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Biomedical subjects

F Mantoux

Publications and source records attributed to F Mantoux.

16 recordsLinked to original sources

Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans.

BACKGROUND: Some cases of dermatofibrosarcoma protuberans (DFSP) do not protrude above the skin. OBJECTIVES: To assess the prevalence of these DFSPs and further to describe their presentation and course. METHODS: One hundred and forty-three patients were retrospectively collected. They were asked to complete a standardized questionnaire indicating the history and appearance of the DFSP from the first skin changes identified to the time of diagnosis. RESULTS: Eighty-one DFSPs were described as protuberant ab initio, and 62 as initially nonprotuberant (npDFSP). The latter remained at this stage for a mean period of 7.6 years. Twenty-nine per cent of npDFSPs were 'morphoea-like', 19% were 'atrophoderma-like' and 42% were 'angioma-like'. Age at diagnosis was similar for both initial presentations. npDFSPs were most often misdiagnosed by physicians. CONCLUSIONS: Nearly half the patients first identified their early DFSP-related skin changes as patches. Both this frequency and the long duration at this preprotuberant stage should prompt dermatologists to consider the diagnosis of DFSP earlier, in order to make surgical treatment easier.

Adolescent↗

[Mycobacterium fortuitum skin infection occurring after a facelift].

INTRODUCTION: Mycobacterium fortuitum skin infections are rare and usually iatrogenic. We report a case with cervical involvement following a facelift. OBSERVATION: A 65 year-old woman, without past history, underwent bilateral surgical facelift, complicated by cutaneous necrosis and treated with directed healing at home. Six weeks later, an abscessed nodule appeared under the left maxillary and was drained surgically. Then other pre-auricular and left cervical inflammatory nodules appeared without adenopathy or fever. M. fortuitum was isolated in bacteriological samples. The initially probabilistic antibiotherapy with carithromycin, subsequently adapted with amikacine and cirprofloxacine and then imipeneme for a total duration of 3 months, led to the clinical cure. DISCUSSION: Mycobacterium fortuitum is a rapidly growing, ubiquitous, mycobacteria responsible for nosocomial infections in immunocompetent patients, notably following plastic surgery. Contamination occurs where there has been a rupture in the skin barrier through contact with a vector (water, surgical material, antiseptic.). Treatment, which is not codified, consists in the association of surgery and antibiotics for several months.

Aged↗

[Hallucinations during treatment with hydrochloroquine].

INTRODUCTION: We report an unexpected cenesthetic hallucination-type neuropsychiatric side effect with hydrochloroquine (Plaquenil) in a patient treated for an erosive plantar lichen planus. OBSERVATION: A 75 year-old woman was hospitalized for a handicapping erosive plantar lichen. Treatment with hydrochloroquine (400 mg/day) was initiated, associated with topical corticosteroids and a short course of oral acorticosteroids (0.5 mg/kg/day of methylprednisolone). After 10 days of treatment, a short episode of temporo-spatial disorientation occurred, followed by a feeling of depersonalization and cenesthetic hallucinations with feelings in the body. These manifestations were preceded by nightmares. Hydrochloroquine was spontaneously stopped by the patient one week later and led to the progressive disappearance of the hallucinations and a return to a normal mental state within one month. Two and a half years later, no relapse of the psychiatric manifestations has been noted. DISCUSSION: Chloroquine and hydrochloroquine may be at the origin of severe psychosis-like psychiatric side effects. Such manifestations are exceptional, little known and principally described during treatment of malaria. The clinical presentation of the psychosis induced by synthetic ani-malarials is fairly homogeneous from one case to the next: onset in a patient without psychiatric past of manifestations such as delirium, hallucinations, maniac episodes or depression after an interval of a few hours to 40 days, usually regressing one week after suspension of the synthetic antimalarial. There is no relationship between the dose of synthetic anti-malarial administered and the onset of psychiatric problems. The triggering-off mechanism is unknown and appears to be an idiosyncratic reaction. Our case report draws the dermatologists' attention to the possibility of the occurrence of potentially severe psychiatric side effects.

Aged↗

[Cutaneous Waldenström's macroglobulinemia].

BACKGROUND: We report the case of a patient in whom the first manifestation of Waldenström' s macroglobulinemia was specific skin lesions, treated with chlorambucil chemotherapy. CASE REPORT: A 76-years old woman was referred to us because of chronic red nodular lesions on her face. A biopsy specimen showed a dense lymphocytic dermal infiltrate and immunohistochemistry identified a monoclonal B lymphoid population with an IgM-kappa phenotype. The patient's disease was diagnosed as Waldenström's macroglobulinemia with cutaneous localization, on the basis of a high level of circulating macroglobulinemia and a lymphoplasmocytic infiltrate in the bone marrow expressing the same monoclonal IgM-kappa as in blood and skin. Treatment with radiotherapy (12 Grays) was unsuccessful. Chlorambucil (16 mg per day, 7 days per month) was then introduced with rapid disappearance of the skin lesions. Neutropenia led to withdrawal of this treatment after 4 courses. The skin lesions relapsed 18 months later and were cured with chlorambucil at a lower dose. DISCUSSION: Specific skin infiltrates have been rarely described during Waldenström's macroglobulinemia. Review of the literature showed eight cases of such lesions treated by chemotherapy with only two successes with oral cyclophosphamide and polychemotherapy (cyclophosphamide, vincristine and CCNU). Chlorambucil was used unsuccessfully three times. We hypothesize that primary resistance to alkylating-agent and the small number of cases of cutaneous Waldenström's macroglobulinemia may explain the poor response to systemic chemotherapy previously reported.

Aged↗

Rab27a: A key to melanosome transport in human melanocytes.

Normal pigmentation depends on the uniform distribution of melanin-containing vesicles, the melanosomes, in the epidermis. Griscelli syndrome (GS) is a rare autosomal recessive disease, characterized by an immune deficiency and a partial albinism that has been ascribed to an abnormal melanosome distribution. GS maps to 15q21 and was first associated with mutations in the myosin-V gene. However, it was demonstrated recently that GS can also be caused by a mutation in the Rab27a gene. These observations prompted us to investigate the role of Rab27a in melanosome transport. Using immunofluorescence and immunoelectron microscopy studies, we show that in normal melanocytes Rab27a colocalizes with melanosomes. In melanocytes isolated from a patient with GS, we show an abnormal melanosome distribution and a lack of Rab27a expression. Finally, reexpression of Rab27a in GS melanocytes restored melanosome transport to dendrite tips, leading to a phenotypic reversion of the diseased cells. These results identify Rab27a as a key component of vesicle transport machinery in melanocytes.

Biological Transport↗

Ras mediates the cAMP-dependent activation of extracellular signal-regulated kinases (ERKs) in melanocytes.

In melanocytes and melanoma cells, cAMP activates extracellular signal-regulated kinases (ERKs) and MEK-1 by an unknown mechanism. We demonstrate that B-Raf is activated by cAMP in melanocytes. A dominant-negative mutant of B-Raf, but not of Raf-1, blocked the cAMP-induced activation of ERK, indicating that B-Raf is the MEK-1 upstream regulator mediating this cAMP effect. Studies using Clostridium sordelii lethal toxin and Clostridium difficile toxin B have suggested that Rap-1 or Ras might transduce cAMP action. We show that Ras, but not Rap-1, is activated cell-specifically and mediates the cAMP-dependent activation of ERKs, while Rap-1 is not involved in this process in melanocytes. Our results suggest a novel, cell-specific mechanism involving Ras small GTPase and B-Raf kinase as mediators of ERK activation by cAMP. Also, in melanocytes, Ras or ERK activation by cAMP is not mediated through protein kinase A activation. Neither the Ras exchange factor, Son of sevenless (SOS), nor the cAMP-responsive Rap-1 exchange factor, Epac, participate in the cAMP-dependent activation of Ras. These findings suggest the existence of a melanocyte-specific Ras exchange factor directly regulated by cAMP.

Animals↗

[Flutamide-induced late cutaneous pseudoporphyria].

BACKGROUND: Pseudoporphyria is a condition characterized by clinical and histological changes resembling porphyria cutanea tarda, in the absence of any demonstrable abnormality in porphyrin metabolism. Etiologies of pseudoporphyria are hemodialysis for chronic renal failure, chronic UV irradiation, and several drugs, particularly naproxen. We report a case of flutamide induced pseudoporphyria. CASE REPORT: A 75-year-old man had prostatic carcinoma and was treated with flutamide for 1-1/2 years. He presented with a several months history of blisters on the back of the hands and fingers, occurring after sun exposures. The bullae were associated with skin fragility and atrophic scarring. Histopathological examination and direct immunofluorescence showed ultrastructural features close to those described in porphyria cutanea tarda. Quantitative analysis of porphyrins in urine and laboratory blood tests were normal. Flutamide was stopped, leading to healing of the lesions, with no relapse after 11 months. DISCUSSION: Flutamide is an antiandrogen used for the treatment of prostatic carcinoma. Its principal side-effect is represented by liver toxicity. Cutaneous side-effects of flutamide are uncommon; three cases of photo-allergic dermatitis have been described, and we report with our observation, the third case of cutaneous pseudoporphyria induced by flutamide.

Aged↗

[Myiasis caused by Dermatoba hominis after an organized tours in Amazonia].

BACKGROUND: Human cutaneous myiasis is a common disease in endemic tropical zones. The incidence of imported cases follows the development of organized tours in the tropics. CASE REPORTS: We report 2 cases of Dermatobia hominis furonculoid myiasis in patients who took 2 different organized tours which both visited the same Amazonian site in Peru (Puerto Maldonado). DISCUSSION: In Latin American countries, D. hominis myiasis is transmitted by Diptera (generally mosquitos, rarely flies) who carry eggs laid by an adult fly to man. This phenomena is called "phoresia" and explains the preferential localization of lesions in exposed areas unlike African myiasis caused by Cordylobia anthropophaga which contaminates clothing soiled when drying.

Adult↗