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Biomedical subjects

H Bergoend

Publications and source records attributed to H Bergoend.

At least 19 recordsLinked to original sources
PubMed1996

IgE autoantibodies directed against the major bullous pemphigoid antigen in patients with a severe form of pemphigoid.

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized in part by circulating and tissue-bound IgG autoantibodies directed against the basement membrane zone. In addition, most of the patients with BP have increased serum IgE levels which seem to be correlated with the disease activity, whereas the presence of circulating anti-basement membrane zone IgE Abs has been reported in some patients. To elucidate whether IgE-dependent mechanisms play a role in the physiopathology of BP, we looked for the presence of IgE Abs specifically directed against the major BP Ag (BPAg1) in sera of BP patients at the onset and after remission of the disease. A radioimmunoassay and a 55-kDa recombinant protein (rBP55) obtained from a cDNA sequence, encoding the C-terminal region of the BPAg1 and containing the BPAg1 immunodominant epitopes, were used. Anti-rBP55 IgE Abs were found in 12 of the 19 sera tested. When the patients were divided into two groups according to the disease severity, anti-rBP55 IgE Abs were found only in patients with a severe form of the disease. Cytophilic IgE was detected on approximately 20% of peripheral blood eosinophils purified from BP patients. Immunohistochemistry studies suggested that some of the IgE-bearing cells in the lesional skin of BP patients are eosinophils. Immunostaining experiments revealed the existence of FcepsilonRI on both peripheral blood and tissue eosinophils. Taken together, these results suggest that IgE-dependent mechanisms could participate in the constitution of the lesions in BP.

Antibody Specificity↗
PubMed1996

Linear and whorled nevoid hypermelanosis versus incontinentia pigmenti: is pigmentary incontinence really a distinctive feature?

This report describes a 3-year-old boy who presented with skin lesions characterized by multiple streaks of hyperpigmentation following Blaschko's lines since 6 weeks of age. The clinical features are consistent with the diagnosis of linear and whorled nevoid hypermelanosis (LWNH), but differ substantially from this entity by the pigmentary incontinence observed on both the paraffin and electron microscopy sections. Despite the presence of pigmentary incontinence, the diagnosis of incontinentia pigmenti (IP) can be ruled out on the basis that the patient is a male, with none of the characteristic anomalies present in this syndrome, and that he never presented with inflammatory or eruptive skin lesions. This observation suggests that pigmentary incontinence may not differentiate IP from LWNH as conclusively as previously reported in the literature.

Age of Onset↗
PubMed1995

[Mycoplasma infection with Stevens-Johnson syndrome and antiphospholipid antibodies: apropos of 2 cases].

We report two cases of Mycoplasma pneumoniae infections associated with Stevens-Johnson syndrome and antiphospholipid antibodies. Such an association has been noted once in the literature. The relationship between antiphospholipid antibodies and Stevens-Johnson syndrome and others cutaneous manifestations of infections diseases is discussed. Though mainly described in systemic lupus erythematosus and autoimmune diseases, anticardiolipin antibodies and lupus anticoagulant have been found in many infectious disorders. But in the latter conditions, they have been considered by many authors as "non pathogenic" or "non prothrombotic" on epidemiologic and immunologic data. We suggest that antiphospholipid antibodies could possibly play a role in their pathogenesis especially as the mechanisms are not to date clearly understood.

Antibodies, Antiphospholipid↗
PubMed1995

[Hamartomatous dysplasias with hemihypertrophy. 2 cases including one with Proteus syndrome].

BACKGROUND: Hamartomatous dysplasias with hemihypertrophy are a part of several congenital diseases, sometimes difficult to classify when their symptomatology is relatively poor. CASE REPORTS: Case 1. A girl, 11 year-old, suffered from gigantism of the feet, left hemihypertrophy of the limbs, scoliosis and kyphosis. X-rays showed frontal hyperostose, bony and soft tissues overgrowth involving the left limbs and pelvis with hip dislocation and coxa valga. The vertebrae were hypertrophic and dysplastic. No tumor could be found. Case 2. A girl, 18 year-old, suffered from left hemihypertrophy of limbs and face associated with homolateral cutaneous hemangioma. X-rays showed enlargement of bone and soft tissue. No tumor or venous abnormalities were observed. CONCLUSION: A diagnosis of Proteus syndrome was made in the first patient while the second patient was considered to have Klippel-Trenaunay syndrome even though hemangiomatous involvement has been seen in Proteus syndrome.

Adolescent↗
PubMed1995

Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal.

Keratoderma with scleroatrophy of the extremities, also referred to as Huriez syndrome, is a rare, autosomal dominant condition, first described in 42 of 132 members of two families from northern France. The term sclerotylosis was proposed because of the pseudosclerodermatous appearance of the hands and digits. The distinctive feature of this syndrome is the risk of the development of squamous cell carcinoma on affected skin. Since the initial description of this disease, three other families, and possibly a fourth, have been reported. In the present study, we reassessed the clinical, pathological and genetic data in 114 members of one of the two original families, of whom 27 were affected by this syndrome.

Acitretin↗
PubMed1995

Dermatomyositis with follicular hyperkeratosis.

We report 2 cases of dermatomyositis with follicular hyperkeratosis (FHK) in children. They occurred in a 10-year-old Vietnamese girl and a 9-year-old Caucasian boy. The girl's FHK disappeared after 2 months of treatment. The boy presented, 15 months after the onset of his dermatomyositis, with a generalised FHK which lasted for 6 months. FHK can appear before, during or after dermatomyositis. It is more often generalized but can be localised. Erector pili myositis and ostial hyperkeratosis may be the explanation. The prognostic value of FHK in dermatomyositis is unknown. This manifestation, initially considered to be more frequent in the Far East, is not as rare in Western countries as the few reported cases suggest.

Child↗
PubMed1995

[Pemphigoid mimicking epidermolysis bullosa acquisita].

INTRODUCTION: Subepidermal autoimmune bullous dermatoses form a clinical entity for which there is not always an individualized clinical and pathological description. CASE REPORT: A patient presented with bullous skin disease of atypical nature. There was an almost total desepidermization of the legs, vast areas of erosion on the trunk and arms with a Nikolski sign in an area of healthy skin, buccal involvement, multiple milium cysts and ungueal dystrophies with nail loss. DISCUSSION: This clinical presentation in this patient suggested acquired bullous epidermolysis. However, according to the recently defined clinical criteria for pemphigoid, the probability of correct diagnosis of pemphigoid was greater than 95 p. 100 since nearly three fourths of the major criteria were present. This diagnosis was confirmed by reference techniques (electron microscopy, indirect electron immunomicroscopy and immunoblotting). Thus, bullous autoimmune diseases of the dermoepidermal junction can be reliably differentiated on the bases of the clinical features, together with direct and indirect immunofluorescence on salt-split skin.

Aged↗
PubMed1995

[Cutaneous pseudolymphoma during treatment of rheumatoid polyarthritis with low-dose methotrexate].

INTRODUCTION: Over the last three years, there have been over twenty case reports of lymphoma in patients given low-dose methotrexate for rheumatoid arthritis. We observed the first case of cutaneous pseudolymphoma. CASE REPORT: A 56-year-old man had been treated with methotrexate (15 mg/day) for 6 years due to rheumatoid arthritis. He developed three isolated papulonodular ulcerations on the limbs. The histology and immunohistochemical examinations demonstrated T and B lymphoplasmocyte infiltration without epidermotropism nor destruction of the annexes. Immunolabelling for anti-Epstein-Barr virus was negative. There was a IgG lambda type monoclonal hypergammaglobulinaemia, Bence-Jones proteinuria and an increase in beta 2-microglobulin. The thoracoabdominal scan, bone marrow biopsy and gallium scintigraphy were normal. There was no sign of a Gougerot-Sjögren syndrome nor of a Felty syndrome. The skin lesions and the Bence-Jones proteinuria disappeared rapidly after withdrawal of methotrexate. There has been no recurrence with a follow-up of 16 months. DISCUSSION: The diagnosis of pseudolymphoma was retained on the basis of the clinical features, the histological and immunohistochemical evidence and especially on the clinical course after methotrexate withdrawal, i.e. spontaneous regression of the lesions within 3 weeks. A similar course has been observed in three cases of lymphocyte proliferation suggesting that this immunosuppressor would be the most probable causative agent. Lymphocyte proliferation, mainly B-cell lymphomas in haematopoietic organs occurring under methotrexate administration have occurred mainly in patients with rheumatoid arthritis. Three cases have also been described in patients with dermatomyositis, but none have been reported in patients with psoriasis. This would suggest that cofactors involved in these autoimmune diseases could also have an effect: immunodepression, potentialization due to associated treatment (corticosteroids), Epstein-Barr virus... CONCLUSION: Data on these observations should be combined in order to analyse the question of the safety of low-dose methotrexate in these patients.

Arthritis, Rheumatoid↗
PubMed1995

[Sclerosing lipogranuloma in a male].

INTRODUCTION: Major trauma was the only aetiologic factor found in a male patient presenting sclerosing lipogranuloma. This condition is well-known in women but is rarely described in men. CASE REPORT: A 31-year-old non-obese man with no sign of gynaecomasty presented with well-delimited plaques of pigmented and sclerosous tissue located at the pubis and both breasts as well as hard nodules of the scrotum and the penis. The lesion developed progressively after a major trauma involving the anterior aspect of the thorax and the public area. An inflammatory border persisted eight years after the initial trauma. Histological examination of a biopsy specimen showed evidence of adipocyte necrosis with lipophagia and lipogranuloma. No foreign body was found. DISCUSSION: The diagnosis of post-trauma cytosteatonecrosis was retained on the basis of the clinical history and the clinical, histological and radiographic evidence. This condition is a well-identified clinical entity which is sometimes confounded with false panniculitis resulting from self-injection of oily products. The endogenous origin of the fatty acids found within the cytosteatonecrosis tissue has recently been proven. Usual localizations are the breast in women and genital organs in men. Favouring factors in women include obesity, age, anticoagulants, frequency and intensity of trauma. The exceptional localization in our patient was strongly related to the earlier trauma.

Adipose Tissue↗
PubMed1994

[Perifolliculitis capitis abscedens and suffidiens. Efficacy of isotretinoin].

Perifolliculitis capitis abscedens et suffodiens is a rare, chronic and suppurative scalp disease. The treatment of this condition is difficult. CASE REPORT. We report a case successfully treated with isotretinoin at the dose of 2/3 mg/kg/day during eight month. DISCUSSION. The main difficulty is the maintenance of remission after drug withdrawal, so, duration of treatment has to be defined. A eight month's therapy seems to constitute the minimum duration in this pathology. A second course of isotretinoin is still possible if relapse occurs. CONCLUSION. A long course of isotretinoin can be considered as the most effective treatment in perifolliculitis capitis abscedens et suffodiens.

Adult↗