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Biomedical subjects

B Guillot

Publications and source records attributed to B Guillot.

At least 163 records · Page 9Linked to original sources
PubMed1987

[Multiple trichoepitheliomas, cylindromas and milia. An entity].

Four patients from two different families presented with multiple papular trichoepitheliomas of the face associated with cylindromas of the scalp and, in one of them, milium. This association, first described by Adamson, has now become classical. It is transmitted as an autosomal dominant trait with variable penetrance. Histochemical studies gave the following results: ATPase negative in the two types of tumour, phosphorylase weakly positive, NADH diaphorase positive in the basal cells of the trichoepitheliomas and diffusely in cylindromas. These results suggest that the cylindromas are of apocrine origin. Using monoclonal antibodies, it has been possible to demonstrate the presence of Langerhans cells in both trichoepitheliomas and cylindromas. The BL9 and KL3 antikeratinocyte monoclonal antibodies were negative, whereas the KL3 antibody, which recognizes the 55-57 Kd polypeptides of keratin, marked the suprabasal part of the tumours. These results are in favour of incomplete cell differentiation. Treatment with retinoids was ineffective, as in all other cases reported. Electrocoagulation or surgical excision om request for cosmetic reasons seem to be only possible treatments.

Antibodies, Monoclonal↗
PubMed1987

[Trichothiodystrophy].

Trichothiodystrophy is a congenital dysplasia of hairs characterized by: a pathognomonic image of the hair shaft under polarized light (the hair is plaited with alternately dark and light oblique bands) and trichoschisis with a clear-cut break; a deficiency of sulphurated aminoacids in the hair. We report a case where trichothiodystrophy was associated with abnormalities of the nails, teeth and eyes, growth and mental retardation, neurological syndrome and hypogonadism. From this case and a review of the cases published so far the following points emerge: 1. Congenital abnormalities of the neuroectodermal system are frequent. They include: skin lesions, such as ichthyosis, photosensitivity and atopic eczema; ungueal dysplasia; dysmorphic syndrome; growth and mental retardation; hypogonadism; neurological and ophthalmic abnormalities. 2. The condition seems to be transmitted as an autosomal recessive trait. A common element, dysplasia, enables us to include in the same nosological group as trichothiodystrophy a number of other pathological conditions, viz.: syndromes hitherto described under various names but almost identical, such as Brown's syndrome and BIDS syndrome (brittle hair, intellectual deficit, decreased fertility, small stature); IBIDS and PIBIDS syndromes which, in addition to the former, comprise ichthyosis and photosensitivity, as well as Tay's syndrome (characterized by the presence in all cases of ichthyosis associated with hair dysplasia); and perhaps some anecdotic cases with either a pathognomonic image under polarized light or a suggestive biochemical profile. Finally, since typical trichothiodystrophy has been reported in patients with Siemens' syndrome or with Marviesco-Sjögren syndrome, these two syndromes may perhaps also be classified in the same category, as borderline forms of the disease.

Abnormalities, Multiple↗
PubMed1987

Dapsone as initial treatment in superficial pemphigus. Report of nine cases.

Nine cases of superficial pemphigus that were treated by dapsone as the initial mode of therapy are reported. Five patients, all having low or negative serum anti-intercellular cement substance antibody titers, responded dramatically, while the other four patients, showing high titers of anti-intercellular cement substance antibodies, did not respond to this treatment. Complications of therapy were observed in three cases.

Adult↗
PubMed1987

[Pyoderma gangrenosum. 14 personal observations and review of the literature].

Fourteen cases of pyoderma gangrenosum are reported. Twelve were associated with various diseases: ulcerative colitis, Crohn disease, colic tumor, rheumatoid arthritis, chronic myeloid leukemia, agammaglobulinemia, respiratory infections (2 cases) and post surgical intervention. In 2 cases pyoderma gangrenosum appeared to be primary. The clinical characteristics of the affection make it a very particular type of ulcer: pustular onset, frequent localization to other than leg, rapid eccentric spread, frequent associated morbidity (mainly digestive and hematologic). The pathogenesis is still unknown and the treatment is not well codified.

Adolescent↗
PubMed1986

[Systemic scleroderma. Contribution of esophageal manometry].

Oesophageal motor function was studied by oesophageal manometry in 48 patients with progressive systemic sclerosis: 25 with proximal scleroderma and 23 with diffuse scleroderma. Oesophageal lesions were noted in 70% (74% in diffuse scleroderma; 64% in proximal scleroderma). Classical manometric signs of scleroderma were found in only 31% of patients. Peristaltic modifications might begin at the junction of the two muscular coats, since a four centimeter long aperistaltic suspended area was noted in that region in 20% of patients, especially in the proximal scleroderma group. Oesophageal motility and low lower oesophageal sphincter pressure account for the gastro-oesophageal reflux and may compromise respiratory function, as suggested by the high frequency of concurrent oesophageal and respiratory dysfunction in diffuse scleroderma. Systematic prevention of gastro-oesophageal reflux should perhaps be advocated as soon as abnormalities in oesophageal motility are diagnosed.

Adolescent↗
PubMed1986

Long term plasma exchange therapy in bullous pemphigoid.

Long term plasma exchanges associated with corticosteroids (11 patients) were compared with corticotherapy alone (10 patients) for the treatment of bullous pemphigoid. The patients having long term plasma exchanges showed a lower rate of relapses at six months and needed less corticosteroids.

Aged↗
PubMed1986

[Treatment of chronic urticaria with ketotifen].

Ketotifen, an inhibitor of the mastocytes degranulation is used in the treatment of allergic asthma. The use of this drug in chronic urticaria is proposed because histamine play a major role in the production of the lesions. In this study, 36 patients with chronic urticaria of various aetiology are treated with ketotifen (2 mg/d). 12 patients were healed, 11 good results and 12 failure were obtained. The best results are obtained in cold urticaria and in food related urticaria. This result were compared with data concerning the treatment of chronic urticaria.

Adolescent↗
PubMed1986

[Chronic herpes of the pyodermatitis vegetans type in chronic cutaneous lymphoid leukemia].

The authors report a case of chronic herpes virus infection of the face which developed in a 70-year old man already affected with chronic lymphocytic leukaemia of the B-cell type (CLL-B) with specific cutaneous localisations. Immunodepression was indicated only by marked hypogammaglobulinaemia. Cell-mediated immunity was preserved. The cutaneous lesions of the face were chronic and presented as pyodermatitis vegetans. A one-week course of acyclovir administered by intravenous infusion in doses of 5 mg/kg three times a day resulted in rapid and dramatic cure, but this result proved transient, since the virus infection relapsed 2 1/2 months later. The new episode also was successfully treated with a second course of acyclovir. The herpes virus infection had developed only on those skin areas that were specifically affected by the leukaemia; after treatment and eradication of the virus, massive lymphocytic infiltration of the dermis persisted in these areas. Involvement of the skin is rare in CLL-B and has been reported mainly in CLL-T. The lesions most frequently encountered are tuberous and papular lesions and infiltrated plaques on the forehead and ears. The pyodermatitis vegetans presentation is unusual. The reasons why viral skin lesions develop on those caused by leukaemia are unknown.

Acyclovir↗
PubMed1986

[Association of a pediatric bullous eruption, cutaneous and muscular atrophy, hyperpigmentation and dysmorphism. A new entity?].

We report the case of a young patient who presented with dysmorphism, bullous eruption of childhood, cutaneous and muscular atrophy and hyperpigmentation. Attempts were made to find out where this case fits in the nosological framework. This young boy without any particular family history was born with facial dysmorphism consisting of micrognathia, right microphtalmos, gothic palate and left facial palsy of the peripheral type. At the age of 2 years and 9 months, generalized fragility of the skin appeared in the form of a recurrent bullous eruption. The bullae left numerous atrophic and depigmented scars; they spared the mucosae, and there was no photosensitivity. At the same time, generalized skin atrophy developed: the subcutaneous venous network was abnormally visible and there was diffuse hyperpigmentation. The hair was fine, curly and thin. The teeth dystrophic and abnormally positioned. The cornea of the right eye was invaded by conjunctiva. The bullous eruption subsided when the child was about 6 years' old, but the skin atrophy became worse and was accompanied with amyotrophy of the limbs and retraction in flexion of the joints at the extremities. Chronic ulcerations were present on the lower limbs. Statural and ponderal growth, as well as mental development were normal. The biochemical examinations performed revealed no abnormality. Light and electron microscopy of the skin showed a normal dermis-epidermis junction, but the anchorage fibres were rarefied. The dermal connective tissue was abnormal, with thin collagen fibres and disorganized fibrillae. Fibroblasts were hypoplastic and numerous. The elastic network was meagre and elastic fibres had a slashed appearance. Biopsy of a palmar nodule showed cheloid-like lesions. The nosological discussion involved some congenital bullous diseases and certain forms of connective tissue dystrophia. Among the bullous diseases, congenital poikiloderma, as described by Weary and Kindler, is unaccompanied with amyotrophy, articular retraction or dysmorphism. According to Verret et al., the bulla is located at the dermis-epidermis junction. Recessive dystrophic epidermolysis bullosa seems to differ from our case in that skin atrophy is localized and amyotrophy, dysmorphism and disorders of pigmentation are absent. In addition, we found no histological evidence of collagenolysis. Mendes Da Costa's bullous dystrophy can be excluded, as there was no nanism, microcephaly or diffuse alopecia in our patient. Among connective tissue dystrophias, acrogeria Ehlers-Danlos type IV syndrome does not include bullous eruption, amyotrophy or articular retraction.(ABSTRACT TRUNCATED AT 400 WORDS)

Abnormalities, Multiple↗
PubMed1986

[Porphyria cutanea tarda and neoplasms. Apropos of 2 cases].

Porphyria cutanea tarda (PCT) may be associated with various neoplasma. Two additional cases are reported here. In the first case, a 58-year old alcoholic man had been presenting for two years with clinical signs of PCT. The diagnosis was confirmed by porphyrin assays in the urine. He also had cirrhosis of the liver. During a routine fibroscopy in search of oesophageal varices, a gastric adenocarcinoma was discovered by chance. Following partial gastrectomy the skin lesions of PCT improved dramatically within a few weeks, leaving only moderate cutaneous fragility. Urinary porphyrin assays performed 18 and 40 months after gastrectomy gave normal results, although no specific treatment had been prescribed. The second case concerns a 67-year old man, also alcoholic, with clinical and biochemical PCT. For the previous 12 months he had received chemotherapy (Adriamycin, then BCNU combined with melphalan, vincristine and prednisone) for multiple IgA K myeloma. The myeloma was active when PCT was diagnosed with, in particular, chronic anaemia. Treatment with chloroquine improved the cutaneous signs of PCT but had no effect on urinary porphyrins after 5 months. Comments PCT has been reported to be associated with cancers. The best known of these cancers is primary carcinoma of the liver (4, 27, 32), but its frequency is diversely evaluated depending on the diagnostic methods (e. g. patient autopsied or not) and on the selection of patients (age, duration of the disease).(ABSTRACT TRUNCATED AT 250 WORDS)

Adenocarcinoma↗
PubMed1985

[Urticaria].

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Adolescent↗