[CONDYDAV: A multicentre observational study of patients presenting external genital warts in France]. / CONDYDAV : étude observationnelle multicentrique des patients présentant des condylomes dans les dispensaires antivénériens en France.

BACKGROUND: Since 2007 in France, human papilloma virus (HPV) vaccination has been licensed for use as a vaccine against HPV 6, 11, 16 and 18. The impact on the epidemiology of external genital warts (EGWs) in a large population remains unclear. OBJECTIVES: To determine epidemiologic and clinical features of patients presenting EGWs in France in the era of HPV vaccination. PATIENTS AND METHODS: In this prospective, observational study, we analyzed clinical features and treatments between January 1st, 2012 and March 31, 2012 for patients consulting for EGWs at 15 STI clinics throughout France. RESULTS: A total of 372 men and 111 women were included; mean age 31.2 years. The women were younger than the men (31.7 and 28.9 years respectively P<0.05). Among the patients, 416 (85.7%) were heterosexual, 13 bisexual and 54 (11.2%) homosexual, including one female. Males reported more sexual partners in the last 12 months (more than 3 partners in 32.6% versus 11.9%, P<0.01). Among the men, 230 had involvement of the penis alone and 46 had involvement of the anus alone. Seventy-six patients had EGWs of the anus, and of these 26 were MSM. In females, 76 had an infection of the vulva alone and 22 co-infection of the vulva and anus. MSM and females were at higher risk than heterosexual males for anal involvement (P<0.0001 and P=0.004, respectively). Three women had been vaccinated: two with Gardasil® and one with Cervarix®. Cryotherapy was the preferred treatment. CONCLUSION: With the advent of HPV vaccination, a global strategy for the prevention and treatment of EGW should be implemented.

[Localised de novo dominant dystrophic epidermolysis bullosa]. / Epidermolyse bulleuse dystrophique localisée dominante de novo.

INTRODUCTION: Dystrophic epidermolysis bullosa is a hereditary heterogeneous blistering disease. Clinical examination and additional tests are not always sufficient to identify the subtype or mode of transmission. We describe a case of de novo dominant inherited dystrophic epidermolysis bullosa localised strictly to the knees. CASE REPORT: A 3-year-old boy presented symmetrical lesions on the anterior aspect of the knees since starting to walk. No nail, dental or mucous dystrophy was observed and the parents presented no clinical abnormalities. Optical microscopy, electron microscopy and immunofluorescence analysis suggested dystrophic epidermolysis bullosa. The genealogical tree allowed no distinction between the dominant de novo and mitis recessive forms. Genetic analysis identified a missense G 1776W mutation at exon 61 of gene COL 7A1 in the child's DNA but not the parents'. DISCUSSION: Dystrophic epidermolysis bullosa may present in generalized or localized forms and the disease may be inherited in either autosomal dominant or recessive mode. Genetic analysis shows mutations in COL 7A1. While the clinical features often allow different types to be distinguished when the parents do not have the disease (with the recessive forms being more severe), genetic analysis is essential to confirm the mode of inheritance. In the dominant forms, and more recently in recessive cases, glycine substitutions have been implicated, although the precise role of glycine substitution has yet to be clarified. Localised involvement of the skin alone, as seen in our case report, is very rare. CONCLUSION: Genetic analysis is important for genetic counselling and determination of risk of recurrence.

[Lyme disease could mimic dermatomyositis]. / Borreliose de Lyme mimant une dermatomyosite.

UNLABELLED: We report a dermatomyositis associated with Lyme disease. OBSERVATION: A 73-years-old woman has developed for 5 months an asthenia, a periorbital oedema and a forearm's skin infiltration without other signs suggesting of dermatomyositis. Laboratory studies showed an elevation of muscular enzymes, and inflammation signs. The skin and the muscles biopsies were compatible with the diagnostic of dermatomyositis. The patient was seropositive for Lyme disease. The patient was efficiently treated with doxycycline. DISCUSSION: Lyme disease could mimic a dermatomyositis. Indeed, Lyme disease should be considered as a differential diagnosis of dermatomyositis.

[Benign familial Degos disease]. / Forme familiale bénigne de maladie de Degos.

INTRODUCTION: Degos' disease or atrophic malignant papulosis is defined by porcelain white cutaneous lesions with atrophic scarring, often associated with severe and fatal systemic involvement (visceral and neurological). Benign forms are rare or under-reported and the familial forms are exceptional. It is a very rare disease, only two hundred cases have been reported in the literature with a sex ratio of 3M/1F. The pathogenesis of Degos' disease remains controversial. The exceptional observation of familial form raises the question of a genetic predisposition of this disease or an infectious aetiology with a low virus. OBSERVATION: A 41 year-old woman was known to have Degos' disease for 26 years with only cutaneous manifestations. One of her two sons developed atrophic cutaneous lesions at the age of 20. In both patients, no thrombotic or immunological abnormalities were found. The karyotype was performed with normal results. DISCUSSION: Degos' disease or malignant atrophic papulosis can have a long lasting benign evolution. Our patient, who had presented a benign form for 26 years, had the longest evolution ever documented in literature. We cannot be sure that her son will have a benign course of his Degos' disease because the diagnosis is recent and because the systemic involvement can appear after many years of evolution. In the familial forms, from our study and the 31 cases previously described in the literature, with ten different families, the course of the disease seems to be less severe than in sporadic forms. Among these familial forms of Degos' disease, only 4 patients presented a malignant form, which in one case did not prove a relationship between the death and the Degos' disease. Are the sporadic forms with only skin involvement less frequently reported? Has the familial form of Degos' disease the same course as a very severe common sporadic form?