International validation of the Bullous Pemphigoid Disease Area Index severity score and calculation of cut-off values for defining mild, moderate and severe types of bullous pemphigoid.

BACKGROUND: The Bullous Pemphigoid Disease Area Index (BPDAI) score has been proposed to provide an objective measure of bullous pemphigoid (BP) activity. OBJECTIVES: The objective of this study was to calculate BPDAI cut-off values defining mild, moderate and severe BP. We also aimed to assess the interrater reliability and correlation with the number of daily new blisters, and anti-BP180 and anti-BP230 antibodies. METHODS: Severity scores were recorded by two blinded investigators. Anti-BP180 and anti-BP230 antibodies were measured using an enzyme-linked immunosorbent assay (ELISA). Cut-off values defining mild, moderate and severe subgroups were calculated based on the 25th and 75th percentiles of the BPDAI score. RESULTS: In total, 285 patients with BP were enrolled from 50 dermatology departments in Europe. Median BPDAI activity was 37·5 points (range 0-164). Cut-off values corresponding to the first and third quartiles of the BPDAI score were 20 and 57, respectively; thus, these values were used to define mild (≤ 19), moderate (≥ 20 and ≤ 56) and severe (≥ 57) BP. The median BPDAI score for patients with ≤ 10 daily new blisters was 26 [interquartile range (IQR) 17-45], and for patients with > 10 daily new blisters the median score was 55 (IQR 39-82). The BPDAI intraclass correlation coefficient measured at baseline was 0·97 and remained higher than 0·90 up to month 6. The improvement in the BPDAI score was correlated with the absolute decrease in anti-BP180 ELISA value (Spearman's rank r = 0·34, P < 0·004), but not with anti-BP230 antibodies (r = 0·17, P = 0·15). CONCLUSIONS: This study suggests cut-off values of 20-57 for BPDAI to distinguish mild, moderate and severe BP, and confirms that it is a robust tool to assess BP severity precisely.

[Drug-induced Sweet's syndrome related to hydroxychloroquine: About 2 cases]. / Syndrome de Sweet médicamenteux à l'hydroxychloroquine : à propos de 2cas.

INTRODUCTION: Hydroxychloroquine is widely prescribed in systemic lupus erythematosus. Dermatologic adverse drug reactions are rare but can mimic a disease specific manifestation of lupus. Exceptionally, Sweet's syndrome, or acute febrile neutrophilic dermatosis, may be drug-induced. CASE REPORTS: Two patients aged 31 and 42 years were treated with hydroxychloroquine for systemic lupus and Sjogren's syndrome, respectively. Three weeks after starting treatment, they had a febrile, purple and erythematous papular rash of the trunk and limbs. There was a biological inflammatory syndrome and skin biopsy disclosed an infiltrate of the dermis rich in neutrophils. Lesions regressed after stopping hydroxychloroquine and introducing systemic corticosteroid therapy. Allergologic tests discussed the differential diagnosis with a delayed-type hypersensitivity reaction. CONCLUSION: We report two exceptional cases of drug-induced Sweet's syndrome related to hydroxychloroquine treatment in autoimmune rheumatic diseases.

Risk factors for treatment failure in scabies: a cohort study.

BACKGROUND: Treatment failure, which occurs in about one-third of cases, is considered as a major factor in the increasing incidence of scabies in developed countries. OBJECTIVES: To identify predictors of treatment failure of scabies in ambulatory populations. METHODS: This multicentre study compared the clinical characteristics and treatment modalities between a group of patients with scabies treated successfully and another group who were not cured 3 months after antiscabies treatment. RESULTS: In total 210 patients with a diagnosis of scabies were included, comprising 98 patients in the treatment success group and 112 in the treatment failure group. The main risk factors for treatment failure were (i) the use of only one type of treatment, topical benzyl benzoate (BB) or oral ivermectin, vs. the combination of both treatments [odds ratio (OR) 2·15, 95% confidence interval (CI) 1·22-3·77]; (ii) the use of a single intake (vs. two) of oral ivermectin (OR 10·2. 95% CI 4·49-23·2); (iii) intake of ivermectin during a meal vs. on an empty stomach (OR 4·31, 95% CI 1·89-9·84); (iv) absence of decontamination of furnishings (OR 8·72, 95% CI 3·50-21·8), in particular sofa and cushions (OR 5·90, 95% CI 2·34-14·9), mattresses (OR 4·16, 95% CI 1·35-12·8) or car seats (OR 6·57, 95% CI 3·27-13·2) and (v) absence of written documents explaining treatment modalities (OR 5·18, 95% CI 2·57-10·4). In multivariate analysis, treatment failure was mainly associated with (i) use of a single intake (vs. two) of ivermectin (OR 6·62, 95% CI 2·71-16·2); (ii) use of BB alone vs. two intakes of ivermectin (OR 3·51, 95% CI 1·55-7·95) and (iii) absence of decontamination of furniture with acaricides (OR 5·81, 95% CI 1·96-16·7). CONCLUSIONS: Use of topical BB alone and a single intake (vs. two) of ivermectin are predictors of treatment failure.

[Mycosis fungoides in a heart transplant recipient]. / Mycosis fongoïde après transplantation cardiaque.

BACKGROUND: Skin cancer occurs frequently in organ transplant patients as a result of induced immunosuppression. Most cases involve carcinomas or B-cell lymphomas induced by the Epstein Barr virus (EBV). Cutaneous T-cell lymphomas remain rare. We report a case of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant recipient. PATIENTS AND METHODS: A 68-year-old man who had received a heart transplant 21years earlier and was being treated with tacrolimus, mycophenolate mofetil and prednisolone had been presenting a psoriasiform rash on his trunk, limbs and head for 4years. The rash was resistant to both PUVA therapy and topical corticosteroids. Histopathological examination suggested epidermotropic cutaneous T-cell lymphoma. There was no impairment of the patient's general state of health nor any adenopathy. Molecular biology revealed TCR rearrangement in both blood and skin. Screening for circulating Sézary cells was negative, and PET scan revealed no signs of extracutaneous localization. Mechlorethamine showed little efficacy, bexarotene was complicated by dysthyroidism, hypertriglyceridemia was ineffective, methotrexate was contraindicated because of calcineurin inhibitor-related chronic kidney failure, and interferon could not be given due to the context of heart transplantation. Finally, we treated our patient with gemcitabine, which initially proved effective but was later complicated by septic shock that resulted in the patient's death. CONCLUSION: The particularities of our observation are the onset of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant patient, and the therapeutic difficulties encountered in a setting of transplantation with immunodepression.

[Syphilitic osteitis in an HIV-negative patient]. / Ostéite syphilitique chez un patient séronégatif pour le VIH.

BACKGROUND: Secondary syphilis with skeletal involvement is rare; herein we report a case concerning an HIV-negative patient. PATIENTS AND METHODS: During the course of secondary syphilis, a 28-year-old male homosexual, HIV-negative and with no medical history, presented intense and localized headaches persisting despite three weeks of antibiotic therapy. Bone scintigraphy revealed three bone lesions evocative of syphilitic osteitis, for which prolonged antibiotic therapy was instituted. DISCUSSION: Few cases of syphilitic osteitis have been described in the recent literature and these are linked to haematogenous diffusion of Treponema pallidum. Skeletal disease is suggested when febrile bone pain is present. Bone scintigraphy remains the best diagnostic tool and treatment comprises prolonged penicillin G or ceftriaxone.

Treatment of earlobe keloids by surgical excision and cryosurgery.

BACKGROUND: The long-term results of keloid treatments remain poor. OBJECTIVE: To assess the long-term efficacy of a technique combining surgical excision and immediate cryosurgery in patients with ear keloids. METHODS: A retrospective study was performed between January 2001 and June 2011. All patients referred for treatment of an ear keloid and undergoing subsequent surgical excision followed by immediate freezing of the postoperative wound using cryosurgery were included. The primary end-point was the number of patients who achieved major flattening of the keloid (defined as a reduction of more than 80% of the keloid) at the time of the study after a minimum follow-up of 24 months after treatment. RESULTS: Sixty-six patients with a total of 97 keloids were included. Median follow-up time after treatment was 43 months. 'Major' flattening was observed in 69 keloids (71%). Fifty-six of these lesions (81%) were treated with one session and 13 (19%) required a second session after keloid recurrence. Fifteen lesions (15%) were considered 'treatment failure', including 11 keloids in eight patients who were lost to follow-up and four keloids which achieved less than 30% reduction of their surface. Thirteen other keloids were evaluated as displaying significant or moderate improvement. Treatment side-effects were partial amputation of the ear lobe in three patients with a very large keloid of the ear lobe and hypopigmentation in five patients. LIMITATION: Retrospective study. CONCLUSION: The combination of surgical excision and cryosurgery can be considered for the treatment of large ear keloids.

[Complication of radical lymph node dissection following sentinel lymph node biopsy in patients with melanoma]. / Complications du curage ganglionnaire réalisé après exérèse du ganglion sentinelle dans le mélanome.

OBJECTIVE: To assess the rate of complications of radical lymph node dissection following sentinel lymph node biopsy in patients with malignant melanoma. PATIENTS AND METHODS: A single-centre retrospective study was performed between 2001 and 2009. All melanoma patients who first had a sentinel node biopsy followed by a radical lymph node dissection were included. RESULTS: Seventy-four patients had radical lymph node dissection after confirmation of metastasis in a sentinel node. The sites of the lymphadenectomy were axillary in 51% of patients, groin in 43%, neck in 5% and popliteal in 1%. A nodal metastasis was observed in 12 of 74 lymph node dissections. Median follow-up time was 41.5 months (3-135 months). At least one complication was seen in 47 patients (64%). Early complications were: postoperative infection (n=9; 12%), lymphocele (n=22; 30%), chronic seroma (n=9; 12%), postoperative haematoma (n=6; 8%), and wound separation (n=7; 9%). Late complications were: lymphoedema (n=30; 41%), joint complications (n=10; 14%), fibrous scar (n=11; 15%), chronic pain (n=6; 8%) and hyposensitivity (n=6; 8%). Two patients were rehospitalised for a complication. The complication rate was 53% for axillary location and 81% for inguinal location (P=0.02). CONCLUSION: Radical lymph node dissection performed after positive sentinel node biopsy in melanoma patients is associated with significant morbidity.