[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.

Joint clinical and pathological review meetings improve patient care: a prospective evaluation in dermatology.

BACKGROUND: Joint clinical and pathological review meeting exists in most academic dermatology departments. OBJECTIVE: The primary objective of the study was to assess the impact of the joint clinical and pathological review meeting in dermatology on patient care. METHODS: Prospective descriptive study over 6 months (May to October 2008) on all clinical cases of dermatology reviewed at the joint clinical and pathological review meeting in our University Hospital. RESULTS: A total of 139 cases were reviewed during the 6-month period. In 97 cases (69.8%), the joint clinical and pathological review meeting had a positive impact on final diagnosis and/or on patient management. For 27 cases, a consensus diagnosis different from the initial proposal was established. In 21 cases, the joint clinical and pathological review meeting led to additional investigations or therapeutic proposals. The impact of the joint clinical and pathological review meeting was highest for inflammatory skin diseases. CONCLUSIONS: The joint clinical and pathological review meeting is a useful procedure to improve diagnostic accuracy in difficult cases.

[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.

Incidence of auto-immune pemphigus in the Midi-Pyrénées region in 2002-2006.

INTRODUCTION: Auto-immune pemphigus is an organ-specific immune disorder due to pathogenic auto-antibodies. Both genetic and environmental factors have been associated with the occurrence of auto-immune pemphigus. Little is known about the epidemiology of auto-immune pemphigus in western Europe. OBJECTIVE: To evaluate the incidence of auto-immune pemphigus in south-western France (namely the Midi-Pyrénées region) in a 5-year period between 2002 and 2006. MATERIALS AND METHODS: We performed a retrospective study of the annual incidence of auto-immune pemphigus diagnosed in the Midi-Pyrénées region, between January 1, 2002, and December 31, 2006. Possible cases of pemphigus were initially selected using skin direct immunofluorescence (DIF) databases from all laboratories in the Midi-Pyrénées region. Systematic validation of all cases was performed by two of the authors (N.M., M.T.). To qualify as a case of auto-immune pemphigus, patients had to fulfil the following criteria: history of clinical signs of pemphigus as assessed by a dermatologist and a positive DIF. To be included in the study, validated cases of auto-immune pemphigus had to fulfil the following criteria: the date of first positive skin DIF between January 1, 2002, and December 31, 2006, and patient living in the Midi-Pyrénées region at the time of the first positive skin DIF. RESULTS: Between 2002 and 2006, 91 patients with positive DIF were identified. Fifty-four patients with positive DIF did not meet the validation criteria for the study. Thirty-seven cases were included in the analysis. The crude annual mean incidence of auto-immune pemphigus between 2002 and 2006 was 2.7 cases/10(6) inhabitants/year (95% CI: 1.87-3.69). The world-population-standardized annual mean incidence of auto-immune pemphigus was 1.55 (95% CI: 0.99-2.11). CONCLUSION: We report a 1.55/10(6) inhabitants/year world-population-standardized incidence of auto-immune pemphigus in the south-west of France. A nationwide epidemiological study of pemphigus should be performed in France.