A multi-centre study is described in which thirty-five adult patients with papillary IgA dermatitis herpetiformis (DH) were compared with forty-two patients with linear IgA deposits, of whom thirty-four had homogeneous-linear (HL) and eight had granular-linear (GL) IgA deposits. The three groups were similar with regard to age of onset, presence of circulating immune complexes and auto-antibodies, incidence of spontaneous remission, histology of lesional skin and response to dapsone. There was a female predominance in the HL group in contrast to the male predominance in the other two. It was not possible to diagnose the HL group clinically. Some patients had a rash typical of DH whilst others resembled pemphigoid. In the majority, however, no specific diagnosis could be made with confidence. The GL group clinically resembled the DH group. The incidence of positive potassium iodide patch tests was greater in the DH group than in the other two. An associated enteropathy was found in 24% of patients in the HL group, 30% of patients in the GL group and 85% of patients in the DH group. Fifty-six percent of HL patients had HLA-B8 compared with 50% in the GL group and 88% in the DH group. Patients with linear IgA deposits may not be a uniform group, but until they can be divided into specific subgroups (e.g. by ultrastructural localization of the deposit or by response to a gluten-free diet) we propose that the term adult linear IgA diseases should be used to distinguish these patients from those with papillary IgA deposits.
threshold dose, laser irradiation has an inhibitory effect on tumour growth as opposed to its simple thermal effect. Our experience suggests, however, that this potential '.cure" cannot be achieved in clinical practice. Simple laser irradiation, as opposed to laser irradiation after previous photosensitisation of tumour tissue with haematoporphyrin dye,12 therefore holds little hope of cure except, perhaps, in rare instances of slowgrowing polypoid tumours within the bronchial lumen. Surgery, however, is the treatment of choice for such growths.
Seventy-eight patients with dermatitis herpetiformis have been followed up for periods ranging from 3 to 14 years (mean 7.4). Forty-two patients were treated with gluten-free diet (GFD) and thirty-six took a normal diet (ND). Thirty of the forty-two (71%) taking the GFD were able to discontinue drugs previously needed to control their rash compared with five (14%) of the thirty-six patients taking a ND. The mean time taken to reduce drug requirements for patients taking a GFD was 8 months (range 4-30), and for stopping drugs, 29 months (range 6-108). The incidence of macroscopic abnormality of the small intestine decreased from 69 to 15%, and the mean intra-epithelial lymphocyte count decreased significantly in those patients taking a GFD, whereas there was no significant change in patients taking a ND. The improvement in the skin and intestinal lesions was related to the strictness of the GFD.
Nine out of thirty-four patients with linear IgA disease (LAD) had oral ulceration. Four out of seven of these patients who were examined by an ophthalmologist had changes of a cicatrising conjunctivitis indistinguishable from those of benign mucous membrane pemphigoid (BMMP). Three of these patients gave no history of ocular symptoms up to the time of examination. These findings indicate a need for oral and ophthalmological assessment in all patients with LAD. Twenty-seven patients with a diagnosis of BMMP were also studied. Nine presented with oral symptoms alone, nine with ocular symptoms alone, seven with oral and ocular symptoms, and two with cutaneous lesions in addition to oral and ocular symptoms. All the patients were examined by the same ophthalmologist. Six of the nine patients who presented with oral symptoms alone had signs of a cicatrising conjunctivitis. Four of these six patients had the clinical pattern of erosive gingivitis which was not previously thought to be associated with a cicatrising conjunctivitis. There is a similar need, therefore, for an ophthalmological assessment in all patients presenting with oral BMMP. Three of the twenty-seven patients with BMMP had homogeneous-linear deposits of IgA in uninvolved skin. The finding of linear IgA deposits in the skin of these patients with only mucous membrane lesions, and the finding that patients with LAD have a high incidence of oral and conjunctival lesions, raise the possibility of a common pathogenic pathway but with varying clinical expressions in these two groups.
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