[Anatomoclinical and immunohistochemical study of hidradenoma papilliferum, a tumor deriving from anogenital mammary-like glands]. / Étude anatomoclinique et immunohistochimique d'une tumeur dérivant des glandes ano-génitales de type mammaire : l'hidradénome papillifère.

AIM: To specify the clinical, histopathological and immunochemical features in a large series of hidradenoma papilliferum patients and to point out similarities with breast disease. PATIENTS AND METHODS: We performed a monocentric retrospective histopathological study. Cases of hidradenoma papilliferum seen at the dermatopathology laboratory of Strasbourg (1977-2017) were included and analyzed with hematoxylin-eosin staining and immunostaining. Diagnosis was confirmed where tubular formations and papillary proliferations were noted and were lined by a luminal layer of columnar cells surrounded by a basal layer of myoepithelial cells. RESULTS: We included 32 cases solely involving women of a mean age of 49 years. Most cases involved the vulva (67 %) or the perianal region (25 %). The lesions ranged in size from 3 to 25mm. They occurred as solitary, non-ulcerated, skin-colored and cyst-like lesions. They were mostly localized in the mid-dermis. We identified several peculiar morphological variants. Three HP had a structure resembling tubular apocrine papillary adenoma, 3 were similar to apocrine cystadenoma, while 9 HP, of which 3 involved epidermal hyperplasia, were connected with the overlying epidermis and had dense stromal infiltrate in plasma cells, closely resembling syringocystadenoma papilliferum. Sixteen tumors had no features of note. Similarities with breast tumors were revealed through columnar cell hyperplasia (6 cases), high mitotic rates (4 cases), and atypical nuclei (3 cases). Anogenital mammary-like glands were found in the immediate vicinity of 10 HP. Myoepithelial cells were immunoreactive for smooth muscle actin and p63, while luminal cells were immunoreactive for CK7, CK8, EMA, and, in a more focused and less consistent manner, for Ber-EP4, CD117, PHLDA1 and androgen receptors. CONCLUSION: Our study highlighted a variety of patterns and cell morphologies, on occasion within a single tumor, identical to breast disease. Histological and immunochemical examination revealed that anogenital mammary-like glands and HP share common features, which militates in favor of a common histogenesis.

[Intravascular lymphoma simulating relapse of breast cancer: An original clinical case]. / Lymphome intravasculaire simulant une récidive de cancer du sein : une présentation clinique originale.

BACKGROUND: Intravascular lymphoma is a rare form of non-Hodgkin's lymphoma with varied but highly evocative clinical presentations, particularly in terms of dermatology. Histological examination of the skin may be sufficient to allow diagnosis. PATIENTS AND METHODS: Herein, we report the case of a 75-year-old woman with a history of infiltrative ductal carcinoma of the left breast who was hospitalised for neurological evaluation following repeated falls. During the course of her hospital stay, the patient's neurological state deteriorated rapidly, and onset of marked laboratory-documented inflammatory syndrome was observed, together with disseminated intravascular coagulation (DIC). At the same time, she developed orange-peel type skin lesions on her left breast followed by a rapidly-spreading livedoid infiltrated plaque on her side. The skin biopsy enabled a diagnosis to be made of intravascular lymphoma based on immunohistochemical demonstration of intravascular tumoral proliferation of lymphoid cells expressing CD45 marker and B-cell marker CD20, as well as Bcl2, MUM 1 and CD5. DISCUSSION: Intravascular B-cell lymphoma is a rare subtype of diffuse large B-cell lymphoma. It carries a poor prognosis and the clinical appearance varies extremely widely. The signs are primarily neurological and dermatological. Because of the varied nature of presentations as well as the rarity of the disease, diagnosis may be delayed, with worsening of the prognosis of the disease, which nevertheless requires rapid and aggressive management. However, the appearance of infiltrated and livedoid lesions on the trunk or limbs is evocative and skin biopsy constitutes a readily accessible diagnostic tool that offers rapid confirmation.

[Suprapubic Nicolau syndrome following subcutaneous injection of glatiramer acetate]. / Dermite livédoïde de Nicolau sus-pubienne après injections sous-cutanées d'acétate de glatiramère.

BACKGROUND: Subcutaneous glatiramer acetate, commercialized under the name of Copaxone®, is licensed for the treatment of relapsing multiple sclerosis. Its major adverse effects are skin reactions at the injection site. Nicolau syndrome is a rare but serious iatrogenic accident. Herein we report a case seen in a setting of change of dosage and administration rate of Copaxone®. PATIENTS AND METHODS: A 64-year-old woman, treated since 2010 with daily sub-cutaneous injections of Copaxone® 20mg/L, reported the appearance of a painful, indurated and erythematous plaque in the suprapubic area following changeover to 40mg/mL injections three times weekly. The suprapubic injections were continued and ugly greyish spots with stellate purpuric borders appeared. Fournier gangrene was ruled out by means of a soft tissue scan. DISCUSSION: We report this latest case of Nicolau syndrome to alert readers to the non-exceptional nature of this complication associated with use of glatiramer acetate, particularly at a dosage of 40mg/L injections three times weekly. In our case, onset of Nicolau syndrome appears to have been favored by continued injection in areas already showing inflammation. Re-injection of the drug in these areas should thus be proscribed.

Secondary pulmonary syphilis: Case report and review of literature.

INTRODUCTION: Syphilis is a sexually transmitted disease that can affect numerous organs in its secondary or tertiary stages. We describe a case of secondary syphilis with pulmonary involvement and we present a literature review. PATIENTS AND METHODS: A 69-year-old male patient was admitted to hospital for dyspnoea and extended papular exanthema with palmoplantar involvement. The serological test for syphilis was positive. Ocular examination showed bilateral papillitis and retinal haemorrhage. Chest radiography revealed an interstitial alveolar infiltrate predominantly in the upper lobes, mild pleural effusion and hilar adenopathy. These infiltrates were slightly hypermetabolic on PET scan suggesting inflammatory or infectious origin. Treatment with intravenous penicillin G was effective on cutaneous, ocular and pulmonary manifestations. DISCUSSION: Lung involvement in secondary syphilis is poorly known and rarely described. We found 27 cases of pulmonary syphilis reported in English and the main European languages since 1967. Mean age at diagnosis was 46 years with clear male predominance (89%). HIV co-infection was declared in 5 cases. Treponema pallidum was found in 6 patients using PCR on bronchoalveolar lavage (BAL) (3 patients) or on a lung biopsy (1 patient), immunohistochemistry (IHC) on BAL (1 patient) and Giemsa staining on a pleural fluid sample (1 patient). Chest X-rays may show unilateral or bilateral infiltrates or nodules with or without pleural effusion or hilar adenopathy. Sub-pleural involvement is frequent and penicillin is the treatment of choice. CONCLUSION: Pulmonary syphilitic involvement should be suspected where pulmonary symptoms or radiological changes occur in secondary syphilis. IHC, special staining or PCR on BAL, pleural fluid or lung tissue are useful for the identification of spirochetes.