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Biomedical subjects

H Bergoend

Publications and source records attributed to H Bergoend.

At least 37 records · Page 2Linked to original sources

[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↗

[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↗

[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↗

[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↗

[Buruli ulcer in a Zairian woman with HIV infection].

INTRODUCTION: Isolated cutaneous mycobacterial infection is exceptional in the acquired immunodeficiency syndrome (AIDS). We report a case of Buruli ulcer observed in a Zairan female infected with the human immunodeficiency virus (HIV). CASE REPORT: An HIV positive female patient was hospitalized for deep skin ulceration with detached and necrotic borders on the posterior aspect of the lower limb. Histological examination of a surgical biopsy showed numerous acid-fast bacilli identified as Mycobacterium ulcerans by thin layer gas chromatography. The clinical course was favourable with a three-drug antibiotic therapy replaced by rifampicin-clarithromycin after three months. The patient's immune status did not change for two years with a CD4 count of 500/mm3. The patient was negative for P24 antigen. DISCUSSION: While it is relatively easy to diagnose Buruli ulcers, identifying the causal bacteria is sometimes quite difficult requiring long culture periods. New techniques such as gas phase chromatography have been very useful in particularly low-count sub-cultures. Surgery remains the predominant therapy in endemic areas but antibiotic combinations are also effective. HIV-related immunosuppression did not change the clinical aspect of cutaneous mycobacteriosis in this patient whose immunological status was not modified.

Adult↗

A new case of isolated trichothiodystrophy.

We describe a new case of isolated trichothiodystrophy. This entity is characterized by sparse and brittle hair, low sulfur hair content, tiger tail pattern of the hair under polarizing microscopy, clean transverse fractures through the hair shaft and absent or defective hair cuticles. To our knowledge there are only two reported cases of isolated trichothiodystrophy without associated additional ectodermal or neuroectodermal dysplasias. Polarized microscopy of cut hair showed transverse fracture points (trichoschisis) and alternating dark and bright bands. Low sulfur content of the hairs was confirmed by amino acid analysis. Many acronyms and eponyms have been created to describe sulfur-deficient brittle hair associated with neuroectodermal abnormalities, leading to confusion. We suggest to use a simple classification scheme, according to increasing severity of associated features as proposed by Van Neste.

Child, Preschool↗

[Mycobacterium kansasii cutaneous infection].

The authors report a case of cutaneous infection caused by Mycobacterium kansasii in an immunocompetent woman. The mycobacterium was identified after a search for mycolic acids and the species-specific phenol-glycolipid K1. As minocycline followed by ciprofloxacin were ineffective, a conventional antituberculous treatment was prescribed and was fully successful.

Ciprofloxacin↗

[Oculocutaneous type II tyrosinosis].

Richner-Hanhart syndrome, also called oculo-cutaneous tyrosinosis type II, is a recessive autosomal genodermatosis consecutive to a disorder of tyrosine metabolism. It presents as a varying association of palmo-plantar keratosis, bilateral keratitis and mental retardation. The authors report a new case which is atypical in that palmoplantar keratosis made a late appearance. The diagnosis was confirmed by the presence of hypertyrosinaemia, hypertyrosinuria and urinary excretion of phenolic acids, and the absence of hepato-renal lesion. Needle biopsy of the liver, which demonstrates the deficiency of soluble cytosolic tyrosine aminotransferase, is not indispensable to the diagnosis and was not performed in our patient. Treatment consisted of a dietary measure: a controlled phenylalanine and tyrosine intake to obtain a tyrosinaemia below 10 mg/100 ml. This resulted in a favourable and durable course of the oculo-cutaneous lesions. In case of isolated skin lesion, retinoids can be prescribed either alone of combined with a diet, making it less strict.

Adult↗

Subcorneal pustular dermatosis in a patient with Crohn's disease.

A case of subcorneal pustular dermatosis (Sneddon-Wilkinson disease) is reported in a patient with a one-year history of Crohn's disease. Subcorneal pustular dermatosis has been described in association with monoclonal gammopathy, but to our knowledge it has not been associated with Crohn's disease. This new association reinforces the hypothesis of a possible common pathogenesis for neutrophilic dermatoses and inflammatory bowel diseases.

Adult↗

[Pemphigus vulgaris induced by radiotherapy].

A 54-year-old male patient who had been suffering from lymphoma for four years developed pemphigus vulgaris three weeks after exposure to radiotherapy. Skin lesions were initially strictly confined to the irradiated area and later extended over other skin areas. The eruption rapidly improved with glucocorticosteroid therapy which could be stopped after six months. Thirteen cases of pemphigus following X-ray irradiation have been published, twelve of them being associated with an internal malignancy. Clinical data strongly indicate radiation therapy as a provoking factor for the development of pemphigus, but a possible role of the neoplasia in our patient cannot be ruled out. It is suggested that immunological disturbances associated with lymphoma lead to an autoimmune response after exposure to X-rays. Ionizing radiation may alter the antigenicity of the epidermal cell surface or unmask epidermal antigens.

Autoantibodies↗