[Acquired hypertrichosis lanuginosa and gastric adenocarcinoma]. / Hypertrichose lanugineuse acquise et adénocarcinome gastrique.

INTRODUCTION: Acquired hypertrichosis lanuginosa is a form of obligatorily paraneoplastic disease characterised by the recurrence of lanuga hair during adulthood. We report a case in which this hypertrichosis allowed diagnosis of gastric cancer. OBSERVATION: A 51 year-old woman was seen for hypertrichosis present for 3 months. Clinical examination led to diagnosis of acquired hypertrichosis lanuginosa, which subsequently resulted in the discovery of gastric adenocarcinoma. Surgical excision of the tumour resulted in the disappearance of hypertrichosis with no recurrence during the ensuing 13 months. DISCUSSION: Acquired hypertrichosis lanuginosa is rare, with only 50 or so cases reported in the literature since the condition was first described in 1865 by Turner. These cases confirm the obligatorily paraneoplastic nature of this particular dermatosis. Our finding is original since it is the first recorded case of association with gastric adenocarcinoma. It is also unique in terms of the strictly parallel development of acquired hypertrichosis lanuginosa and the tumour, with complete disappearance of the hypertrichosis in the weeks following surgical removal of the tumour, and in terms of prolonged survival (complete remission 17 months after the onset of symptoms). The mechanism responsible for acquired hypertrichosis lanuginosa is unknown. Two hypotheses have nevertheless been suggested: acquired hypertrichosis lanuginosa could be associated with secretion by the tumour of an as yet unidentified serum factor, or with a nutritional deficiency that may accompany this form of cancer.

[Nicorandil-associated anal ulceration]. / Ulcérations anales induites par le nicorandil.

BACKGROUND: Nicorandil is a potassium-channel activator used in the treatment of angina pectoris. The first cases of anal ulcerations induced by nicorandil were published in 2002. CASE REPORT: A 71-year-old man presented with a 2-year history of anal ulcerations occurring within a few months of initiation of treatment with Nicorandil. Histological tests on a biopsy sample showed granulation tissue with non-specific chronic inflammation. Nicorandil was stopped and this resulted in complete healing of the ulcers after three months. DISCUSSION: Nicorandil can induce chronic and extensive anal ulcerations. The pathogenesis is unknown. Patients are usually treated with high doses of nicorandil. Dermatologists should be aware of this rare side-effect which heals after withdrawal of the drug.

Use of allogenic epidermal sheets for difficult wound healing: selection and testing of relevant growth factors.

The clinical interest of using allogenic epidermal sheets (AES) has largely been shown [1,2,3]. As well as covering, they also stimulate healing, by simultaneously secreting numerous growth factors (GFs), although little is known on their mechanism of action. Our objectives were to: (a) devise a test for the efficacy of AES release, (b) select keratinocyte-secreting strains and optimal culture conditions. Three GFs were selected: IL-1alpha, IL-8 and VEGF. Three different keratinocyte strains were cultured for 3 and 6 days after confluence for 3 passages. Assays were performed after 3 h and 24 h+3 h after dispase treatment (AES conservation for 24 h then change of medium and sampling after 3 h). AES were found to secrete GFs in DMEM and the amounts were greater when cultured for 6 rather than 3 days after confluence. Each strain had different secretory patterns depending on passage and time in culture, this variability being explained by inter-individual heterogeneity.

[Drug hypersensitivity syndrome associated with a primary HHV6 infection]. / Syndrome d'hypersensibilité médicamenteuse associé à une primo-infection HHV6.

INTRODUCTION: Drug hypersensitivity syndrome is a severe life-threatening drug reaction. An association between this syndrome and HHV6 reactivation has been hypothesized. CASE-REPORT: A 45 year-old women was treated with sulfasalazine for polyarthralgia. One month after beginning the treatment, she developed a drug hypersensitivity syndrome with severe acute hepatic failure. HHV6 serology and serum PCR revealed a primary HHV6 infection. DISCUSSION: We report the second case of drug hypersensitivity syndrome associated with a primary HHV6 infection. An immunological disorder may explain such an association by increasing viral replication. Detection and titration of anti-HHV6 antibodies in each case of drug hypersensitivity syndrome should help to confirm this association and possibly modify treatment strategy.

[Kaposi's sarcoma and organ transplantation: 22 cases]. / Maladie de Kaposi et transplantation d'organes: 22 cas.

BACKGROUND: The discovery of the Human Herpes virus 8 (HHV8) improved our knowledge of the pathogenesis of Kaposi's sarcoma. After organ transplantation, Kaposi's sarcoma exhibits distinctive features compared with other forms of the disease. PATIENTS AND METHODS: We report 22 cases of post-transplant Kaposi's sarcoma (12 kidneys, 2 kidney-pancreas, 6 livers and 2 hearts). The aim of this retrospective study was to analyze clinical and virological characteristics in these transplant patients and to specify the frequency of HHV8 seroconversions in this population. RESULTS: Twenty-one patients showed cutaneous lesions and 9 had visceral involvement. HHV8 serology was positive in 16/20 patients at transplantation and in 21/22 cases at the time of Kaposi's sarcoma diagnosis. Most cases corresponded to viral reactivations whereas seroconversions occurred in 2 cases and may have been linked to viral transmission by the graft. Treatment led to recovery in 68p. 100 of the cases. Two heart-transplant patients died from their disease. We included in our series two cases of re-transplanted patients without recurrence of Kaposi's sarcoma and one case of familial Kaposi's sarcoma. DISCUSSION: Seroconversions after transplantation emphasize the interest of systematic screening of HHV8 serology in transplant recipients and their donors.

[Correction of facial lipoatrophy with a biodegradable material in HIV-infected patients]. / Correction par produit biodégradable des lipoatrophies faciales des malades infectés par le VIH.

INTRODUCTION: Facial lipoatrophy in HIV-infected patients under tri-therapy occurs frequently and alters their quality of life. No systemic treatment is capable of curing this problem. PATIENTS AND METHODS: Eighty-one patients were included in an open, prospective, compassionate study. A strict intradermal injection of a biodegradable, polyacrylamide cationic copolymer was administered in several sessions. RESULTS: In all the patients, the correction of the facial lipoatrophy was considered satisfactory and stable over a period of 6 months using comparative photographic clichés. The thickness of the dermal skin, assessed by sonography, was doubled. No local or systemic side effect was reported. CONCLUSION: Our study shows that is it possible to durably correct the facial lipoatrophy of HIV-infected patients under tri-therapy by using a biodegradable implant.

TGFbeta inhibits CD1d expression on dendritic cells.

The CD1 family of cell surface glycoprotein has been demonstrated to be a third lineage of antigen-presenting molecules for specific T cell responses. They present lipidic, glycolipidic antigen and hydrophobic peptide to T cells. CD1d restricted T cells play a role in autoimmune disease and in tumor immunity. Transforming growth factor beta (TGFbeta), a member of the family of polypeptide growth factors synthetized by human keratinocytes, has inhibitory effects on proliferation and differentiation of immune cells, especially on CD1d-restricted natural killer T cells. These properties led us to investigate the role of TGFbeta in CD1d expression on dendritic cells (DC), which are known to play a key role in initiation of the immune response. Here, we observed CD1d molecules on DC developed from PBMC with GM-CSF and IL4 but not with GM-CSF, IL4 and TGFbeta for 7 d. RT-PCR and FACS analysis (mAb 42.1) performed at various stages of differentiation on CD34+ HPC show that CD1d mRNA levels and CD1d molecule expression at the cell surface decreased progressively during the differentiation process. Thus, while committing DC-precursors differentiation toward the Langerhans cell (LC) pathway, TGFbeta likely inhibits CD1d transcription. Therefore, LC freshly recovered from epidermal sheet were evaluated by flow cytometry. In accordance with in vitro observation, they did not expressed measurable levels of CD1d molecules at the cell membrane. Thus, TGFbeta produced by keratinocytes contribute to selectively downregulate CD1d expression on intraepidermal-resident LC.

Milia-like idiopathic calcinosis cutis.

Milia-like idiopathic calcinosis cutis is a rare entity. Only 17 cases have been reported so far. Two-thirds of these have been associated with Down syndrome. We report the fifth case occurring in a child without Down syndrome. Milia-like idiopathic calcinosis cutis has long been regarded as a peculiar subtype of idiopathic calcinosis cutis. The pathogenesis of the disorder remains unclear.

Vulvar cicatricial pemphigoid of childhood.

We report a 9-year-old girl with a vulvar autoimmune bullous dermatosis. A diagnosis of localized bullous pemphigoid or cicatricial pemphigoid was made on the basis of immunohistologic data. Since the lesions were unresponsive to topical corticosteroids but healed completely on dapsone at a dosage of 1.5 mg/kg/day, we favor the diagnosis of vulvar cicatricial pemphigoid. Only two such cases have been reported thus far. The diagnostic criteria and therapeutic modalities are discussed.

[Cutaneous lipids: from physiology to the clinical]. / Les lipides cutanés: de la physiologie à la clinique.

The primary function of the epidermis is to produce the waterimpermeable stratum corneum that permits terrestrial life. Extracellular glycosylceramides are the main precursors of the stratum corneum ceramides which play a role in the cohesion of corneocytes and the establishment of the barrier function. The physiological desquamative process depends upon the transformation of glycosylceramides to ceramides and of cholesterol sulfate to cholesterol. Intracellular ceramides participate to the terminal differentiation process of the epidermis. Abnormalities in the synthesis and/or the secretory mechanisms of the epidermal lipids induce various skin disorders. Such diseases may have a genetic background or result from the influence of environmental factors.