[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.

[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.

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.

[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.

Clinical features and histological findings are potential indicators of activity in lesions of common vitiligo.

BACKGROUND: Unstable vitiligo lesions are usually considered to be contraindications for surgical treatment. Unfortunately, in the majority of common vitiligo cases, neither accurate clinical signs nor routine blood tests are available to determine whether or not the disease is active. OBJECTIVES: To establish a direct correlation between the clinical aspect of vitiligo lesions and their activity. METHODS: This was a prospective observational study that took place over 1 year. For each patient, a standardized evaluation included both a careful daylight and Wood's lamp examination, pictures, biopsies performed at the marginal area and histological and immunohistological studies. A second examination to assess the activity of the lesions correctly was performed 1 year after inclusion in the study. Both the clinical changes and the histological features of the lesions in actively spreading vitiligo were compared with those in stable vitiligo. RESULTS: This study included 50 patients. The lesions were classified as hypomelanotic with poorly defined borders (HPDB, 29 cases) or amelanotic with sharply demarcated borders (ASDB, 21 cases). One year after the biopsy, of the 48 patients still in the study, 20 had lesions that were considered to be stable and 28 had active lesions. Correlations were successfully obtained between clinical aspects, histological findings and vitiligo activity. The HPDB and ASDB lesions were correlated respectively with active and stable status (P < 0·001). CONCLUSIONS: A simple clinical examination including a Wood's lamp examination may allow reliable and efficient evaluation of the stability of vitiligo lesions.

[Kaposi's disease: an unusual side-effect of topical corticosteroids]. / Maladie de Kaposi : un effet secondaire inhabituel des dermocorticoïdes.

BACKGROUND: Kaposi's sarcoma is an angiogenic tumour associated with HHV8 infection. There are several clinical forms, including those related to acquired immunosuppression. We report a case of nodular lesions in Kaposi's disease that appeared during treatment of bullous pemphigoid with topical corticosteroids. PATIENTS AND METHODS: A 74-year-old female patient presented bullous pemphigoid lesions on all four limbs that were treated with clobetasol cream, a topical corticosteroid. After 2 months of treatment, the patient noted the appearance of several asymptomatic purple, erythematous, papulonodular lesions of elastic consistency on her right thigh and both legs. The histological examination revealed Kaposi's disease with positive immunolabelling for HHV8 in the tumour cells. HIV serology was negative. The lesions disappeared 4 months after cessation of corticosteroid therapy and the patient was still in remission one year later. COMMENT: Authentic "Mediterranean" cutaneous Kaposi's disease was apparently revealed in this patient during treatment with extremely potent topical corticosteroids, before regressing completely on discontinuation of the medication. We discuss the various mechanisms that could have contributed to this side-effect of dermal corticosteroids, whose sustained application over a wide area caused HHV8 antigen reactivation.

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.