Decreased numbers of circulating plasmablasts and differences in IgA1-plasmablast homing to skin in coeliac disease and dermatitis herpetiformis.
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Impetigo herpetiformis with postpartum flare-up: a case report.
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Impetigo herpetiformis is a rare dermatosis of pregnancy with typical onset during the last trimester of pregnancy and rapid resolution during the postpartum period. It is still a matter of debate whether it is a variant of pustular psoriasis or a separate entity. We report a case of impetigo herpetiformis with an earlier onset during the second trimester of pregnancy (G4 P2) and an atypical postpartum flare-up. Even though the case is not new per se, it reveals the natural course of this rare disease because the condition was neglected and given no oral treatment throughout the entire pregnancy. Consequently, the patient had a premature delivery with premature rupture of membranes, placental insufficiency, and intrauterine growth retardation. (+info)
Pupil cycle time and early autonomic involvement in ocular leprosy.
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Ocular complications of leprosy patients often develop insidiously and with few if any symptoms. This study involves measurement of the pupil cycle time (PCT) to evaluate the autonomic nerve system of the iris to determine the presence of subclinical intraocular involvement. The study included 19 lepromatous (LL), 19 borderline lepromatous (BL), and five borderline tuberculoid (BT) leprosy patients and involved 25 healthy volunteers, 10 patients with pulmonary tuberculosis and eight with Duhring disease. The PCT was measured in these groups. In all leprosy groups included in the study the PCT was higher than in the control groups. Moreover, the PCT of the leprosy patients without any intraocular involvement was higher than in the controls. These results show that in the ophthalmic examination of leprosy patients without any symptoms the fact that autonomic nerve system of the eye is affected by the leprosy can often be determined by measuring the PCT. (+info)
Small intestinal function and dietary status in dermatitis herpetiformis.
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Small intestinal morphology and function were assessed in 82 patients with dermatitis herpetiformis, 51 of whom were taking a normal diet and 31 a gluten free diet. Methods used were histopathological evaluation of jejunal mucosal biopsy specimens, quantitation of intraepithelial lymphocytes, cellobiose/mannitol permeability test, tissue disaccharidase values, serum antigliadin antibodies, and formal assessment of dietary gluten content by a dietician. There was no correlation between dietary gluten intake and the degree of enteropathy in the 51 patients taking a normal diet, whereas biopsy specimens were normal in 24 of the 31 patients on a gluten free diet, all previously having been abnormal. Eighteen patients on gluten containing diets had normal jejunal histology and in seven of these all tests of small intestinal morphology and function were entirely normal. Intestinal permeability was abnormal and serum antigliadin antibodies were present in most patients with enteropathy. Studies of acid secretion in seven patients showed that hypochlorhydria or achlorhydria did not lead to abnormal permeability in the absence of enteropathy. This study shows that a combination of objective tests of small intestinal architecture and function will detect abnormalities in most dermatitis herpetiformis patients, including some with histologically normal jejunal biopsy specimens. Nevertheless there is a small group in whom all conventional intestinal investigations are entirely normal. (+info)
The inhibition of polymorphonuclear leukocyte cytotoxicity by dapsone. A possible mechanism in the treatment of dermatitis herpetiformis.
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The effect of the sulfone compound 4,4'-diaminodiphenyl sulfone (dapsone) on normal human polymorphonuclear leukocytes (PMNL) has been investigated in vitro. The drug has a dramatically beneficial effect in dermatitis herpetiformis in which the PMNL and immune complexes has been stressed to be of importance for the development of the skin lesions. Pruritus disappears and the inflammatory eruptions clear within a few days of starting therapy. The effect of dapsone has been evaluated on the different stages of phagocytosis. Using dapsone concentrations (1-30 mug/ml) comparable with those found after therapeutic doses, we have found that the drug interferes primarily with the myeloperoxidase (MPO)-H(2)O(2)-halide-mediated cytotoxic system in the PMNL. No effect was observed on random locomotion, chemotaxis, phagocytic ingestion, oxidative metabolism, or the release of lysosomal enzymes. Kinetic studies in a cell-free system with purified MPO revealed a competitive type of inhibition using varying concentrations of NaI. Furthermore, the inhibition resulted in reduced candidicidal activity during phagocytosis of Candida albicans, and reduced cytotoxicity to adjacent mammalian cells measured as the (51)Cr release from virus-induced lymphoma cells. Because the MPO-H(2)O(2)-halide system not only fulfills the antimicrobial activity but is suggested to be a modulator of the inflammatory reaction as well, the action of dapsone in dermatitis herpetiformis may in part be explained by its effect on this system. (+info)
Dermatitis herpetiformis sera or goat anti-transglutaminase-3 transferred to human skin-grafted mice mimics dermatitis herpetiformis immunopathology.
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Letter: Pemphigus herpetiformis of age of onset at 6 years.
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INTRODUCTION: Pemphigus herpetiformis is a rare entity that combines the clinical features of dermatitis herpetiformis with the immunologic and histological features of pemphigus. It generally affects adults and pediatric cases are uncommon. We report a case of pemphigus herpetiformis in a 6-year-old girl treated successfully with dapsone. OBSERVATION: A 6-year-old girl presented with a pruritic, erythematous and vesiculous eruption. Histological examination showed acantholysis of the medial and superficial parts of the epidermis. Direct immunofluorescence study revealed cell surface immunoglobulin G and C3 deposits and staining on the dermo-epidermal junction. The child was diagnosed as having pemphigus herpetiformis. DISCUSSION: Pemphigus herpetiformis produces clinically a pruritic and atypical eruption that resembles dermatitis herpetiformis, with standard histology and immunofluorescence patterns recalling those of pemphigus. Considered to have a good prognosis, it responds well to dapsone and/or to low dose oral corticosteroids. The age range of onset varies from 31 to 81 years according to studies, without a male or female predilection. This is a report of a 6-year-old girl presenting with pemphigus herpetiformis, successfully treated with dapsone for two years with no relapse. (+info)