Penoscrotal Paget's disease.

Paget's disease (PD) denotes an initially intra-epidermal adenocarcinoma that can later invade the dermis and metastasise. Among the extramammary forms of PD (EMPD), penoscrotal presentations are rarer than the vulvar and perianal forms. Once diagnosis has been confirmed by histopathological examination, a search for associated neoplasia must be conducted, although penoscrotal EMPD is less frequently associated with underlying neoplasia than mammary PD (MPD). The associated cancer most often involves a neighbouring organ, with prostate cancer being the most common, or in some cases consists of underlying cutaneous adnexal tumours. First-line therapy consists of surgical excision. Alternatives to surgery (imiquimod, CO2 laser vaporisation, dynamic phototherapy) may be considered in certain cases.

[Penile intra-epithelial neoplasia]. / Les néoplasies intra-épithéliales du pénis.

INTRODUCTION: Penile intra-epithelial neoplasia (PIN) are precancerous lesions, characterized by architectural and cytological abnormalities of the genital epithelium, from which can arise squamous carcinomas. METHODS: A literature review was performed on the Medline database, considering the articles listed between October 2007 and October 2017 dealing with PIN, using the following keywords "intraepithelial neoplasia" or "Bowen's disease" or "erythroplasia of Queyrat" or "bowenoid papulosis" and "penis ¼. Papers were selected according to their language (English and French) and their relevance. RESULTS: One hundred seventy four articles related to PIN were listed. Twenty-five of them were selected for their relevance. The analysis of the references of these articles identified 6 relevant papers published before October 2007, which were considered for this review based on a total of thirty-one articles. We describe clinical and pathological characteristics of PIN, emphasizing treatment modalities. CONCLUSION: Urologists should distinguish HPV-related and non HPV-related PIN, both of them sharing clinical presentation, but needing different management.

[Precancerous lesions of the buccal epithelium]. / Les lésions précancéreuses épithéliales buccales.

Squamous cell carcinomas of the oral cavity and lips consistently have a high incidence (they constitute the fifth most common form of cancer in France) and carry a heavy prognosis, particularly if diagnosed late. According to different studies, between 10 and 80% of such carcinomas occur in a pre-existing or precancerous lesion. The World Health Organisation (WHO) recommends the use of two terms for such lesions: precursor lesions, i.e. histological lesions associated with intraepithelial neoplasia (IEN) and verrucous hyperplasia (VH), frequently resulting clinically in leukoplakia or erythroplakia; "at risk" lesions, which include lichen planus, submucosal oral fibrosis and certain forms of genodermatosis.

Plantar keloids: diagnostic and therapeutic issues in six patients.

BACKGROUND: Keloids are benign fibro-proliferative skin lesions that very rarely occur on the soles. Because of their rarity, the diagnosis of plantar keloids can be difficult. We describe the clinical and histopathological characteristics of eight plantar keloids. METHODS: All patients presenting with plantar keloids between 2005 and 2012 in our Dermatology unit were retrospectively included. Diagnosis was definitely established by re-reading of pathological slides in all cases. Clinical characteristics, histopathological features, treatments given and their results were collected. RESULTS: Six patients were included. Five patients had a single plantar keloid and one had three lesions. They all were of African descent. Only one patient remembered of a previous injury at the site of the keloid. Three patients presented with associated extra-plantar keloids. In four patients, the diagnosis of keloid was not initially suspected clinically or histologically. Re-reading of the clinical photographs showed that the eight plantar keloids shared common morphological features, leading to a distinctive clinical picture, defined by a hardened lesion of rounded or polycyclic shape, with a pink surface crossed by keratotic furrows and the presence of a hyperkeratotic rim. Concerning pathological features, typical hyalinized collagen can be missing and deep fibrosis should not rule out the diagnosis of keloid. Intralesional injection of triamcinolone acetonide and orthopaedic shoes were useful. All patients who had surgical excision presented recurrence. CONCLUSION: The knowledge of the clinical features of plantar keloids is helpful to the diagnosis. There is no well-established treatment, but supportive measures are important.

Anatomoclinical study of 30 cases of sclerosing sweat duct carcinomas (microcystic adnexal carcinoma, syringomatous carcinoma and squamoid eccrine ductal carcinoma).

BACKGROUND: Microcystic adnexal carcinoma (MAC), syringomatous carcinoma (SC) and "Squamoid eccrine ductal carcinoma" (SEDC) are rare sclerosing adnexal tumours. OBJECTIVE: To understand the histogenesis of these tumours and possible clinical implications. METHODS: We conducted a retrospective study of 30 cases, 18 MAC, 5 SC and 7 SEDC reviewed and classified by a panel of dermatopathology experts, with immunohistochemical analysis of keratins, including K77, a new keratin specific of eccrine ducts, and PHLDA1 expressed in adnexal structures. RESULTS: There was a strong female predominance, with only five cases occurring in men. Patients with MAC and SC were younger (mean age 56 and 47 years) than those with SEDC (mean age 81 years). The most common localization was the cheek in SC and SEDC and the periocular area in MAC. Two cases of SEDC were found in organ transplant patients. No recurrence or metastases were observed after complete surgery of MAC, or SC (mean follow-up 7.2 years and 4.7 years), whereas one case of SEDC recurred and another could not be fully excised. MAC and SC had similar histological features, except for cysts. In MAC, calcifications, granulomas, connection to follicles, keratin expression pattern, PHLDA1 positivity and K77 negativity indicated a follicular histogenesis, whereas in SC, K77 positivity and keratin expression pattern were consistent with a differentiation towards eccrine apparatus. SEDC was composed of strands centred by ducts and nests with squamous differentiation and displayed K77 ductal positivity in all cases, a finding consistent with an eccrine origin. CONCLUSION: Our study demonstrated that MAC and SC have similar clinical characteristics, although histogenesis differs and show arguments for the individualization of SEDC.

Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients.

BACKGROUND: The prognostic and therapeutic features of scleredema are poorly documented. OBJECTIVES: To describe the characteristics of patients with scleredema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions and course. METHODS: We conducted a retrospective multicentre study. RESULTS: We identified 44 patients (26 men).The mean age at diagnosis was 53.8 years. The most common associated disorders were endocrine/metabolic diseases including 30 patients suffering from diabetes, mostly type 2 diabetes. Monoclonal gammopathies were confirmed in five cases. A preceding respiratory tract infection was not a feature. Treatments with different combination or sequential modalities were used with variable results. Phototherapy (UVA1 or PUVA) was the treatment associated with higher, although partial response. Systemic corticosteroids and immunosuppressive drugs were reserved to patients with severe disease in whom phototherapy had failed or for patients with multiple myeloma. Forty-one patients were followed up (mean period: 32.2 months).Thirty-nine patients are alive, 30 with and 9 without skin disease. Two patients died of cardiovascular complications due to myeloma and severe diabetes. CONCLUSIONS: Scleredema is a chronic debilitating disease associated with diabetes and metabolic syndrome, unresponsive to various treatments but not necessarily a life-threatening condition. Although there is no definitive treatment, phototherapy should be attempted first. Treatment of primary disease including strict glycaemic control combined with physical therapy should be also employed.

[Dissecting cellulitis of the scalp: a retrospective study of 7 cases confirming the efficacy of oral isotretinoin]. / Cellulite disséquante du cuir chevelu: étude rétrospective de 7 cas confirmant l'efficacité de l'isotrétinoïne per os.

BACKGROUND: Dissecting cellulitis of the scalp (DCS) is a rare and long-standing disease and its aetiopathogenesis remains unclear. Various therapeutic alternatives exist. We attempted to assess the efficacy of oral isotretinoin in a series of patients. PATIENTS AND METHODS: Seven cases of DCS followed and treated by one of the authors at the Sabouraud Centre in the Saint-Louis Hospital between 2007 and 2013 were retrospectively studied and the efficacy of oral isotretinoin was evaluated. RESULTS: The seven patients included were males aged between 22 and 39 years, with alopecic nodules on the occiput (two cases) or the top of the vertex (five cases). Most patients were given oral isotretinoin at a dosage of 0.75mg/kg/day for at least nine months. The outcome was satisfactory with complete healing of the lesions and good regrowth in all cases. Follow-up after recovery lasted between 16 and 42 months. CONCLUSION: Based on published studies and the results of the present study, oral isotretinoin at a dose of 0.75mg/kg/day may be proposed as first-line treatment for DCS.

First evidence of bacterial biofilms in the anaerobe part of scalp hair follicles: a pilot comparative study in folliculitis decalvans.

BACKGROUND: The cause of folliculitis decalvans (FD) remains unknown. We hypothesized that a bacterial biofilm could be involved in its pathogenesis. OBJECTIVE: To assess the presence or not of a bacterial biofilm in the hair roots of the scalp in FD. PATIENTS AND METHODS: Hairs plucked from four patients and three controls were examined by field emission scanning electron microscopy (FESEM) and confocal laser scanning microscopy (CLSM). RESULTS: Bacterial communities organized as biofilms were observed both by FESEM and CLSM in the under infundibular part of hair follicles in all patients and in two of the three controls. In patients and controls, these biofilms were formed exclusively of bacilli of comparable shapes. CONCLUSION: This pilot study provides the first evidence of the presence of bacterial biofilms in the infra infundibular part of human scalp hair follicles. These biofilms were detected both in FD patients and controls, suggesting their ubiquity as a commensal biofilm with a possible pathogenic shift in FD.

[The value of biopsy in the diagnosis of penile dermatoses]. / Intérêt de la biopsie cutanée dans le diagnostic des dermatoses péniennes.

BACKGROUND: Penile biopsy can be useful to establish an accurate diagnosis when managing male genital dermatoses. We investigated the value of genital biopsy by comparing suspected clinical diagnosis with final histologic diagnosis. PATIENTS AND METHOD: We retrospectively examined the clinical file of men with genital dermatoses undergoing genital biopsy. We collected details of age, biopsy site, suspected diagnosis after clinical examination and histologic diagnosis. RESULTS: One hundred and thirty-eight male patients were enrolled in the study. Their mean age was 52 years. Most biopsies (41%) were performed on the glans. Microscopic examination failed to rule out a diagnosis in 14.5% of the cases. It confirmed the clinically suspected diagnosis in 29.7% of cases and ruled out a particular diagnosis from among several clinical hypotheses in 26.8% of cases. The histological diagnosis had not been suspected in 18.1% of cases. DISCUSSION: Penile biopsy has an important role in the diagnosis of penile dermatoses. In our study, in 44.9% of cases, a clinically unsuspected diagnosis was identified or a differential diagnosis was resolved.

[Metastatic extramammary Paget's disease of the scrotum]. / Maladie de Paget du scrotum d'évolution métastatique.

BACKGROUND: Extra-mammary Paget's disease (EMPD) is currently considered a slowly progressing in situ adenocarcinoma. It is much more rarely associated with an invasive underlying adenocarcinoma than in mammary sites, where this association is almost always seen. However, an invasive course is to be feared where EMPD has been progressing for several years. PATIENTS AND METHODS: A 59-year-old man consulted for treatment of scrotal Paget's disease present for over 15 years and treated with topical emollients alone. Two infiltrated areas had recently appeared and their excision led to a diagnosis of invasive Paget's disease at these two points. Metastasis occurred 10 months later and rapidly proved fatal. DISCUSSION: Until now, non-invasive EMPD has been considered a fairly non-aggressive disease due to its indolent progression. However, it is characterised by considerable risk of relapse following surgical excision, regardless of the surgical margin. Despite a good prognosis at the intraepithelial stage, the rising life expectancy of the population means a greater likelihood of an invasive course or of the appearance of an underlying adenocarcinoma, and management of this condition can thus no longer be neglected. The present clinical case underscores the need for closer surveillance of the disease as of the intraepithelial stage.

[Post-radiation atypical vascular lesions and angiosarcoma: 11 cases]. / Lésions vasculaires atypiques et angiosarcomes post-radiothérapie : 11 cas.

BACKGROUND: Post-radiation atypical vascular lesions of the skin display clinical and morphological overlap with well-differentiated angiosarcomas, and correct diagnosis may be difficult. PATIENTS AND METHODS: We studied clinical, histological and immuno-histochemical aspects (CD31, CD34, D2-40 and VEGFR-3) of eight post-radiation atypical vascular lesions comparatively with three post-radiation angiosarcomas. RESULTS: All patients were female and received radiation therapy for breast carcinoma. On average, atypical vascular lesions occurred 4.3 years after radiation therapy and presented as small papulonodules or erythematous plaques. The clinical course after simple excision was benign. Histologically, they were relatively circumscribed lesions and showed slit-like vessels dissecting dermal collagen in all cases. On average, angiosarcomas occurred 5 years after radiation therapy and presented as more extensive lesions with a more aggressive clinical course. The lesions showed histological overlap with atypical vascular lesions, but were poorly circumscribed, with deeper invasion, cytological atypia and mitosis. Although the immuno-histochemical profiles were similar, expression of VEGFR-3 was greater in two cases of angiosarcoma. CONCLUSION: Post-radiation atypical vascular lesions are benign lesions that display clinical, histological and immuno-phenotypic overlap with well-differentiated angiosarcoma, and diagnosis requires good clinicopathological correlation. VEGFR-3 may be useful for differential diagnosis, as well as amplification of the MYC gene.

[Clinical features of Spitz naevus in children: a retrospective study of 196 cases]. / Caractéristiques du nævus de Spitz chez l'enfant. Étude rétrospective de 196 cas.

AIMS: To better define the characteristics of Spitz naevus (SN) in children, to determine whether it was clinically diagnosed and to examine the differential diagnoses made according to age. In addition, to determine whether atypical spitzoid tumors (AST) have a different presentation from other forms of SN. PATIENTS AND METHODS: A two-centre retrospective survey was made of histopathological reports written over a 4-year period in children aged under 18 years. The inclusion criterion was unequivocal diagnosis of SN or AST. Age, gender, site, size, course, excision methods, presumptive clinical diagnoses and the percentage of correct diagnosis were analyzed for four distinct age groups. RESULTS: One hundred and ninety-six patients were included, 186 with SN and 10 with AST. Mean age at diagnosis of SN was 9 years. Female predominance and predilection for the lower limbs were seen for all age groups. Facial involvement was less frequent and chiefly affected children aged under 11 years. Most SN lesions measured between 4 and 8mm. They were often confused with either pyogenic granuloma or juvenile xanthogranuloma, mainly before the age of 11 years. An accurate diagnosis was made in 29% of cases, chiefly in the 0 to 5 year-old age group. No cases of AST were clinically recognized, but it was diagnosed occasionally on histological grounds for very small tumours and in very young children. CONCLUSION: Clinical diagnosis of SN is not always straightforward and in this study, AST exhibited no special features allowing it to be distinguished from SN. These results underline the need for caution in the event of SN in children, regardless of age or lesion size.