Giant cell arteritis associated with scalp, tongue or lip necrosis: A French multicenter case control study.

BACKGROUND: Scalp, tongue and/or lip necrosis are rare complications of GCA. OBJECTIVES: To describe characteristics and outcome of patients with giant cell arteritis (GCA) -related scalp, tongue and/or lip necrosis. METHODS: A retrospective nationwide multicenter study included 20 GCA patients with scalp, tongue, and/or lip necrosis diagnosed between 1998 and 2021 and 80 GCA control patients matched for age, sex and management period. Logistic regression analyses were conducted to identify baseline characteristics associated with scalp, tongue and/or lip necrosis. RESULTS: Compared to controls, patients with scalp, tongue and/or lip necrosis showed significantly more cranial manifestations (headache, p=0.045; scalp tenderness, p=0.006; jaw claudication, p=0.02). No differences were observed between both groups regarding the occurrence of visual symptoms or large vessel involvement. At diagnosis, GCA patients with necrosis more likely received IV methylprednisolone infusions and higher doses of oral prednisone. There were no differences regarding vascular complications during follow up. Compared to controls, survival was decreased in GCA patients with necrosis (p=0.003). In a multivariable logistic regression model, scalp tenderness [odds ratio (OR) 4.81(95 % CI: 1.57, 14.79), p = 0.006] and cognitive disorder [OR 6.42 (95 % CI: 1.01, 40.60), p=0.048] were identified as factors associated to scalp, tongue, and/or lip necrosis. CONCLUSION: Our results suggest that scalp, tongue, and/or lip necrosis is associated to higher mortality in GCA patients. Scalp tenderness and cognitive disorder were significant factors associated to this very rare complication of GCA.

Anti-BP180 IgG antibody ELISA values correlate with adverse pregnancy outcomes in pemphigoid gestationis.

BACKGROUND: Adverse pregnancy outcomes (APO) occur in 35% of patients with pemphigoid gestationis (PG). No biological predictor of APO has been established yet. OBJECTIVES: To assess a potential relationship between the occurrence of APO and the serum value of anti-BP180 antibodies at the time of PG diagnosis. METHODS: Multicentre retrospective study conducted from January 2009 to December 2019 in 35 secondary and tertiary care centres. INCLUSION CRITERIA: (i) diagnosis of PG according to clinical, histological and immunological criteria, (ii) ELISA measurement of anti-BP180 IgG antibodies determined at the time of PG diagnosis with the same commercial kit and (iii) obstetrical data available. RESULTS: Of the 95 patients with PG included, 42 had one or more APO, which mainly corresponded to preterm birth (n = 26), intrauterine growth restriction (IUGR) (n = 18) and small weight for gestational age at birth (n = 16). From a ROC curve, we identified a threshold of 150 IU ELISA value as the most discriminating to differentiate between patients with or without IUGR, with 78% sensitivity, 55% specificity, 30% positive and 91% negative predictive value. The threshold >150 IU was confirmed using a cross-validation based on bootstrap resampling, which showed that the median threshold was 159 IU. Upon adjusting for oral corticosteroid intake and main clinical predictors of APO, an ELISA value of >150 IU was associated with the occurrence of IUGR (OR = 5.11; 95% CI: 1.48-22.30; p = 0.016) but not with any other APO. The combination of blisters and ELISA values higher than 150 IU led to a 2.4-fold higher risk of all-cause APO (OR: 10.90; 95% CI: 2.33-82.3) relative to patients with blisters but lower values of anti-BP180 antibodies (OR of 4.54; 95% CI 0.92-34.2). CONCLUSION: These findings suggest that anti-BP180 antibody ELISA value in combination with clinical markers is helpful in managing the risk of APO, in particular IUGR, in patients with PG.

Duration of efficacy increases with the repetition of botulinum toxin A injections in primary palmar hyperhidrosis: a study of 28 patients.

BACKGROUND: Intradermal injections of botulinum toxin are effective but transitory in primary palmar hyperhidrosis. These injections are repeated when the symptoms recur. We do not know how the duration of efficacy changes when injections are repeated. OBJECTIVE: In this retrospective study, we aimed to investigate the change in the duration of efficacy of botulinum toxin A (Dysport, Ipsen, Boulogne-Billancourt, France) with the repetition of injections in patients with primary palmar hyperhidrosis. METHODS: From May 2001 to April 2012, 28 patients were treated with a dose of 250 U of botulinum toxin A per palm. We compared the duration of efficacy of the first and last toxin injections. RESULTS: The median duration of efficacy was 7 months for the first injection and 9.5 months for the last, the difference being statistically significant (P = .0002). LIMITATIONS: Study limitations include a relatively small number of patients treated at a single center and evaluated retrospectively. CONCLUSION: To our knowledge, this study is the first to report a significant increase in the duration of efficacy of botulinum toxin A injections with the repetition of injections in patients with primary palmar hyperhidrosis. The reasons for this effect may be linked to the mechanism of action of botulinum toxin, and may improve our understanding of its pharmacologic effects.

Duration of efficacy increases with the repetition of botulinum toxin A injections in primary axillary hyperhidrosis: a study in 83 patients.

BACKGROUND: Intradermal injections of botulinum toxin are effective but transitory in primary axillary hyperhidrosis. These injections are repeated when the symptoms recur. The reported duration of efficacy is variable, from 2 to 24 months, but it is unknown how the duration of efficacy changes when injections are repeated. OBJECTIVE: In this retrospective study, we aimed to evaluate changes in the duration of efficacy of botulinum toxin injections (Dysport, Ipsen, Boulogne-Billancourt, France) with the repetition of injections in patients with axillary hyperhidrosis. METHODS: From May 2001 to April 2012 inclusive, 83 patients were treated with a dose of 125 U per underarm. We compared the duration of effect of the first and last toxin injections. RESULTS: The median duration of efficacy for the first injection was 5.5 months, whereas that for the last injection was 8.5 months. The difference between these 2 durations is statistically significant (P = .0002). LIMITATIONS: Although retrospective and based on the declarative, this work is the first to our knowledge to highlight this benefit of treatment and to evaluate botulinum toxin over such a long period (11 years). CONCLUSION: There appears to be an increase in the duration of efficacy of botulinum toxin A injections with the repetition of injections in patients with primary axillary hyperhidrosis. The reasons for this effect may be linked to the mechanism of action of botulinum toxin, and may improve our understanding of its pharmacologic effects.

Rituximab for primary cutaneous diffuse large B-cell lymphoma-leg type.

Primary cutaneous diffuse large B-cell lymphoma leg type (PCDLBCL-LT) is a rare type of lymphoma, of poor prognosis, which affects elderly people. Rituximab is an anti-CD20 monoclonal antibody and has demonstrated its efficiency in the treatment of nodal lymphomas. Rituximab with polychemotherapy has been reported in PCDLBCL-LT with a good response but many adverse effects. We evaluated the risk-benefit ratio of treatment with single-agent rituximab in a retrospective study on 8 patients with PCDLBCL-LT treated with rituximab. The main evaluation clinical endpoint was the rate of objective responses to the treatment. The secondary endpoints were the adverse effects, disease-free survival and overall survival. After 4 courses of single-agent rituximab, 75% of objective responses were achieved. 100% of patients relapsed (median disease-free survival: 5.25 months, median follow-up: 17.7 months). The tolerance was excellent with one adverse event (Grade I). Rituximab monotherapy induces a rate of objective responses which is less than rituximab with polychemotherapy, with no lasting therapeutic response. The tolerance of rituximab monotherapy is higher than rituximab with polychemotherapy. The risk-benefit ratio is a bit lower but rituximab is well tolerated and may be useful for short term palliative treatment.