Syndrome De Lyell Et Syndrome De Stevens-Johnson: Étude Rétrospective De 30 Cas.

Stevens-Johnson syndrome and Lyell syndrome are severe bullous drug reactions that can be life-threatening. The aim of this study is to describe the epidemiological, etiological, clinical, therapeutic and evolutionary data of patients hospitalized in our Dermatology Department. This is a retrospective descriptive study over a period of 10 years. All records of patients admitted to the Dermatology Department for these cutaneous adverse drug reactions were included. A total of 30 patients were recorded, with a male predominance. There were 18 cases of Lyell syndrome, 8 cases of Stevens-Johnson syndrome and 4 cases of overlap syndrome. The mean time to onset after drug administration was 7.5 days. The average skin area detached was 48%. Visceral involvement was frequently observed: pulmonary involvement, renal involvement, hepatic cytolysis and hematological involvement. The notion of medication was found in all our patients, with self-medication in 23% of cases. The reason for prescription was dominated by post-surgical anticonvulsant prophylaxis. All our patients received symptomatic treatment, and corticosteroid therapy was administered in only one patient for macrophagic activation syndrome. The mortality rate was of 17%. Skin area involved, presence of renal failure or respiratory distress were the main prognostic factors.

[Recurrent postpartum pyoderma gangrenosum and fatal cardiomyopathy]. / Pyoderma gangrenosum récidivant du post-partum et cardiomyopathie fatale.

BACKGROUND: We report a case of recurrent post-partum pyoderma gangrenosum (PG) complicated by post-partum cardiomyopathy (PPCM). PATIENTS AND METHODS: A 23-year-old woman presented with a previous medical history of aseptic abscess of the left breast in her fourth pregnancy, which developed after surgical drainage of an inflammatory ulceration treated by atraumatic topical care. During her fifth pregnancy, the patient presented a large and painful ulceration in relation to the scar of the Caesarean section, despite the introduction of broad-spectrum antibiotic therapy. Bacteriological samples were negative. Histological examination militated in favor of PG. One week after initiation of corticosteroid therapy, the patient suddenly showed signs of heart failure. Based on trans-thoracic echocardiography PPCM was diagnosed, and the outcome was fatal. DISCUSSION: This observation raises the question of the relationship between PG and pregnancy and describes the association of PG and PPCM. PG occurs rarely during pregnancy and it may be induced by the rise in G-CSF levels found in pregnant women. The association with PPCM seen in our patient could have been due to the development of an anti-angiogenic climate at the end of pregnancy, together with inflammatory myocardial aggression linked to the PG.

[Sudden-onset bullous pemphigoid in a 12-year-old child]. / Pemphigoïde bulleuse d'installation brutale chez un enfant de 12 ans.

Bullous pemphigoid (BP) is an acquired autoimmune disease that mainly affects the elderly. It is very rare in children. We report a sudden polymorphic case of BP in a 12-year-old child.

[Diffuse cutaneous mastocytosis of an infant: A case report]. / Mastocytose cutanée diffuse du nourrisson : à propos d'un cas.

Mastocytosis is a group of diseases related to abnormal accumulation and proliferation of mast cells in one or more organs. They may be associated with an acquired point mutation and the activation of the receptor tyrosine-kinase c-KIT of CFS (mast cell growth factor). The clinical manifestations are varied and secondary to the release of mast cell mediators and/or infiltration of various organs. There are two main types of mastocytosis: pure cutaneous mastocytosis and systemic mastocytosis when more than two organs are involved in mast cell infiltration (bone marrow, gastrointestinal tract, bone, liver and spleen, lymph nodes). Mastocytosis affects children in two thirds of cases, most frequently as an isolated cutaneous form. The most common clinical form in children is urticaria pigmentosa and solitary mastocytoma; bullous diffuse mastocytosis is rare. We report the case of an 8-month-old infant who presented with a diffuse pruritic bullous eruption. The histology and immunohistochemistry results were suggestive of mastocytosis. A serum tryptase test yielded positive results. Laboratory investigations did not identify systemic involvement. The patient was given antihistamine H1 medication and local care. Advice regarding the disease was offered to the parents. The course of the disease was marked by a decrease in the number of blisters and attenuation of the pruritus at the 6-month follow-up. This observation emphasizes the importance of awareness of this rare entity, which should be considered in all cases of bullous dermatosis in children, thereby allowing for early treatment.

[Multiple facial nodules revealing disseminated cryptococcosis in an immunocompetent patient]. / Nodules multiples du visage révélant une cryptococcose disséminée chez un patient immunocompétent.

BACKGROUND: Cryptococcosis is a potentially severe infection that usually occurs in a setting of immunosuppression. Its occurrence outside of this context is rare. We report a case of disseminated cryptococcosis revealed by a spectacular skin disease in an immunocompetent patient. PATIENTS AND METHODS: A 40-year-old male patient had been presenting multiple nodules and tumors on his face for one month in a context of asthenia and intermittent fever. Histological examination of a skin biopsy revealed encapsulated yeasts strongly suggestive of Cryptococcus neoformans. Mycological examination of the skin biopsy and cerebrospinal fluid isolated Cryptococcus gattii. The blood cultures were positive. Brain MRI demonstrated cryptococcal parenchymal involvement. Screening for primary or secondary immunodeficiency was negative. The patient received amphotericin B 1mg/kg/day and fluconazole 600mg/day but died 2months after diagnosis. DISCUSSION: Cryptococcosis is a potentially severe infection caused by C. neoformans. This rare condition occurs most commonly in patients with profound deficiency in terms of cellular immunity. Although rare, the occurrence of cryptococcosis in immunocompetent patients is possible, and in this event the signs are highly polymorphic, which usually makes it very difficult to diagnose. The diagnosis of cryptococcosis is based on the identification by direct examination and after staining with India ink of encapsulated yeasts of the Cryptococcus genus. Culture on Sabouraud medium is essential for identification of the species. Treatment for disseminated cryptococcosis involves amphotericin B, often associated with flucytosine IV. In the event of meningitis infection in non-HIV patients, mortality continues to be around 15%, despite adequate medical treatment. CONCLUSION: Although rare, cryptococcosis can occur in immunocompetent subjects. The prognosis is severe even after treatment.

[Recurrence of cutaneous sarcoidosis during immune reconstitution syndrome in an HIV-infected patient]. / Récidive de sarcoïdose cutanée lors du syndrome de restauration immunitaire chez une patiente infectée par le VIH.

BACKGROUND: Association of sarcoidosis and HIV can occur in the context of immune reconstitution syndrome (IRS) after initiation of antiretroviral therapy (ART). Herein we report a case of cutaneous sarcoidosis in remission in an HIV-infected patient but relapsing during IRS associated with initiation of ART. PATIENTS AND METHODS: A 33-year-old female HIV-infected patient from Cameroon was treated with triple therapy with good efficacy. The patient previously had a small nodular lesion on her left cheek which disappeared spontaneously 2 months before the diagnosis of HIV infection. Three months after initiation of triple ART, the patient consulted again for recurrence of the lesion, which had gradually increased in size. Clinical examination revealed a purplish-red nodular plaque of lupoid appearance under vitropression, located between the inner corner of the eye, the nasal wing and the left cheek. A skin biopsy revealed giant-cell epithelioid dermal granulomas without caseous necrosis. Blood angiotensin-converting enzyme levels were elevated and intradermal reaction to tuberculin was negative. A diagnosis was made of cutaneous sarcoidosis. The patient was treated with chloroquine 200mg/day for 3 months, resulting in total subsidence of the lesions. No recurrence was observed at 1 year. DISCUSSION: Introduction of ART has changed the dermatological aspect of HIV infection. In addition to specific dermatological signs specific to HIV and to immunosuppression, there are the cutaneous adverse effects of antiretroviral drugs and skin disorders indicating reconstituted immunity during IRS. Schematically, three forms of IRS may be distinguished: the paradoxical form, the infectious form, and the inflammatory form. The latter corresponds to the onset or exacerbation of inflammatory conditions or autoimmune diseases after the start of ART. Thirty cases of association between sarcoidosis and HIV have been described, of which two-thirds occurred during IRS. The central role of CD4 in sarcoidosis explains its occurrence in HIV patients during reconstitution of the CD4 count. CONCLUSION: In HIV-infected patients treated with anti-retroviral treatment, certain skin diseases such as sarcoidosis may be related to IRS.

[Extensive ulcerations in children: The difficulty diagnosing pyoderma gangrenosum]. / Ulcérations extensives chez l'enfant : penser au pyoderma gangrenosum.

Pyoderma gangrenosum is an amicrobial neutrophilic dermatosis of unknown cause with a chronic course. We report a case in a 30-month-old child who presented with progressive and painful skin ulcers. Lesions were quickly extensive, refractory to local and systemic antibiotic therapy. Histopathology of the skin biopsies confirmed the diagnosis of ulcerative pyoderma gangrenosum. Only 4% of the cases reported in the literature are in children below the age of 4 years. This neutrophilic dermatosis is associated in half of the cases with a systemic disease. The treatment is based on corticosteroids. Immunosuppressant drugs such as tacrolimus, cyclosporine, and mycophenolate mofetil may be effective for pyoderma gangrenosum refractory to corticosteroids. Currently, anti-TNF is a promising treatment for refractory PG.

Possible patterns of epidermal melanocyte disappearance in nonsegmental vitiligo: a clinicopathological study.

BACKGROUND: The depigmentation of vitiligo results in a progressive and chronic melanocyte loss with rare melanocytes occasionally remaining in the epidermis or the hair follicle reservoirs. Destruction by immune infiltrates in close contact with melanocytes within microvesicles and/or detachment of melanocytes followed by their transepidermal elimination should be regarded as possible mechanisms of chronic loss of pigment cells. OBJECTIVES: To assess the frequency of these two histological findings and to establish a direct correlation with clinical features. METHODS: This was a prospective observational study that took place over 1 year. Each patient received a standardized evaluation that included daylight and Wood's lamp examinations, pictures, biopsies performed on the marginal area, and histological and immunohistological studies. A second examination to assess the activity of the lesions was performed 1 year after inclusion in the study. Clinical changes associated with microvesicles were compared with those associated with detached melanocytes from the basal layer. RESULTS: This study included 50 patients. The histological findings were classified as inflammatory with isolated microvesicles (29 cases), noninflammatory with only detached melanocytes from the basal layer (12 cases) and a combination of coexisting microvesicles and detached melanocytes (six cases). Correlations were obtained between the histological findings and clinical features (aspect and activity of the lesions) and E-cadherin expression. CONCLUSIONS: Our data suggest the existence of two patterns of melanocyte disappearance in nonsegmental vitiligo.