[Pyoderma gangrenosum following hematopoietic stem cell transplantation]. / Pyoderma gangrenosum après allogreffe de cellules souches hématopoïétiques.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare form of neutrophilic dermatosis and is a potential complication in a number of systemic diseases. These include blood diseases, which represent 3.5% of cases, with the main forms being monoclonal gammopathy and acute myeloid leukemia. PATIENTS AND METHODS: Herein we report a case of pyoderma gangrenosum in a female patient who had undergone haematopoietic stem cell allograft six months earlier as part of her treatment for acute T-cell leukemia. DISCUSSION: This condition forms one of the general disorders potentially associated with PG and is a dermatological disorder that can occur in marrow graft patients.

Toxic epidermal necrolysis in a female with metastatic breast cancer treated with vinorelbine.

Vinorelbine is an anticancer agent with high clinical efficacy for the treatment of metastatic breast cancer. Toxic epidermal necrolysis is a rare but serious cutaneous adverse reaction associated with drug therapy. We hereby present a case report of a patient with metastatic breast cancer, treated with vinorelbine, who developed toxic epidermal necrolysis. To the best of our knowledge, ours is the second case report describing this exceptional dermatologic emergency associated with vinorelbine. In June 2014, a 33-year-old female was treated for localized breast cancer. In December 2016, a brain magnetic resonance imaging revealed cerebral and cerebellar recurrence of the breast cancer. Whole brain radiation therapy was administered and treatment with vinorelbine was subsequently initiated. On day 3 of the first cycle of chemotherapy, she presented a general malaise and an itchy rash with conjunctivitis, oral ulcers and diffuse alopecia. The clinical diagnosis was toxic epidermal necrolysis due to vinorelbine. The patient was transferred to the burn unit. Treatment with intravenous steroids, topical steroids and desloratadine was initiated. She subsequently developed Staphylococcus aureus bacteremia and died of multi-organ failure. Toxic epidermal necrolysis is an extremely rare, acute hypersensitivity reaction involving the skin and mucous membranes. Features more suggestive of toxic epidermal necrolysis are acute onset and rapid worsening of painful lesions of the skin and mucous membranes. Specific treatment with active interventions should be practiced in the context of an international and multicentre clinical study in order to give sufficient power for such trials in this rare disease.

La vinorelbine est un agent anticancéreux très efficace dans le cancer du sein métastatique. La nécrolyse épidermique toxique est un effet indésirable médicamenteux cutané rare mais grave. Nous présentons le cas d'une patiente avec un cancer du sein métastatique traité par vinorelbine, qui a développé une nécrolyse épidermique toxique. A notre connaissance, c'est le deuxième cas de la nécrolyse épidermique toxique due à la vinorelbine. En juin 2014, une femme de 33 ans a été traitée pour un cancer mammaire localisé. En décembre 2016, elle a présenté des métastases cérébrales et cérébelleuses. Une radiothérapie encéphalique a été administrée puis un traitement par vinorelbine a été initié. Au troisième jour du premier cycle de chimiothérapie, elle a présenté un malaise général et une éruption cutanée avec démangeaisons accompagnée de conjonctivite, d'ulcères buccaux et d'alopécie diffuse. Le diagnostic était en faveur de la nécrolyse épidermique toxique à la vinorelbine. La patiente a été transférée à l'unité de brûlure et a reçu des stéroïdes intraveineux et locaux et la desloratadine. Elle a ensuite développé une bactériémie à Staphylococcus aureus et est décédée d'une défaillance multiviscérale. La nécrolyse épidermique toxique est une réaction d'hypersensibilité aiguë extrêmement rare touchant la peau et les muqueuses. Elle est évoquée devant l'apparition aiguë et l'aggravation rapide de lésions douloureuses de la peau et des muqueuses. Un traitement spécifique avec des interventions actives doit être recherché dans le cadre d'une étude internationale et multicentrique, afin de donner assez de puissance à de tels essais dans cette maladie rare.

[Dapsone efficacy in lupus miliaris disseminatus faciei: two cases]. / Efficacité de la dapsone dans deux cas de lupus miliaire disséminé de la face.

BACKGROUND: Lupus miliaris disseminatum faciei (LMDF) is a rare, chronic and benign facial dermatosis that is regarded as an enigmatic diagnostic and therapeutic entity with spontaneous regression in 2 to 4 years leaving pock-like scars. CASE REPORT: We present two cases of LMDF: the first concerns a 46-year-old woman who 6 months earlier presented a papular and pustular eruption on her face leaving small pitted scars. The inefficacy of treatment with cyclines, metronidazole and crotamiton as well as the clinical and histological examination results allowed a diagnosis of lupus miliaris disseminatus faciei to be made. The patient was placed on dapsone 100mg per day, which led to a remarkable improvement in the second week, but with depressed scars. The second case concerned an 18-year-old man who for 3 months had been presenting red-brown papules of the face that were resistant to cyclines and to topical retinoids and caused scarring. This clinical aspect, consolidated by the histological result, allowed the diagnosis of LMDF to be made. Administration of dapsone 100mg per day resulted in improvement from the first month, although there were residual cupuliform scars. DISCUSSION: Dapsone appears to be effective in the management of this disease, as illustrated in our two case reports. However, further studies are needed to confirm these results.

[Erysipelas of the face revealing a mucormycosis]. / Érysipèle de la face révélant une mucormycose.

BACKGROUND: Mucormycosis is an opportunistic fungal infection, typically affecting immunocompromised patients. Rhino-orbital location is the most frequent form with cerebral blood vessels invasion and a fatal outcome. CASE REPORT: An immunocompetent 38-year-old woman, with previous history of primary cutaneous mucormycosis, was admitted for a febrile erythemato-oedematous lesion of the face with well-demarcated edge evoking erysipelas. No cutaneous portal of entry was identified. Oral cavity examination found an ulceronecrotic lesion of the hard palate. Diagnosis of mucormycosis was retained after nasal endoscopy and histological findings. The patient was first treated for erysipelas, then by ascending dose of intravenous amphotericin B. A good outcome was observed despite the occurrence of nephrotoxicity which normalized by alkaline hyperhydratation. Healing was obtained after 1month of effective dose leaving cleft palate sequelae. DISCUSSION: Mucormycosis occurs rarely in immunocompetent, even more if it is a second episode. Affecting the face, it may simulate or be revealed by superficial skin infection. Within that, a meticulous otorhinolaryngeal examination is required when the portal of entry is not obvious.