Maintenance treatment of pemphigus with rituximab in real life: A single-center study of 50 patients.

BACKGROUND: Following the RITUX 3 therapeutic trial, the French national diagnosis and care protocol (NDCP) for the treatment of pemphigus was updated in 2018. The updated protocol recommends initial treatment with rituximab (RTX) followed by maintenance therapy at 12 and 18 months, and potentially at 6 months where there are risk factors for early relapse. We evaluated these recommendations regarding the management of our own patients. PATIENTS AND METHODS: Our single-center retrospective study included all patients with pemphigus diagnosed between 01/2015 and 10/2020 and receiving at least one initial infusion of RTX. We collected the following data: type of pemphigus, severity, levels of anti-desmoglein 1 and 3 antibodies at diagnosis and between 2 and 6 months after initial RTX, presence or absence of maintenance therapy and modalities, time to first relapse and duration of associated systemic corticosteroid therapy ≥5 mg/day. Maintenance treatment modalities were as follows: no maintenance treatment, maintenance "on demand" (MT1) i.e. not performed at the rate imposed by the NDCP, and maintenance "according to NDCP" (MT2). RESULTS: Fifty patients were included (women 54%, median age 58 years, pemphigus vulgaris 68%, moderate to severe 68%). Initial RTX was combined with systemic corticosteroid therapy at 0.5 to 1 mg/kg in 74% of cases. Twenty-seven patients (54%) received no maintenance therapy, 13 were on an MT1 regimen (26%), and 10 were on an MT2 regimen (20%). Median follow-up was 42 months. At the last follow-up, 39 patients (78%) were in complete remission. A total of 25 patients (50%) relapsed: 18/27 (67%) patients without maintenance, 5/13 (38%) with MT1, and 2/10 (20%) with MT2 (p = 0.026). The probability of relapse over time was significantly lower in patients receiving maintenance therapy compared to those who receiving none (p = 0.022). The median time to relapse was 15 months in patients without maintenance, and 30 and 28 in those with maintenance (p = 0.27). The median duration of systemic corticosteroid therapy ≥ 5 mg/day in the no-maintenance group was 10 months, compared to 7 and 9 months respectively in MT1 and MT2 (p = 0.91). CONCLUSION: Our study confirms the value of RTX maintenance therapy in pemphigus in real life.

[Severe immediate hypersensitivity reaction with generalized contact urticaria after cutaneous application of topical permethrin]. / Réaction d'hypersensibilité immédiate sévère avec urticaire de contact généralisée secondaire à l'application cutanée de perméthrine topique.

BACKGROUND: Permethrin is a synthetic pyrethroid used as a chemical insecticide that obtained an MA in the management of human scabies in 2014. We report a case of severe immediate hypersensitivity (IH) reaction with generalized contact urticaria secondary to the cutaneous application of 5% permethrin cream (Topiscab®). OBSERVATION: A 44-year-old woman with no personal history of atopy was treated with oral ivermectin, Topiscab® and levocetirizine for suspected scabies. Eight hours after taking a levocetirizine tablet and five hours after the application of a tube of Topiscab® together with oral ivermectin, she presented generalized urticaria, nausea and diarrhoea, followed by loss of consciousness. Skin prick-tests for ivermectin and levocetirizine were negative. We noticed non-significant erythema with permethrin. The open application test with Topiscab® was strongly positive at 20min with the appearance of an urticaria plaque in the area of application. The open test with sorbic acid was positive at 2h. Accidental exposure to permethrin spray caused dyspnoea and recurrence of urticaria. DISCUSSION: Mild and transient symptoms are regularly described after cutaneous application (burning, paraesthesia or increased itching). Delayed hypersensitivity reactions such as contact dermatitis have been reported in the literature. Exceptional cases of severe IH reactions have been described following occupational exposure to airborne pyrethroid insecticides. No cases of severe IH reaction secondary to application of topical permethrin have been reported.

[Angiomatoid fibrous histiocytoma of the radial pulse groove]. / Histiocytome fibreux angiomatoïde de la gouttière du pouls radial.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a slowly progressing rare soft-tissue tumour of moderate malignant potential. It is most commonly seen in children and young adults. Clinically, the lesion is easily confused with a haematoma or soft-tissue haemangioma, and the radiological aspects are not specific. PATIENTS AND METHODS: A 16-year-old male patient presented with a nodular lesion situated very close to the right radial artery, vein and nerve and which had been developing for several years. Surgical resection was carried out with sparing of vasculonervous and functional structures. Histological examination revealed a tumour of plurinodular architecture, surrounded by a fibrous pseudocapsule consisting of histiocytoid or fusiform cells in short bundles associated with a mononuclear inflammatory reaction of nodular architecture. The tumour cells expressed the following immunomarkers: desmin, smooth muscle actin, CD99, and epithelial membrane antigen. Fusion transcript EWSR1-ATF1 was found. DISCUSSION: In this case, as occurs in the literature, a diagnosis of AFH was not made on clinical examination or imaging. The enlarged excision normally recommended was greatly restricted in our patient due to the complex localization of the lesion, which was in contact with major anatomical structures. The diagnosis was based on histological examination of the surgical excision and identification of the fusion gene. Long-term follow-up is required to detect local recurrence or metastasis. Management is decided in multidisciplinary meetings.