[Pigmented Paget's disease of the breast]. / Maladie de Paget mammaire pigmentée.

INTRODUCTION: Pigmented Paget's disease of the breast is an uncommon disease. Histology shows intraepidermal pagetoid cells positive for cytokeratin7. We report a difficult case with an atypical clinic presentation as a pigmented lesion and unusual immunohistochemical results such as absence of expression of cytokeratin7 (CK7). OBSERVATION: A 68-year-old woman presented a heterogeneous pigmented lesion on the left nipple extending to the areola, over a period of six years. Histologic study of biopsy showed intraepidermal proliferation of atypical cells positive for melanocytic markers but negative for CK7. The clinicopathological features were consistent with malignant lentigo. Nevertheless, histological study of the whole lesion after complete surgical excision was in favour of mammary Paget's disease, while CK7 remained negative. DISCUSSION: Pigmented Paget's disease of the breast is infrequent, with only a few cases reported in the literature and is suggestive of malignant melanoma or pigmented metastasis of mammary adenocarcinoma. Immunohistochemistry is necessary, especially using CK7 staining, which is usually positive (sensitivity of almost 100%), except in some rare cases such as ours. In these difficult cases, study of a wider panel of antibodies may be necessary for diagnosis.

[Lethal pulmonary involvement of neutrophilic dermatosis following erythropoietin therapy]. / Extension pulmonaire d'une dermatose neutrophilique rapidement létale dans les suites d'un traitement par érythropoïétine.

BACKGROUND: Neutrophilic disease is characterized by aseptic visceral infiltration by normal polymorphonuclear leukocytes that can occur in any organ. Association with an underlying systemic disease, particularly haematological malignancy or inflammatory bowel disease, is frequent. This may produce a multisystem disorder, but diagnosis is usually based on skin lesions because of their clinical and histological accessibility. Pulmonary manifestations are the most common extracutaneous symptoms but may be misdiagnosed, as in our case report. CASE REPORT: A 77-year-old woman with IgA myeloma presented with an inflammatory bullous plaque of the leg coupled with fever lasting one week. The clinical and histological examinations were evocative of a neutrophilic dermatosis such as Sweet's syndrome. Significant improvement was initially obtained with systemic corticosteroids and colchicine. The course became complicated by necrotic neutrophilic papulopustular lesions of the upper limbs and pulmonary manifestations, with fever and decline in overall condition occurring the day after administration of erythropoietin. A hypothesis of septic aetiology prompted antibiotic and antifungal therapy, which remained ineffective. The patient died the day after the second erythropoietin injection. DISCUSSION: This case involved late identification of the aseptic neutrophilic aetiology of pulmonary manifestations. Several factors favouring their appearance and the fatal outcome may be suggested: the existence of a myeloma, association with myelodysplastic syndrome and the possible iatrogenic action of erythropoietin. To the best of our knowledge, this is the first reported case of extracutaneous neutrophilic infiltrate occurring in a patient treated with this haematopoietic hormone.

[Cutaneous, pulmonary and bone aspergillosis in a patient presumed immunocompetent presenting subacute cutaneous lupus erythematosus]. / Aspergillose cutanée, pulmonaire et osseuse chez un malade ayant un lupus érythémateux cutané subaigu sans déficit immunitaire associé.

INTRODUCTION: We report a case of cutaneous, pulmonary and bone aspergillosis successfully treated after many years of progression in a patient presumed immunocompetent presenting subacute cutaneous lupus erythematosus. CASE-REPORT: A 43-year-old man, treated with thalidomide for subacute cutaneous lupus erythematosus, presented chest pain with haemoptysis and dyspnea. A pulmonary nodule was detected but the microbiological investigation was negative. The histological examination showed granuloma with round structures. No cause was found. Three years later, skin lesions appeared on the patient's face concomitantly with a pulmonary relapse. Histopathological examination of these lesions demonstrated septate hyphae. Aspergillus fumigatus was isolated in skin and lung. Disseminated aspergillosis was then diagnosed as spondylodiscitis developed. Treatment with combined voriconazole and caspofungin produced significant and rapid improvement of lesions. DISCUSSION: While aspergillosis is commonly seen in immunocompetent patients, angiotropic dissemination points to cellular immunodepression. Our patient, however, was not presenting immunodepression. We discuss the possible contributory role of thalidomide in dissemination of aspergillosis given that the literature to date contains only one reported case of cutaneous aspergillosis secondary to A. fumigatus in an immunocompetent patient. We would also point out the specific histopathological pattern of this disseminated aspergillosis with both septate hyphae and round structures. Invasive aspergillosis is highly lethal but the chances of recovery are now greater thanks to new antifungal agents.

[Aortoduodenal fistula revealed by acute fever with heavy and swollen red leg]. / Grosse jambe rouge aiguë fébrile révélant une fistule aorto-duodénale.

BACKGROUND: Aortoduodenal fistulas are rare and severe complications of aortic prostheses. The clinical picture usually includes digestive features and fever, unlike our observation where fistula was revealed by heavy and swollen leg with cutaneous septic abscesses but no digestive signs. PATIENTS AND METHODS: A 59 year-old man who had undergone aortoiliac prosthetic repair of an aortic aneurysm 6 years earlier was hospitalized in a dermatology department for fever beginning three months previously associated with a heavy and swollen leg. Clinical and ultrasound examination revealed vascularised cutaneous nodules on the leg. Abdominal CAT showed left iliac venous compression caused by periprosthetic inflammation and particularly retroperitoneal fluid accumulation, gas bubbles in which suggested aortoduodenal fistula, which was confirmed during surgery. Aspirative puncture of abscesses was positive for E. Coli and Candida Glabatra. DISCUSSION: Aortoduodenal fistula is a rare complication of vascular prostheses. Clinical features include digestive symptoms and fever. However, diagnosis may be difficult and delay surgery. Cutaneous manifestations appear later and are often associated with other symptoms; they are caused by septic emboli or vascular compression. Imaging methods may assist diagnosis, but surgical procedures provide confirmation and form the cornerstone of management.

[Melanoma of the penis: 6 cases]. / Mélanomes du pénis: 6 cas.

BACKGROUND: Melanomas of the penis are rare tumors of poor prognosis. METHODS: Six cases of melanoma of the penis, followed between 1975 and 2002, were retrospectively reviewed. We collected the data on epidemiological, clinical and pathologic factors, treatment and follow-up. RESULTS: The mean age was 44 years. The time to diagnosis was 2 years. Two patients had general predisposing factors for melanoma, and 3 patients local predisposing factors. Two patients had partial penectomy and 4 patients had conservative excision. One patient had local recurrence, and another had metastatic course resulting in death. Five patients out of 6 were alive and disease free at time of the study, with a 24 month follow-up. DISCUSSION: The identified risk factors for the development of penile melanoma are melanosis and pre-existing nevus. Delayed diagnosis explains the usually bad prognosis. Classical surgical treatment used to be radical, but recently, conservative surgery has been proposed. For an early diagnosis, genital melanosis requires surgical excision, when technically feasible, and any atypical lesion of the penis should be submitted to a biopsy.