PubMed Health⌕ Search

Biomedical subjects

G P Haffenden

Publications and source records attributed to G P Haffenden.

17 recordsLinked to original sources
PubMed1988

Immunofluorescent studies in ocular cicatricial pemphigoid.

Twenty nine patients with cicatrizing conjunctivitis were studied; 17 with a clinical diagnosis of cicatricial pemphigoid, five with a clinical diagnosis of pseudopemphigoid caused by long-term application of topical medication and seven who had a cicatrizing conjunctivitis from other causes. Biopsies from clinically uninvolved bulbar conjunctiva were taken for direct immunofluorescence and blood was taken for indirect immunofluorescence using normal human conjunctiva, oral mucosa and skin as substrates. On direct immunofluorescence, in vivo bound immunoglobulins were found along the basement membrane in 10 of the 17 patients with cicatricial pemphigoid, one of the five with pseudopemphigoid and two of the seven with a cicatrizing conjunctivitis associated with other diseases. Circulating anti-basement membrane zone antibodies were found only when conjunctiva was used as a substrate. These were present in seven of the patients with cicatricial pemphigoid, three of those with pseudopemphigoid and two of those with a cicatrizing conjunctivitis caused by other diseases. These results indicate that direct immunofluorescence is a useful, but not absolute diagnostic marker for ocular cicatricial pemphigoid. The results in the pseudopemphigoid group argue that this is an immunologically mediated disorder indistinguishable from spontaneous cicatricial pemphigoid and probably triggered by the drugs. The presence of circulating antibodies should allow for precise identification of the antigen involved in cicatricial pemphigoid using SDS electrophoresis and Western blot analysis.

Adult↗
PubMed1987

Gliadins bind to reticulin in a lectin-like manner.

It has previously been reported that gliadins bind to reticulin in tissue sections. Three lines of evidence are reported in this study which indicate that the gliadins bind to reticulins because they are lectins which bind to sugars expressed on glycoproteins in reticulin and other sites. First, immunofluorescence studies on tissue sections showed that although gliadin binding is largely confined to areas rich in reticulin, it is, nonetheless, also seen in one or two other sites devoid of reticulin. Second, by using fluorescein-labelled lectins of known specificity, it has been shown that the areas to which gliadins bind in tissue sections (including those sites devoid of reticulin) are rich in particular sugars. Third, it has been shown that one of these sugars, alpha-D-mannose, partially inhibited gliadin binding to tissue sections.

Animals↗
PubMed1987

Experience with a gluten free diet in the treatment of linear IgA disease.

A study was undertaken to determine whether the skin eruption of linear IgA disease (LAD) was gluten dependent. Six patients with LAD were treated with a gluten free diet (GFD) for an average period of 33 months (range 19-48). Although one patient with LAD had an enteropathy which was clearly gluten sensitive, there was no convincing evidence that the rash of any of the patients responded to a GFD. Four of the six patients showed no significant alteration in their drug requirements. The remaining 2 patients showed a fall in minimum drug requirement but there was no increase after gluten challenge indicating that they were entering spontaneous remission. This contrasts to the situation in dermatitis herpetiformis, where both the rash and the enteropathy are gluten dependent. These data add further to the evidence that LAD and dermatitis herpetiformis are separate entities.

Adult↗
PubMed1985

Skin diseases and the dry eye.

Patients with oral cicatricial pemphigoid, who present to an oral medicine department, should be examined by an ophthalmologist because over 65 per cent have been found to have an asymptomatic cicatrising conjunctivitis. Early detection and treatment of these patients may help to slow down the scarring process that eventually leads to a dry eye and blindness. The same is true for a group of patients who present to the dermatologist with a non-scarring bullous dermatosis distinguished by the presence of a linear deposit of IgA along the dermo-epidermal junction of uninvolved skin examined by direct immunofluorescence. The cicatrising conjunctivitis found in about 50 per cent of adults with this condition is indistinguishable from that of cicatricial pemphigoid. Ten per cent of patients with cicatricial pemphigoid affecting mucous membranes alone were found to have linear deposits of IgA along the dermo-epidermal junction of uninvolved skin. This suggested that there may be a common pathogenic pathway with varying clinical expression in these groups of patients and presents new possibilities for therapy in some patients with cicatricial pemphigoid.

Adolescent↗
PubMed1984

The relationship between linear IgA disease and benign mucous membrane pemphigoid.

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.

Adult↗
PubMed1984

Evidence that the IgA in patients with linear IgA disease is qualitatively different from that of patients with dermatitis herpetiformis.

A previous study using immunofluorescent techniques showed J-chain to be present in the uninvolved skin of patients with papillary IgA dermatitis herpetiformis (DH) in a distribution that was coextensive with the IgA. This implied that the IgA was dimeric and of mucosal origin. In this study, fifteen patients with papillary IgA deposits, fifteen with homogeneous-linear (HL) IgA deposits and four patients with granular-linear (GL) IgA deposits were tested for the presence of in vivo bound J-chain. All fifteen patients with papillary IgA deposits and all four with GL IgA deposits had J-chain staining coextensive with the IgA. However, only one of fifteen patients with HL IgA deposits demonstrated in vivo bound J-chain that could not be accounted for by coexisting IgM deposits. These findings indicate that the IgA in patients with HL deposits is qualitatively different from that in patients with papillary and GL IgA deposits and makes the distinction between the two types of linear fluorescence particularly important.

Adult↗
PubMed1983

Increased incidence of malignancy in dermatitis herpetiformis.

A retrospective study of 109 patients with dermatitis herpetiformis showed that malignant tumours had developed in seven patients, the expected incidence being 2.93, giving a relative risk of 2.38. In three of the seven patients the malignancy was a lymphoma, giving a relative risk of 100 for this tumour (expected incidence 0.03). In six of the seven patients who developed malignancies small-intestinal biopsy specimens were macroscopically abnormal, giving a relative risk of 4.22 in this group, which is similar to that reported in adult coeliac disease. Patients treated with a gluten-free diet appeared to have a reduced risk of developing malignancy compared with those taking a normal diet (relative risk with gluten-free diet 1.01 and with normal diet 3.09). A small subgroup of eight patients with linear IgA dermatitis herpetiformis were also studied: three developed malignant disease and in one the tumour was a lymphoma.

Dermatitis Herpetiformis↗
PubMed1983

Histology of linear IgA disease, dermatitis herpetiformis, and bullous pemphigoid.

The histologic appearances of cutaneous biopsy specimens from 30 patients with linear IgA disease with a continuous band of IgA along the basement membrane, four patients with a linear pattern of granular IgA along the basement membrane, 26 patients with dermatitis herpetiformis who had IgA in the papillary dermis, and 23 patients with bullous pemphigoid who had IgG and/or C3 along the basement membrane were compared. Those with linear and granular IgA and dermatitis herpetiformis differed from those with bullous pemphigoid in five respects. Multiple microabscesses and fibrin at tips of papillae and leukocytoclasis were less common in bullous pemphigoid, whereas a dense infiltrate of eosinophils in and below bullae and a linear infiltrate of eosinophils along the basement membrane were more common in bullous pemphigoid. Also, multilocular bullae and acantholysis were more common in dermatitis herpetiformis than in bullous pemphigoid. Linear IgA disease differed from dermatitis herpetiformis in two respects. Acantholysis and fibrin at the tips of papillae and leukocytoclasis were more common in dermatitis herpetiformis. The specimens from patients with granular IgA did not differ significantly from those with linear IgA or dermatitis herpetiformis. The appearances of biopsy specimens of patch tests with potassium iodide taken from 11 patients with dermatitis herpetiformis and linear or granular IgA disease were similar to those taken from spontaneous lesions.

Adult↗
PubMed1983

Skin biopsies in relatives of patients with dermatitis herpetiformis.

Out of 109 patients with dermatitis herpetiformis (DH) only one was known to have a first-degree relative with the disease. This is in keeping with previous reports that the familial incidence of DH is low. Study of the family in which two siblings were affected showed both to have HLA-B9 whereas a third, unaffected sibling did not have this antigen. This finding supports the view that both genetic and environmental factors are involved in the pathogenesis of DH, 20 first-degree relatives of 10 other patients with DH had skin biopsies examined by immunofluorescence for the presence of IgA, but none proved positive.

Adolescent↗
PubMed1982

Linear IgA disease in adults.

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.

Adolescent↗
PubMed1982

Ultrastructural localization of IgA deposits in adult linear IgA disease.

Two cases of adult linear IgA disease are reported. They demonstrate the two sites of deposition of IgA at an ultrastructural level: sub-basal lamina and lamina lucida. This finding supports the view that there are subgroups of this disease. Clinical presentation and histological appearance of lesional skin are misleading, and the immunofluorescent appearance is unhelpful in predicting the ultrastructural localization of the IgA deposit.

Aged↗
PubMed1980

The potassium iodide patch test in the dermatitis herpetiformis in relation to treatment with a gluten-free diet and dapsone.

The potassium iodide patch test was studied in twenty-six patients with dermatitis herpetiformis. Histological assessment was found more sensitive than clinical. All of five patients with active disease and not on treatment had a positive test, whereas only two of six patients taking a gluten-free diet (GFD) and one of eight taking dapsone were positive. In another two patients taking a GFD, but in whom the diet had not been strict, the test was positive. All three patients in remission and both patients with the linear pattern of IgA (but with active disease) were negative. Immunofluorescence studies showed no difference in the presence, quantity, or distribution of immunoglobulin, complement or fibrinogen between the patch test site and uninvolved skin, or in the uninvolved skin between patients with and without active lesions.

Dapsone↗
PubMed1978

Immunoglobulin and complement in normal skin.

Cryostat sections of normal skin from 57 white adults were examined by direct and indirect immunofluorescence for immunoglobulins, complement factors, and transferrin. The results for basement membrane zone (BMX) were significantly different for the 11 face and 46 non-face biopsies: in the face, IgM was found in five, IgG in two, IgA in one, and C3 in none, whereas, in non-face, IgM was present in six, IgG in none, IgA in one, and C3 in five. The results for dermal vessel walls (DV) were not apparently different for face and non-face; in the 57 biopsies IgM was present in one, IgG in none, IgA in one, and C3 in one. The 11 biopsies from the face and 26 of the non-face biopsies were examined further. No IgD or C4 was identified, but one case (scalp) showed BMZ Clq, properdin, and transferrin, and in two cases (one face, one non-face) DV properdin was found. Cytoid bodies (IgM and IgA) were present in moderate numbers in one case; all other positive reactions were finely granular.

Adult↗
PubMed1977

Alpha-1-antitrypsin bodies in the liver.

The cytoplasmic bodies in hepatocytes thought to indicate possession of the Z allele for alpha 1-antitrypsin deficiency were found in necropsy in 10 of 64 adults with cirrhosis, four of nine with hepatic fibrosis, and four of 15 with hepatocellular carcinoma. They were also found in six of 76 adults with severe panacinar emphysema, and in four of a control series of 110 adults with neither emphysema nor liver disease. The association of the bodies with each of the three liver diseases was statistically significant, but the association of the bodies with emphysema was not. It is considered probable that heterozygous (PiMZ) alpha 1-antitrypsin deficiency is associated with an increased incidence of cirrhosis, hepatic fibrosis, and hepatocellular carcinoma.

Carcinoma, Hepatocellular↗
PubMed1977

Aetiology of cirrhosis, hepatic fibrosis, and hepatocellular carcinoma.

Histological study of 69 cases of cirrhosis, 9 of severe generalised hepatic fibrosis, and 19 of hepatocellular carcinoma showed an association with alcohol, hepatitis B surface antigen (HBsAg) or a1-antitrypsin bodies in, respectively, 41 (cirrhosis), 5 (fibrosis), and 9 (carcinoma). Eight of the cirrhotic cases and two of the carcinoma cases had double associations, HBsAg being present in all. Torcein and aldehyde fuchsin staining gave both false positive and false negative results when compared with immunofluorescence and immunoperoxidase methods for HBsAg. Large amounts of copper were found in four cirrhotic livers, and moderate amounts in 13: the diagnostic value of copper staining is questioned.

Alcoholism↗