[Fatal outcome of Netherton syndrome due to excessive use of topical corticosteroids in an adult patient]. / Évolution fatale du syndrome de Netherthon due à une utilisation excessive de dermocorticoïdes chez un adulte.

INTRODUCTION: Netherton syndrome (NS) is a rare disease caused by SPINK5 mutations associated with ichthyosis (erythroderma and desquamation), alopecia and atopic manifestations. There are no effective treatments. Topical corticosteroids may be used for a limited period in the event of eczema. Herein we report on a patient with fatal complications related to misuse of topical corticosteroids. PATIENTS AND METHODS: A 38-year-old woman with NS had been using betamethasone for about ten years for severe pruritus. Consumption was estimated at 7.2kg per year. On examination, she had osteoporosis, Cushing's syndrome, corticotropic insufficiency and inframammary, axillary, and intergluteal superinfected intertrigo. During hospitalization for necrotic leg wounds on severe skin atrophy, she sustained a fracture on falling down. The course was marked by the onset of septic shock of unknown etiology, complicated by acute adrenal insufficiency leading to fatal multi-organ failure. DISCUSSION: Many iatrogenic cases related to topical corticosteroids in children have been reported in the literature, including one case of fatal outcome (CMV infection) in an infant. Such iatrogenic cases are rarer in adults and we observed no fatal cases. In NS, the adverse effects of topical corticosteroids are amplified due to the major defect in the skin barrier which enhances the systemic passage of these drugs. In the absence of any effective therapeutic alternative, weaning patients off topical corticosteroids is usually difficult. CONCLUSION: This case illustrates the severity of iatrogenic effects secondary to misuse of topical corticosteroids in NS as well as the need to find effective new treatments for this syndrome.

[Solitary subungual glomangioma]. / Glomangiome sous-unguéal solitaire.

BACKGROUND: Glomangiomas are benign vascular tumours. They are usually multiple, painless and extradigital. Herein we report a case of a solitary subungual glomangioma. PATIENTS AND METHODS: This 65-year-old woman presented with a history of bluish, asymptomatic, subungual lesions located in the lunula of her right thumb. Surgical exploration by a transungual approach showed a large bluish, well-circumscribed tumour, which was completely excised. Histological examination revealed numerous dilated blood vessels surrounded by aggregates of glomus cells, which was consistent with the diagnosis of glomangioma. DISCUSSION: Glomus tumours are benign tumours arising from glomus cells. Histopathologically, based on the predominant tissue type present, glomus tumours are classified as solid glomus tumours, glomangiomas or glomangiomyomas. The classical form usually consists of a painful erythematous nodule with exaggerated sensitivity to cold and pressure. The nails are frequently involved, with two sites of predilection: the matrix and the nail bed. Vascular forms of glomus tumours or glomangiomas have a different clinical presentation and are usually multifocal, bluish, painless and extradigital. Diagnosis is frequently based on histological examination. Our observation raises the question of differential diagnosis with regard to matrix melanocytic tumours (blue nevi or melanomas). CONCLUSION: We report the case of a solitary subungual glomangioma. Histological examination is necessary to rule out a clinically indistinguishable benign or malignant melanocytic tumour.

[Antidesmoglein antibodies in a patient with Hailey-Hailey disease]. / Détection d'anticorps antidesmogléines circulants chez un patient atteint de maladie de Hailey-Hailey.

BACKGROUND: Hailey-Hailey disease (HHD) is a rare hereditary disease in which the genetic defect is characterized by mutation in the ATP2C1 gene coding for a transmembrane calcium pump. It is generally considered a non-immunologic acantholytic dermatosis in which direct and indirect immunofluorescence studies are negative, unlike in autoimmune pemphigus. PATIENTS AND METHODS: We describe a case of HHD associated with antidesmoglein antibodies in a 53-year-old woman. The clinical symptoms and histology were typical of HHD. Antidesmoglein antibody tests were positive on several occasions and a difference was found between the two types of Elisa test performed (positive with the MBL kit, negative with the Euroimmun kit). DISCUSSION: The positive result for desmoglein antibodies could be due to unmasking of antigens by the mechanism of acantholysis. The specificity of the main desmoglein Elisa tests also requires discussion.

[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.