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Biomedical subjects

P Foulc

Publications and source records attributed to P Foulc.

9 recordsLinked to original sources
PubMed2003

Prognostic factors in Sézary syndrome: a study of 28 patients.

BACKGROUND: The new European Organization for Research and Treatment of Cancer classification considers Sézary syndrome (SS) among the aggressive epidermotropic cutaneous T-cell lymphomas (ECTLs). Recent technological advances have facilitated the diagnosis of this disease, but it remains practically incurable, with a median survival of about 2.5-5 years. Deaths are due in part to the iatrogenic effects of treatments, which suggests that the management of SS could be improved. OBJECTIVES: Retrospectively to study the prognostic criteria related to disease progression. METHODS: Thirty patients with SS were followed up in the Dermatology Department of the University Hospital in Nantes, France, between January 1989 and May 2000. The diagnosis of SS was based on at least three of the following criteria: erythroderma, histological evidence of ECTL, a level of 20% or more circulating Sézary cells, and loss of My7 antigen expression by basal cells of the epidermis. Two patients not seen again after the initial diagnosis were excluded from the statistical study. RESULTS: The median disease-specific survival of the 28 patients was 64.55 +/- 10.11 months. The prognostic factors found in univariate analysis were age at diagnosis (P = 0.0109), interval before diagnosis (P = 0.0566), lactate dehydrogenase (LDH) level (P = 0.042) and presence of the Epstein-Barr virus (EBV) genome (BHLF in in situ hybridization) in skin (P = 0.0079). The prognostic factors found in multivariate analysis were age, interval before diagnosis and presence of the EBV genome in keratinocytes. A decreased number of Langerhans cells in the epidermis did not appear to be a prognostic factor. CONCLUSIONS: Our study confirms the prognostic value of age and LDH level, and for the first time demonstrates the prognostic value of the identification of the EBV genome in the skin. This seems consistent with a marked immune deficit during severe forms of SS.

Adult↗
PubMed2003

[Severe cutaneous drug reactions to celecoxib (Celebrex)].

INTRODUCTION: Celecoxib (Celebrex) is a Cox 2 selective non steroidal anti-inflammatory agent. Adverse reactions reported are usually benign, such as maculopapular exanthema. However, recently severe toxiderma have started to appear. We report two patients who developed a sever celecoxib drug skin reaction. OBSERVATIONS: Case no 1. In a 73 year-old woman, treated with celecoxib for cervical arthralgia, a maculopapular exanthema five days after treatment was started. The exanthema, initially edematous and purpural became bullous with multi-visceral failure (disseminated intravascular coagulation, renal failure, hepatitis and pancreatic). The disease slowly regressed. Case no 2. A 72 year-old man, treated for cervicalgia with celecoxib, presented a pustular exanthema of the face and the trunk, ten days after introduction of the treatment, associated with an inflammatory syndrome and hepatic cytolysis. Within 8 days the disease had regressed. In both these patients, the celecoxib patch tests were positive. DISCUSSION: Until now, the side effects reported with celecoxib have been generally benign exanthemas. We report two cases of severe celecoxib-induced toxiderma: a hypersensitivity syndrome with multi-organ failure and an acute generalized atypical exanthematous pustulosis. These severe toxidermas have rarely been reported in the literature, but we now need to reassess the risks with these new molecules.

Aged↗
PubMed2002

Evaluation of a 1-h exposure time to mechlorethamine in patients undergoing topical treatment.

BACKGROUND: Mechlorethamine is frequently used in the treatment of cutaneous lymphoma, but its application is limited in 30-80% of cases because of cutaneous intolerance. Reducing the concentration to avoid this side-effect has been only modestly successful. OBJECTIVES: To investigate whether a shorter application period could reduce the frequency of intolerance. METHODS: In an open prospective study in 39 patients with cutaneous T-cell lymphoma or parapsoriasis, mechlorethamine was applied according to the usual practices of the participating physicians (number of weekly applications, treatment confined to lesions or performed over the entire body) and then washed off after 1 h in all cases. RESULTS: Cutaneous intolerance was observed in 19 of 39 patients (49%). Six of these patients showed allergic contact dermatitis to mechlorethamine after a mean period of 9.3 weeks, while the other 13 developed irritant contact dermatitis after a longer period. Cutaneous intolerance did not differ significantly according to the number of applications per week or the extent of body area treated. The therapeutic response rate was 69%, and no difference in therapeutic efficacy was noted between daily and intermittent applications. CONCLUSIONS: Comparison with published studies showed no significant difference in the number of cases of cutaneous intolerance after short-term application, although their occurrence was delayed. Therapeutic response was decreased appreciably by short-term application as compared with results in the literature.

Administration, Topical↗
PubMed1999

[Disclosure of Behcet disease by concomitant dental infection].

BACKGROUND: Behçet's disease can be aggravated by chronic infection. Our case illustrates how the disease can be disclosed during an acute activation of a chronic infectious focus. CASE REPORT: A 40-year-old man had Behçet's syndrome associating fever, bipolar aphtosis, cutaneous pseudo folliculitis, anterior uveitis, and meningitis following an acute episode of a dental infection, with periapical granuloma. Antibiotic therapy was ineffective in calming this first flare-up of Behçet's disease. Corticosteroid and colchicine therapy were effective. DISCUSSION: Clinical and experimental work would implicat buccal streptococcal flora in the pathogenesis of Behçet's disease. Antibiotics offer interesting perspectives but further studies are needed to define their role in the treatment of Behçet's disease.

Adult↗
PubMed1999

[Trichothiodystrophy: progresssive manifestations].

INTRODUCTION: Trichothiodystrophy is an autosomal recessive genodermatosis associating congenital dysplasia of the hair and neuroectodermal defects. Clinical expression is variable, although abnormalities are generally noted from birth. We report trichothiodystrophy in two brothers with the same phenotype who presented unusual progressive manifestations. OBSERVATIONS: Case 1: A six-year-old boy was seen for vesicular blisters due to photosensitization. Clinical examination showed dry, brittle, unmanageable hair, discrete koilonychia-type nail defects and an ichthyosiform state. The teeth were normal. In addition to psychomotor retardation, the patient presented a dysmorphic syndrome (poorly rimmed low-set ears; thick, triangular upper lip; scaphocephalic skull; short hands) and congenital bilateral cataract. The diagnosis of trichothiodystrophy was confirmed by a study of DNA repair after exposure to ultraviolet light. A repair defect was found similar to that in xeroderma pigmentosum group D. The patient experienced a worsening of psychomotor retardation and episodes of hair loss with edema and inflammation of the scalp resulting from infections. He also showed marked asthenia which resolved spontaneously within a few months. Case 2: The other brother, born as a collodion baby, presented the same clinical picture (cutaneous, exoskeletal, dysmorphic), including congenital bilateral cataract, photosensitivity and a parenchymatous blister-type pulmonary lesion probably secondary to bronchiectasis. The patient's cutaneous state progressively improved. He was seen at six years of age for an episode of inflammatory edema of the scalp with hair loss. Within six months, all of the hair redrew. The diagnosis of trichothiodystrophy was confirmed by a DNA repair defect after exposure to ultraviolet light. DISCUSSION: Trichothiodystrophy is clinically associated with photosensitivity (P), ichthyosis (I), dry, brittle hair (B), intellectual impairment (I), decreased fertility (D) and short stature (S), which accounts for the acronym PIBIDS or IBIDS syndrome, depending on whether photosensitivity is involved or not (actually in about 50 p. 100 of cases). Other possibly associated features include ungueal dysplasias, bilateral cataract, defective teeth, dysmorphic disorders predominant in the ears, neurologic disorders, pulmonary bronchiectasis and recurrent infections. The two cases presented here were thus very symptomatologically complete. The two problems of current concern are psychomotor retardation and temporary hair loss as a result of infections. The latter has only been described once in the literature. This case was similar to ours since photosensitivity was involved. Analysis of DNA repair also showed a defect after exposure to ultraviolet light similar to that found in xeroderma pigmentosum group D. Thus, episodic hair loss could be a symptom characteristic of forms of trichothiodystrophy with a DNA repair defect. However, the explanation for this hair loss is not known. Other ectodermal dysplasias can be complicated by hair loss with superinfection, such as AEC syndrome (ankyloblepharon, ectodermal dysplasia, cleft palate).

Abnormalities, Multiple↗