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Biomedical subjects

G Laplanche

Publications and source records attributed to G Laplanche.

18 recordsLinked to original sources
PubMed1997

[Hypersensitive urticarial vasculitis after natisedine intake].

BACKGROUND: Various skin and mucosal reactions can be observed after administration of quinidine derivatives. CASE REPORT: A patient who was taking Natisédine (quinidine phenylethyl barbiturate) intermittently and at reintroduction developed a papulopurpuric eruption (without thrombopenia) producing extensive centrifugal annular infiltration and central healing which regressed approximately one week after drug withdrawal. This eruption was associated with moderate 24 h proteinuria. The clinical aspect was that of vasculitis purpura as confirmed histology. Direct immunofluorescence only demonstrated C3 deposits in the vessel walls of the superficial dermis. The quinidine moiety of this drug (currently removed from the formulation) appears to be the responsible agent (imputability score: 13 B3). DISCUSSION: Thrombopenic purpura by synthesis of anti-platelet antibodies induced by quinidine derivatives is well known. Inversely, cases of non-thrombopenic purpura imputable to these same derivatives is uncommon (7 reported cases). The pathophysiological mechanisms involved might be similar (antigenic similarity between the platelet surface and endothelium).

Aged↗
PubMed1994

Neutrophilic dermatosis: a case of overlapping syndrome with monoclonal antineutrophil cytoplasmic autoantibody activity.

A 66-year-old male patient presented, over a 10-year period, polymorphic cutaneous manifestations with extensive neutrophilic infiltration which supports the diagnosis of overlapping syndrome of neutrophilic dermatoses. This was associated with a benign monoclonal gammopathy of IgA lambda type that had antineutrophil cytoplasmic autoantibody (ANCA) activity. Neutrophilic dermatoses may be associated with or can trigger ANCA.

Aged↗
PubMed1994

[Multicenter histiocytosis].

INTRODUCTION: Multicentric histiocytosis is a rare systemic disease with active episodes and prolonged periods of remission. Immunosuppressor treatment (alkylating agents) can be effective. CASE REPORT: A new case of multicentric histiocytosis was observed in a 38-year-old man who was successfully treated with cyclophosphamide at the dose of 100 mg/d. Treatment was withdrawn after several weeks due to drug-induced hepatitis and replaced with chlorambucil at the dose of 0.1 mg/kg/day for 6 months. No relapse has occurred after a follow-up of 14 months. DISCUSSION: This case is particularly interesting because of the exceptional nature of spontaneous haemarthrosis which was the inaugural sign of joint manifestations with mediastinal lymph nodes and initially isolated pruritus occurring before the typical skin manifestations. Different management protocols have been discussed with emphasis on the presence of acute cyclophosphamide induced hepatitis. This immunosuppressor is not usually hepatotoxic. CONCLUSION: This case demonstrates the systemic nature of the disease and emphasizes the beneficial effect of alkylating agents.

Adult↗
PubMed1988

[Surgical treatment of malignant melanoma of the limbs combined with thermochemotherapy under extracorporeal circulation].

In spite of the better results observed during the last decades and particularly due to earlier diagnosis and earlier surgical excision, malignant melanoma remains a tumour with a disconcerting course. This relative therapeutic impotence explains the variety of treatments used, including chemotherapy, radiotherapy, immunotherapy, etc. Surgical excision after chemotherapy under hyperthermic regional perfusion with extracorporeal circulation is an original method which has been applied for more than 3 decades with conflicting results but which may prove useful in several circumstances. The principle of regional perfusion using cytostatic drugs injected into the regional arterial blood flow resulted from a study by Klopp et al. in 1950. This technique was developed in practice by Creech et al. in 1957, and it was improved about 10 years later by additional hyperthermia, an idea suggested by Cavaliere et al. and by Stehlin. In Strasbourg, we have been using this method since January, 1982. Indications for perfusion are melanoma of the limbs with Breslow thickness greater than or equal to 0.85 mm, but from 1984 onwards these indications have been limited to patients with high-risk tumours such as melanoma with a Breslow thickness greater than or equal to 1.5 mm. Such melanomas are situated on the upper limb below the brachial insertion of the deltoid muscle, and on the lower limb below the upper third portion of the thigh. The technique consists of regional chemotherapy of the limb using extracorporeal circulation with hyperthermic perfusion. Melphalan is the drug used, and its dosage is based on body-weight: 1.4 mg/kg for the lower limb, and 0.9 mg/kg for the lower limb. Melphalan is introduced in the perfusion when the subcutaneous temperature near the tumour reaches 38 degrees C. The temperature of the limb is maintained at 40-42 degrees C for 45 minutes. At completion of the perfusion a wash-out is performed, and the tumour is excised with a 3 cm margin. From January, 1982 to January, 1987, 68 patients with malignant melanoma were treated by this method. Perfusion could not be performed in 5 patients because the small caliber of the vessel did not allow sufficient perfusion flow rate. No lethal complication occurred, and morbidity was very low.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed1984

A complex poroma-like adnexal adenoma.

Hidroacanthoma simplex, eccrine poroma, and dermal duct tumor are benign adenomas that develop from excretory ducts of eccrine glands and all three are variants of eccrine acrospiroma. To date, counterparts in apocrine or sebaceous glands have not been reported, but in this study we describe an adnexal, poroma-like adenoma that showed apocrine and sebaceous differentiations. Apocrine structures have the same embryonic origin as does the pilosebaceous system; both are derived from the primary epithelial germ. We suggest that the lesion we describe is truly a sebaceous and apocrine poroma. It must be distinguished from an infundibular adenoma whose pattern reproduces that of follicular poroma.

Adenoma↗
PubMedDate not supplied

Panniculitis revealing qualitative alpha 1 antitrypsine deficiency (MS variant).

A 16-year-old girl presented painful, red, nodular lesions on the abdomen. A cutaneous biopsy showed inflammatory cell infiltrate and fibrosis in the dermis and in the septa with isolated adipocyte lobules. alpha1-antitrypsin level was found to be normal but M1S phenotype of alpha1-antitrypsin was determined by isoelectric focusing in polyacrylamide gel. alpha1-antitrypsin level was normal for her family but M2S phenotype was found in her father. Alpha 1-antitrypsin (alpha1 AT) deficiency is a common hereditary disorder of Caucasians. The locus is pleiomorphic and 75 alleles have been identified. Numerous pathological mutations can be classified by the mechanisms which cause the deficiency. The major clinical importance of this deficiency is emphysema and liver disease. Panniculitis is rarely reported and seems to occur principally for the ZZ or MZ phenotype and for low levels of alpha1 AT. MS phenotype has been more rarely reported and triggering agents such as trauma and infections must be present. However, normal levels of alpha1 AT in the serum have previously been reported as in our case, and we suggest the study of alpha1 AT phenotype even if the plasma level is normal.

Abdomen↗
PubMedDate not supplied

[Congenital homolateral epidermal hyperplasia and hypoplastic hemidysplasia (splitting of the Solomon's syndrome) (author's transl)].

The observation of a 16-year-old girl born with an ectromelia and an ipsilateral inflammatory verrucous epidermal nevus led us to a synthetic study of 17 similar cases already published since 1927: all these cases concern female patients and are characterized by a unilateral hypoplastic dysplasia, most often of limbs, and inflammatory epidermal hyperplastic lesions described as ichthyosiform, psoriasiform or verrucous, usually distributed on the same side on the skin overlying the dysplastic body areas. The skin lesions may be partly regressive after birth and their histological features are suggestive of inflammatory linear verrucous epidermal nevus (I. L. V. E. N.). These associations may be representative of a special form of Solomon's syndrome whose heterogeneity has be recently emphasized. We propose to subdivide it in 3 forms: the epidermal nevus syndrome (Solomon's syndrome)--the organoid nevus syndrome (Schimmelpenning's syndrome)--the I. L. V. E. N. syndrome, probably X-linked dominant inherited (lethal for hemizygous males), associated with ipsilateral hypoplastic body lesions and, however less frequently than in the epidermal nevus syndrome, with ocular and nervous abnormalities. The distribution of cutaneous lesions has some similarities with the pattern of skin symptoms of X-linked dominant traits such as chondrodysplasia punctata, focal dermal hypoplasia or incontinentia pigmenti. The most typical feature of this syndrome is the strong inflammatory aspect of the epidermal nevus erroneously described in previous cases as unilateral psoriasis or ichthyosiform erythroderma.

Adolescent↗