[Acyclovir-resistant perineal HSV infection revealing chronic lymphoid leukaemia]. / Herpès chronique périnéal résistant à l'aciclovir révélateur d'une leucémie lymphoïde chronique.

BACKGROUND: Chronic HSV infection is a cause of chronic perineal ulcerations. We report a case of a chronic and refractory HSV infection revealing chronic lymphoid leukaemia. PATIENTS AND METHODS: An 85-year-old woman with an 8-month history of chronic perineal ulcerations was referred to our dermatology department. She had no previous medical history of herpes infection. Skin biopsies ruled out carcinoma but were consistent with HSV infection. A local swab was positive for HSV2. Treatment with valaciclovir and intravenous acyclovir (ACV) at the recommended doses was ineffective. Laboratory tests revealed type-B chronic lymphoid leukaemia. Molecular biology studies confirmed the presence of ACV-resistant HSV via decreased thymidine kinase activity (stop codon: M183stop). Foscarnet was administered for a period of 3 weeks with almost complete healing of the ulcerations. Treatment was stopped prematurely due to acute renal insufficiency and the remaining lesions were treated using imiquimod cream. Valaciclovir was prescribed to prevent further episodes. The condition recurred a mere 11 months later. DISCUSSION: The prevalence of ACV-resistant HSV is 0.32 % in immunocompetent patients and 3.5 % in immunocompromised patients. Insufficient dosing regimens or prolonged treatment with TK inhibitors result in the local selection of pre-existing mutant HSV viruses. Foscarnet, a DNA polymerase inhibitor, is the treatment of choice in HSV-resistant infections. ACV-resistant HSV is less virulent and replicates less, with reactivations being mainly due to wild-type HSV latent in the neural ganglia. Valaciclovir can be used as a preventive treatment. To our knowledge, this is the first case of ACV-resistant HSV infection revealing chronic lymphoid leukaemia. CONCLUSION: Chronic perineal ulcerations can be the first manifestation of immunodeficiency seen for example with haematological diseases. In the event of clinical resistance of an HSV infection to recommended thymidine kinase inhibitor regimens, the use of foscarnet should be considered.

Assessment of diagnostic criteria between primary cutaneous anaplastic large-cell lymphoma and CD30-rich transformed mycosis fungoides; a study of 66 cases.

BACKGROUND: Transformed mycosis fungoides (TMF) large cells may express CD30 antigen, and because of this, the differential diagnosis between CD30-rich TMF and primary cutaneous anaplastic large-cell lymphoma (cALCL) may be difficult, and especially in distinguishing cALCL associated with MF vs. CD30-rich TMF. OBJECTIVES: To find clinical, histological and molecular diagnostic features useful for differential diagnosis between cALCL and CD30-rich TMF. To analyse and compare the prognostic value of clinical and pathological factors in these two diseases. MATERIAL AND METHODS: We conducted a retrospective study (1999-2012) of 32 patients with cALCL and 34 with CD30-rich TMF, seen in reference centres of the French Study Group of Cutaneous Lymphoma. Clinical, histological and molecular features were analysed and compared to determine their diagnostic and prognostic value. RESULTS: Comparison of the two groups showed that age ˃ 60 years, ≥ 5 skin lesions, early progression, absence of spontaneous regression and trunk involvement were significantly associated with the diagnosis of TMF. Abnormal T-cell phenotype and perforin expression were significantly more frequent in cALCL (both P < 0·001). Overall survival (OS) at 5 years was 77·4% for cALCL and 20·7% for CD30-rich TMF. Stage T3, ≥ 5 skin lesions, lower limb involvement for cALCL and stage T4, extracutaneous involvement, B symptoms, high levels of lactate dehydrogenase for CD30-rich TMF were associated with poor OS and progression-free survival. DUSP22 gene rearrangement had no diagnostic or prognostic value. CONCLUSIONS: Clinical features and outcome are the most discriminative to differentiate the two entities. Even histological and molecular markers were not fully specific; abnormal vs. normal T-cell phenotype and perforin expression may constitute helpful tools.

Hepatitis B screening: who to target? A French sexually transmitted infection clinic experience.

BACKGROUND & AIMS: Hepatitis B virus (HBV) infection is a major public health burden in France and worldwide. Routine screening for hepatitis B is not currently recommended in France. Medical experts and public health agencies opinions can differ concerning targeting criteria. Our study aims at developing a risk assessment strategy for identifying possible hepatitis B cases among the patients consulting in a French Sexually Transmitted Infection (STI) clinic. METHODS: 6194 asymptomatic patients requesting an STI screening were also screened for hepatitis B infection. The association between hepatitis B surface antigen (HBsAg) positivity and/or total hepatitis B core antibody (anti-HBc) positivity and self-reported risk factors for hepatitis were analysed. RESULTS: Only male gender, lack of employment, and birth, in medium or high endemic country, were independently associated with HBsAg positivity in multivariate analysis. Sexual behaviour or self-reported vaccination status is therefore not necessary to target high-risk populations. These three simple criteria could save 25% of unnecessary tests and 6-16% undiagnosed hepatitis B compared to usual targeting criteria. CONCLUSIONS: To detect HBsAg carriers, only three simple targeting criteria, without taking into account the self-reported vaccination status or sexual behaviour, could improve screening efficiency and save unnecessary testing.

Sentinel lymph node biopsy for melanoma is becoming a consensus: a national survey of French centres involved in melanoma care in 2008.

BACKGROUND: The role of sentinel lymph node (SLN) biopsy in melanoma care remains controversial and is not included in most guidelines for the management of melanoma in Europe. OBJECTIVE: To evaluate the practice of SLN biopsy for melanoma. METHODS: In 2008, a self-administered questionnaire was mailed to physicians in 49 hospitals in France. RESULTS: Questionnaires were returned by 34 (69.3%). A median number of 90 new cases of melanoma were treated each year per centre. SLN biopsy was performed routinely in 21 (61.7%) centres. The practice of SLN biopsy for melanoma was recommended in the local guidelines in 53% of centres. The proportion of patients reported as undergoing SLN biopsy for melanoma was significantly higher in centres with local guidelines than in centres without local guidelines (33.4 ± 21.4% vs. 13.1 ± 21.8%; P = 0.003). Where the local guidelines recommended SLN biopsy (n = 21), it was advocated in the case of Breslow thickness ≥1.0 mm (76%) and/or ulceration of the primary melanoma (38%) and/or histological regression of the primary melanoma (24%). CONCLUSION: Our study may be considered representative of SLN practice in France. Contrary to current national guidelines for melanoma care in France, SLN biopsy is routinely recommended in the majority of centres. Our study shows that the practice of SLN biopsy for melanoma is increasingly performed in patients with intermediate Breslow melanoma.

[Anti-p200 pemphigoid: a spectacular response to dapsone]. / Pemphigoïde anti-p200 : réponse spectaculaire à la dapsone.

BACKGROUND: Types of subepidermal autoimmune bullous dermatosis (AIBD) are classified by anatomoclinical picture and target antigen. A new entity has recently been identified: anti-p200 pemphigoid. PATIENTS AND METHODS: An 82-year-old man consulted for a profuse pruritic bullous eruption refractory to the standard treatments for bullous pemphigoid (BP). Direct immunofluorescence examination of a skin biopsy revealed linear deposits of IgG and of C3 at the dermal-epidermal junction, but Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. Indirect immunofluorescence (IIF) testing of cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting was negative for a dermal extract but showed an antibody directed against a 200-kD epidermal antigen. A diagnosis of anti-p200 pemphigoid was eventually made and the patient was successfully treated with dapsone. DISCUSSION: The diagnosis of anti-p200 pemphigoid was made in this case in spite of discrepancy between the IIF and immunoblotting results, and despite the fact that the target antigen in this disease is considered as being restricted to dermal sites. Anti-p200 pemphigoid usually begins in the second part of life and differs from standard bullous pemphigoid in terms of more frequent mucous membrane and cephalic involvement, as well as a greater degree of miliary scarring. This disease appears more prominent in males and is associated with psoriasis in around one third of cases. Autoantibodies recognize laminin gamma-1, an extra-desmosomal protein that contributes to dermal-epidermal adhesion. CONCLUSION: This recently described disease as probably under-diagnosed in France. It should be considered in atypical presentations of bullous disease. Diagnosis is confirmed by immunoblotting detection of autoantibodies directed against a 200-kD antigen normally present in the extract. Dapsone appears to be the most effective treatment.

Topical methyl aminolevulinate photodynamic therapy for management of basal cell carcinomas in patients with basal cell nevus syndrome improves patient's satisfaction and reduces the need for surgical procedures.

BACKGROUND: In basal cell nevus syndrome, basal cell carcinomas occur in early life. The treatment of basal cell carcinomas requires surgical excisions and may lead to unaesthetic scars. Photodynamic therapy (PDT) is a validated treatment of skin cancers, with good cosmetic outcomes. OBJECTIVES: The aim of the study was to evaluate patient's satisfaction, cosmetic outcome and number of surgical excisions before and after PDT, in patients with basal cell nevus syndrome treated with PDT. METHODS: A cross-sectional evaluation of all patients with basal cell nevus syndrome, treated with PDT for basal cell carcinomas. A questionnaire evaluated satisfaction, cosmetic outcomes for surgery and PDT. The number of surgeries before and after PDT was noted and efficacy was evaluated. RESULTS: Seven patients were evaluated; 85% of patients were satisfied with PDT vs. 55% for surgery. The average visual analogue score for the cosmetic result was 8.42/10 for PDT vs. 6.3/10 for surgery. The mean number of surgical excisions was 4.4 during the 6 months before the first session of PDT and 0.57 after. CONCLUSION: Methylaminolevulinate-photodynamic therapy seems an interesting option for the treatment of basal cell carcinomas in patients with basal cell nevus syndrome.

[Recurrent breast cellulitis associated with lymphangiectasia after tumorectomy for breast cancer]. / Érysipèle récidivant associé à des lymphangiectasies après traitement conservateur d'un cancer du sein.

BACKGROUND: Recurrent breast cellulitis has been described as a complication following breast conservation therapy. OBSERVATION: A 50-year-old woman undergoing tumour excision, postoperative radiotherapy and chemotherapy presented recurrent breast cellulitis in the same region. The presence of lymphangiectasia suggested a complication subsequent to lymph stasis. DISCUSSION: Conservative therapy for breast cancer, allowing the development of subclinical or patent lymphœdema, constitutes a prominent risk factor for recurrent cellulitis. This complication has also been considered in patients with lower extremity cellulitis following saphenous venectomy for coronary bypass surgery. The unusual presence of lymphangiectasia observed in our patient provides clear evidence that lymphœdema is the most prominent risk factor for the development of cellulitis after breast conservation therapy.

[Hurt eczematiforme column (chronicle) of the breast after implementation of a surgical clip]. / Lésion eczématiforme chronique du sein après mise en place d'un clip chirurgical.

We report a case of atopic dermatitis in relation with a surgical titanium clip. Such a complication has only been reported once in the literature. We advocate to ask the patients about query atopic manifestations especially contact dermatitis to metal before any procedure involving metallic implants.

Prevalence and prognostic value of cutaneous manifestations in patients with myelodysplastic syndrome.

BACKGROUND: Skin manifestations associated with myelodysplastic syndrome (MDS) may reveal bone marrow transformation into acute myeloid leukaemia. OBJECTIVE: The objective of this study was to assess the prevalence of skin manifestations associated with MDS. In addition, we evaluated the risk of acute myeloid leukaemia transformation associated with skin manifestations. METHODS: We studied a cohort of 157 patients with primary MDS followed up prospectively for a median of 44 months. Skin lesions were prospectively assessed as part of medical examination every 6 months by a board certified dermatologist. Survival analyses were performed to assess the association between the presence of skin lesions and the risk of acute myeloid leukaemia. RESULTS: Fifteen patients (9.55%) experienced skin lesions previously reported as associated with MDS. These were neutrophilic dermatosis (7, 4.46%), specific lesions (5, 3.18%), cutaneous vasculitis (2, 1.27%) and Behçet disease (1, 0.63%). Survival analysis showed that the risk of transformation into acute myeloid leukaemia was slightly but not significantly increased in patients with skin lesions as compared with patients without skin lesions with a relative risk of 2.08 (95% CI 0.92-4.67). CONCLUSION: The prevalence of skin lesions, mostly neutrophilic dermatosis and specific lesions, is relatively high in patients with MDS. There is a trend for a higher risk of transformation into acute myeloid leukaemia in patients with skin lesions.

Histological regression in primary melanoma: not a predictor of sentinel lymph node metastasis in a cohort of 397 patients.

BACKGROUND: Regression has been proposed as a potential marker of dissemination in thin melanomas. Previous studies have shown conflicting results. OBJECTIVE: To determine if regression in melanoma is associated with an increased risk of sentinel lymph node (SLN) metastasis. METHODS: A cohort analysis was conducted. Data on all patients were collected on a standardized case report form during 10 years. A total of 397 consecutive patients with melanoma who underwent a SLN biopsy were analysed. All cases of melanoma and SLN biopsies were examined by the same two pathologists. Differences between melanomas with and without SLN metastasis were compared using Fisher's exact test or the two-sample t-test and the chi(2) test. Multivariable logistic regression was used to adjust for possible confounding factors. RESULTS: We analysed 397 patients (411 melanomas) who underwent a SLN biopsy. The median Breslow index was 1.8 mm (interquartile range 1.1-3). Regression was observed in 23% (n = 94). SLN metastases were observed in 26% (n = 106). The frequency of SLN metastasis was 16% in melanomas with regression and 29% without regression (P = 0.012). The adjusted odds ratio (OR) for regressive melanoma was 0.9 [95% confidence interval (CI) 0.4-1.9; P = 0.777]. The risk of SLN metastasis was increased in melanoma cases with a Breslow index from 1.5 to < 2.0 mm (adjusted OR 3.1; 95% CI 1.4-7.1; P = 0.006) and >or= 2.0 mm (adjusted OR 3.5; 95% CI 1.7-7.4; P = 0.001) and ulceration of the melanoma (adjusted OR 1.8; 95% CI 1.1-3.2; P = 0.03). CONCLUSION: Regression is not an independent predictor of the risk of SLN metastasis in melanoma.

[BCG infection of the glans penis after intravesical BCG therapy]. / BCGite du gland post-BCGthérapie intravésicale.

BACKGROUND: BCG therapy is an effective adjuvant treatment for superficial bladder tumors. Therapy involves intravesical instillation of live attenuated Calmette-Guérin bacilli. BCG infection of the glans is a rare local complication associated with this treatment, two cases of which are reported below. PATIENTS AND METHODS: Case 1: A 77-year-old man presented relapsing urothelial bladder carcinoma treated by endoscopic resection and BCG therapy. One week after the seventh instillation, severe balanitis developed. Three months later, examination revealed massive painful perimeatal ulceration with yellowish papules in the peripheral regions. Histology revealed epithelioid giant-cell granulomas. Ziehl-Neelsen staining was positive. Slow cure of the lesions was achieved within 12months using double antitubercular antibiotic therapy. Case 2: In a 61-year-old man receiving BCG therapy for relapsing bladder carcinoma in situ, the sixth instillation was considered traumatic since it was highly painful. One week later, papular nodules appeared on the glans with a sclerosing lesion of the balanopreputial sac, dark purple perimeatal papules and a mass beneath the mucosa of the glans. Antibiotic treatment comprising ofloxacin followed by rifampicin for two months proved ineffective. Histology revealed granulomatous dermal lesions with eosinophilic necrosis. Triple antitubercular antibiotic therapy was initiated. DISCUSSION: The first reported case of BCG infection of the glans in patients undergoing intravesical BCG therapy was published in 1992. Since then, there have been nine other reports. There is no stereotypical clinical presentation. In most cases, an infiltrated erythematosus plaque is seen together with yellowish papules in certain patients. Diagnosis is based upon history and histological examination.

[Addictions and psoriasis: an example of the dermatologist's implication in preventive medicine ?]. / Troubles addictifs et psoriasis : un exemple du rôle du dermatologue dans la médecine préventive ?

Association between psoriasis and addictive disorders has been longtime suspected and several studies are supporting the association of psoriasis and alcohol, and of psoriasis and tobacco. The association of psoriasis and other addictive disorders has not yet been reported. The association of psoriasis and alcohol is not restricted to alcoholism (defined as excessive alcohol consumption with psychic and/or psychic (correction of physic) dependence). It has been suggested that psoriasis is more closely linked to alcohol misuse than it is to alcoholism. The association of psoriasis and alcohol seems not been influenced by the gender, and shows a dose-effect relation. The most striking link between cigarette smoking and psoriasis has been established in palmo-plantar pustulosis. This link also seems to exist for other forms of psoriasis, with a dose-effect relation. Cigarette smoking could be involved in the high prevalence of lung cancer and cardio vascular disorders in psoriatic patients. There are a number of difficulties in the assessment of the correlation between psoriasis, cigarette smoking, and alcohol, and even more so in establishing a causal or etiologic relationship between the three, because of several confusion factors. This must not occult the reality of this association and its impact of psoriatic patients' health and the importance of detecting and preventing them. The detection and the prevention of the complications of these addictions belong to the dermatologists.

[Bexarotene therapy in folliculotropic cutaneous T-cell lymphoma]. / Lymphomes cutanés T folliculotropes traités par bexarotène.

BACKGROUND: Folliculotropic lymphoma is a variant of fungoid mycosis distinguished by its clinical features, histology, poor prognosis and poor response to the standard treatments for cutaneous T-cell lymphomas. The purpose of our study was to evaluate the efficacy and safety of bexarotene, an RXR receptor-selective retinoid, in the treatment of folliculotropic lymphoma after 3 months and 6 months of therapy. PATIENTS AND METHODS: This retrospective, prospective, descriptive study was conducted between October 2004 and November 2005. It was carried out using all available dossiers for patients with folliculotropic lymphoma treated with bexarotene. Patients were included where diagnosis of folliculotropic lymphoma was based on histological evidence, provided they had received at least one prior treatment (PUVA therapy, Retinol/PUVA therapy, Caryolysine, Bicnu, methotrexate), and the affected body surface area could be calculated from initial whole-body photographs using the rule of 9. RESULTS: Eight patients were included, all males. Partial remission was seen after 3 months and 6 months of treatment in 75% of patients. According to the Physical Global Assessment Scale (7-item evaluation scale), 75% of patients presented global improvement after 3 months of treatment compared with 87.5% after 6 months. Five of the 8 patients experienced sexual dysfunction while on treatment with bexarotene, which resolved one month after discontinuation of therapy in four cases. Onset or worsening of dyslipidaemia was seen in all patients, with five developing central hypothyroidism. DISCUSSION: These global response levels confirm the place of bexarotene in the treatment of folliculotropic lymphoma. Combined therapy appears to be warranted due to the difficulty in maintaining optimal dosages of the drug because of the frequency of adverse effects. Preventive therapy for the hypothyroidism seen in practically all cases should prevent adverse effects of the drug on sexual function.

[Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis: role of gadolinium?]. / Dermopathie néphrogénique fibrosante traitée par photochimiothérapie extracorporelle: rôle du gadolinium?

BACKGROUND: Nephrogenic fibrosing dermopathy is a cutaneous and systemic sclerosis affecting patients with renal failure. CASE-REPORT: A 68-year-old man with renal insufficiency and on dialysis developed hardening of the skin and severe joint contractions. He had previously undergone angiography with gadolinium-containing contrast agents. A skin biopsy confirmed nephrogenic fibrosing dermopathy. The patient was treated by oral steroids followed by extracorporeal photopheresis. An improvement was seen after 12 cycles. DISCUSSION: Treatment of nephrogenic systemic fibrosis is not codified and is normally based on the methods used for other forms of systemic sclerosis. Six cases of patients showing improvement under extracorporeal photopheresis have been published. The physiopathology of the disease is unknown. Gadolinium could act as a triggering agent by attracting circulating fibrocytes in the dermis of patients. Medical authorities recommend avoidance of gadolinium in patients with advanced kidney failure unless strictly necessary.

Histological evolution of recurrent basal cell carcinoma and therapeutic implications for incompletely excised lesions.

BACKGROUND: It has been proposed that the management of incompletely excised recurrent basal cell carcinomas (BCCs) should depend on their histological appearance, and that nonaggressive recurrent BCCs may not require re-excision. OBJECTIVES: To determine the histological evolution of recurrent BCCs. METHODS: In a 14-year retrospective study analysing histological sections of recurrent BCCs, 390 specimens from 191 patients were blindly classified by three physicians into aggressive and nonaggressive types according to Sexton's classification. Initial histological sections were available for 33 of the recurrent BCCs. Descriptive analysis was performed. RESULTS: Eight of 33 (24%) recurrent BCCs became histologically more aggressive. Four of 20 (20%) originally nonaggressive BCCs became aggressive during recurrence and four of 13 (31%) originally aggressive BCCs showed a more aggressive component during recurrence. These incompletely excised aggressive BCCs were sited in periorbital and perinasal areas and on the cheek, and were re-excised. CONCLUSIONS: Management of incompletely excised nonaggressive BCCs (nodular or superficial types) is still a matter of debate. Previously reported studies have shown recurrence in < 10% of nonaggressive incompletely excised BCCs. Our study showed that rare recurrences of these initially nonaggressive BCCs showed an aggressive component in 20% of cases. These results suggest that initially nonaggressive incompletely excised BCCs do not require re-excision except if they are located in sites with a poor prognosis.

[Trigeminal neuralgia presenting as a deep recurrent desmoplastic neurotropic melanoma of a lentigo maligna]. / Névralgie faciale révélatrice d'une récidive desmoplastique profonde et neurotrope d'un mélanome de Dubreuilh.

INTRODUCTION: Neurotropic melanoma is a particular anatomopathological form corresponding to dermal proliferation of desmoplastic cells of neuroid differentiation. We report a new case of neurotropic melanoma revealed by facial neuralgia. CASE REPORT: A 64 year-old man presented in 1996 with a lentigo maligna on the right cheek treated by complete excision. After 2 years of medical supervision, a pigmented lesion recurred leading to new surgical treatment. The histological examination of the total lesion showed intra-epidermal atypical melanocyte proliferation without dermal invasion. In 1999, right trigeminal neuralgia occurred without associated cutaneous change. Cranial MRI revealed an infiltration of the right trigeminal nerve. Endo-buccal surgery disclosed a black swelling of the trigeminal nerve. Histological examination and immunohistochemistry revealed a desmoplastic melanoma. DISCUSSION: Neurotropic melanoma with nerve invasion by malignant cells presenting as a trigeminal neuralgia is rare. Our case report underlined the depth of the neurotropic melanoma and the initial existence of a lentigo maligna without associated "neurotropic" melanoma.

[Palisading granuloma of the penis: a variant of subcutaneous granuloma annulare]. / Granulome palissadique du pénis: une variante de granulome annulaire profond.

INTRODUCTION: Subcutaneous granuloma annulare on the penis has only been reported exceptionally. OBSERVATION: A 41 year-old man presented with four small, asymptomatic nodules on the glans penis. Histological examination of a biopsy revealed a deep palisading granuloma progressing not only around the alcian-blue necrobiosis areas but also around the smooth muscle fibers. The lesion regressed spontaneously after two years of progression. DISCUSSION: Palisading granuloma of the penis appears to be a most particular clinical form of subcutaneous granuloma annulare. It is characterized by small single or multiple nodules localized on the penis. On histological examination, the granuloma is deep and concentrates around the network of smooth muscle fibers of dartoic origin.

Ivermectin-responsive Demodex infestation during human immunodeficiency virus infection. A case report and literature review.

We report the case of a 56-year-old HIV-seropositive man who presented a facial Demodex infection developed 2 months after initiation of highly active antiretroviral therapy. The Demodex infection was confirmed by scrapings and histopathologic examination and by the dramatic response to antiparasitic treatment with oral ivermectin associated with 5% permethrin cream.

Gynaecomastia in a male patient during stavudine and didanosine treatment for HIV infection.

We report a case of gynaecomastia developed in a HIV-seropositive man, associated with a severe lipodystrophy. We hypothesize the responsibility of stavudine and didanosine in the development of these 2 complications. If many reports suggest that the protease inhibitors may promote gynaecomastia, long-term nucleoside analogue therapy may also cause this side effect.