Dermatoses In Adults Research Articles

An Analysis of 24 Patients with IgA Deposition at the BMZ

A study of 24 patients with IgA deposition at the BMZ of the skin showed that five conditions could be recognized: 1) linear IgA bullous dermatosis in adults (LAD, 7 cases); 2) linear IgA and IgG bullous dermatosis in adults (LAGD, 10 cases); 3) chronic bullous disease of childhood (CBDC, 3 cases); 4) dermatitis herpetiformis (DH, 1 case), and 5) systemic lupus erythematosus (SLE, 3 cases). Histopathologically, 5 of 7 patients with LAD were similar to the DH group, but 7 of 10 patients with LAGD were similar to the BP group. Half the patients with LAD and LAGD had oral lesions, and most of them had excellent responses to dapsone and Tripterygium Wilfordii, but the patients with CBDC did not respond to these treatments. In the patients with LAD and LAGD, the positivity rates of IgA anti-BMZ antibodies examined by indirect immunofluorescence (IIF) on intact skin and NaCl split skin were 41% and 64%, respectively. The heterogeneity of the histopathologic pictures of LAD and LAGD, the incidence of DH, and the value of using NaCl split skin for IIF are discussed.

Immunoelectronmicroscopic differentiation of linear IgA bullous dermatosis of adults with coexistence of IgA and IgG deposition from bullous pemphigoid

The differentiation between linear IgA bullous dermatosis (LABD) and bullous pemphigoid (BP) is sometimes difficult in patients who have both IgA and IgG deposition in a linear pattern at the basement membrane zone. We address whether two cases of acquired subepidermal blistering disease with coexistence of IgA and IgG deposition in a linear pattern at the basement membrane zone are LABD or BP. The two cases were investigated by immunoelectron microscopy and compared with two typical cases of LABD. In both cases, the deposition of IgA and IgG was ultrastructurally localized below the lamina densa in close association with anchoring fibrils, as was seen in two cases of typical LABD. These findings indicate that our two cases of acquired blistering disease with co-existence of IgA and IgG deposition are LABD, rather than BP.

Linear IgA bullous dermatosis of adults

The skin disorder designated as linear IgA bullous dermatosis (LABD), identified in 1979 (l), had been for many years a matter of controversy. This controversy was due mainly to its clinical diversity, as some cases closely resemble dermatitis herpetiformis (DH), some cases are indistinguishable from bullous pemphigoid (BP), and still other cases combine features of both (2). No wonder that some authors considered this disease as DH or its variety (3 6), others as BP (7,8), and yet others as intermediate or mixed forms of DH and BP (2,9). In support of the relationship with DH were the skin immune deposits of immunoglobulin A (IgA) class and, as a rule, a dramatic response to sulfones. Supporting the relationship with BP were the linear pattern of IgA deposits along the basement membrane zone (BMZ) and, occasionally, the concomitant presence of complement in the same distribution (1,2,9). Furthermore, at least in some cases, the ultrastructural depositions of IgA, in the lamina lucida, similar to that in BP, suggested some relationship between LABD and BP (10 12). In a proportion of cases of LABD, the linear IgA deposits are found below the basal lamina (12-14) or in both locations (6,11,12,15). The main difference from BP is the IgA-class skin deposits, papillary microabscesses in the histology, and, as a rule, a good response to sulfones. The most important reason for separation of LABD from DH is that LABD is not associated with gluten-sensitive enteropathy and, as could be expected, a glutenfree diet is not effective treatment for LABD (16). Recognition of LABD as a distinct entity, despite the clear-cut differences from DH and BP, took a long time until more data on immunoelectron microscopy, serology, genetics,

Linear IgA bullous dermatosis of adults and children: an immunoelectron microscopic study.

The ultrastructural localization of the IgA deposits in the skin of 15 patients with linear IgA bullous dermatosis of adults (LAD), 13 with chronic bullous dermatosis of childhood (CBDC) and three with childhood cicatricial pemphigoid (CCP) were studied. The site of the antigen was determined using sera from two LAD, 13 CBDC and two CCP patients. In all 31 patients the IgA was located predominantly below the lamina lucida (sublamina densa). Similarly, the indirect immunoelectron microscopic studies demonstrated the antigen to be present at the same site, below the lamina densa. This suggests that in linear IgA bullous dermatosis the antibody reacts with the antigen in the sublamina densa region of the basement membrane zone.

Circulating antibodies to gliadin subfractions in dermatitis herpetiformis and linear IgA dermatosis of adults and children.

Dermatitis herpetiformis (DH) and coeliac disease (CD) are linked but their association with linear IgA dermatosis (LAD) is unclear. Thirty‐seven patients with DH and 27 with linear IgA dermatosis were investigated, of which 23/37 DH patients and 1/10 LAD patients had small intestinal enteropathy. Elevated IgG and IgA gliadin antibodies were found in the DH patients with enteropathy (CD). IgG gliadin antibodies in DH patients were directed against alpha, beta, gamma and omega gliadin subfractions. Elevated IgA gliadin antibodies in the adult LAD patients (n= 14) suggests that this condition may be associated with enteropathy or an IgA diathesis.

A comparative study of benign chronic bullous dermatosis of childhood and linear IgA dermatosis of adults

Benign chronic bullous dermatosis of childhood (BCBDC) is an apparently self‐limiting vesiculo‐bullous disease showing linear IgA deposits in the basement membrane zone and which seems to occur exclusively in children. Its relationship to linear IgA dermatosis of adults (LAD) is uncertain and for this reason we studied 28 patients with BCBDC (onset before puberty) and compared them with 18 patients with LAD (onset after puberty). No significant clinical, HLA, histological or immunopathological differences were found between the two disorders. Surprisingly, nine patients in each group had conjunctival scarring which in some produced a cicatricial pemphigoid‐like appearance and in one case of BCBDC resulted in blindness. Remissions with loss of IgA from the skin occurred in patients from both groups. However, the disease duration varied considerably (BCBDC 2–28 and LAD 1–40 years) with six BCBDC patients having had their disease for over 10 years and five of them passing puberty. All patients (six BCBDC, eight LAD) studied by immuno‐electronmicroscopy showed localization of IgA beneath the basal lamina. Direct and indirect immunofluorescence studies using suction blisters also gave similar results in both groups. As a result of this study many of our former views on BCBDC have been revised and we now believe that both BCBDC and LAD are manifestations of the same disease.

IgA linear dermatosis of childhood (chronic Bullous disease of childhood)

Of twenty-seven cases of subepidermal blistering disease of children twelve corresponded clinically, histologically and immunologically to dermatitis herpetiforms of adults, six to bullous pemphigoid, and eight to chronic bullous disease of childhood (CBDC), i.e. IgA linear dermatosis. This latter disease seems to be a distinct entity, different from both dermatitis herpetiformis and bullous pemphigoid, and is characterized immunopathologically by linear IgA deposits at the basement membrane zone. These cases usually do not show intestinal involvement and respond well to combined treatment with sulphones and corticosteroids, whereas sulphones or sulphapyridine alone are, even in very high doses, not sufficient for full control of the disease. CBDC or IgA linear dermatosis of childhood may be regarded as a counterpart of IgA linear dermatosis of adults.

IgA linear dermatosis of childhood (chronic bullous disease of childhood)

Of twenty-seven cases of subepidermal blistering disease of children twelve corresponded clinically, histologically and immunologically to dermatitis herpetiforms of adults, six to bullous pemphigoid, and eight to chronic bullous disease of childhood (CBDC), i.e. IgA linear dermatosis. This latter disease seems to be a distinct entity, different from both dermatitis herpetiformis and bullous pemphigoid, and is characterized immunopathologically by linear IgA deposits at the basement membrane zone. These cases usually do not show intestinal involvement and respond well to combined treatment with sulphones and corticosteroids, whereas sulphones or sulphapyridine alone are, even in very high doses, not sufficient for full control of the disease. CBDC or IgA linear dermatosis of childhood may be regarded as a counterpart of IgA linear dermatosis of adults.