. During the study period, 32 patients with SLE and 28 patients with DLE were included. The final classification of cases was based on the American Rheumatism Association Criteria (ara ) for sle , histopathology, and consensus of the clinical staff. Thirty controls, 15 each for DLE and sle , were chosen from a group of patients, who had lesions clinically simulating dle and sle , but could be excluded by histopathology, laboratory tests, and follow-up. Histopathologic examinations and direct immunofluorescence (DIF) tests were done on lesional skin. Results. In dle , the sensitivity of the LE band test was 58% and the specificity 87%. The positive predictive value was 95% and negative predictive value was 32%. In sle the sensitivity was 93% and specificity 87%. The positive predictive value was 64% and negative predictive value 98%. Conclusions. The high negative predictive value of lbt in sle suggests that it is valuable in excluding diseases clinically similar to sle .     

STUDIES ON LOW DOSE CHLOROQUINE THERAPY AND THE ACTION OF CHLOROQUINE IN SYMPTOMATIC PORPHYRIA

Summary.— Eight patients with symptomatic porphyria (porphyria cutanea tarda symptomatica, PCT) were treated with small doses of chloroquin sulphate over periods ranging from 2 to 16 weeks. A good clinical and biochemical response was obtained in all but one patient, who relapsed after initial improvement. Manifestations of acute untoward reaction were seen in one patient only. It is concluded that low dose chloroquine therapy is both safe and effective in PCT.
Studies in porphyric rats indicate that the mechanism of action of chloroquine in PCT is probably not related, as has been suggested, to the formation of a chloroquine-porphyrin complex which damages liver cells and thereby leads to a reduction in hepatic porphyrin stores.
Chloroquine therapy led to increased urinary iron excretion and an increase in the desferrioxamine-chelatable iron in patients with PCT. It is suggested that the prolonged beneficial effects of chloroquine treatment may be related to reduction in a specific pool of liver iron.