Bullous Pemphigoid Associated With Dipeptidyl Peptidase-4 Inhibitors: A Case Report and Review of Current Evidence.

Dipeptidyl peptidase-4 inhibitors (DPP-4i), or gliptins, are a widely used glucose-lowering agents. A growing amount of evidence pointed to a possible role of DPP-4i in the induction of bullous pemphigoid (BP), which is an auto-immune skin blistering disease that mainly affects the elderly. In this article we discuss a case of DPP-4i associated BP and we provide an updated review of the current knowledge regarding this emerging entity. Use of DPP-4i, particularly vildagliptin, was found to significantly increase the risk of BP. BP180 would be in the center of the aberrant immune response. DPP-4i induced BP is thought to be associated with male gender, mucosal involvement, and milder inflammatory phenotype especially in Asian population. Generally, patients may not remit fully after DPP-4i withdrawal only and require either topical or systemic glucocorticoid courses.

Late onset of flesh-colored papules of the neck.

Papular elastorrhexis is a rare, acquired disorder of elastic tissue, occurring in adolescent females, characterized by flesh-colored monomorphous papules usually located on the trunk and the proximal portion of the extremities. We report a case in an old woman with atypically isolated localization on the neck.

Atypical spindle cell lipomatous tumor of the tongue: A rare entity arising in an unusual location.

Atypical spindle cell/pleomorphic lipomatous tumor (ASCPT) constitutes an emerging entity of lipomatous tumors. It is a benign tumor. It occurs typically in limbs and limb girdles. The occurrence in oral cavity is unusual. The diagnosis of this neoplasm is challenging. Herein, we report a case of ASCPT arising in the tongue.

Alveolar Adenoma: A Rare Benign Tumour of the Lung with A Challenging Diagnosis.

Alveolar adenoma is a rare lung benign tumour originating from type II pneumocytes. It presents as a well-defined nodule. In some cases, it is difficult to differentiate from lung cancer. Few cases of this tumour have been reported. We describe here a case of alveolar adenoma in a 63-year-old man discovered incidentally on chest X-ray. The lesion was reported as lepidic adenocarcinoma in bronchoscopic biopsy. The patient underwent a thoracoscopic left lower lobectomy. The histopathological and immunohistochemical examinations resulted in a diagnosis of alveolar adenoma. We report this case to describe its morphological and immunohistochemical characteristics and to emphasize its diagnostic difficulties.

[A rare cause of AA amyloidosis: Hereditary epidermolysis bullosa]. / Une cause rare de l'amylose AA : les épidermolyses bulleuses héréditaires.

INTRODUCTION: Recessive dystrophic epidermolysis bullosa is a rare genetic condition characterized by fragile skin and mucous membrane, caused by mutations in the COL7A1 gene. AA amyloidosis is a rare complication of these genodermatosis. OBSERVATIONS: Two patients with recessive dystrophic epidermolysis bullosa, generalized severe in the first case and generalized intermediate in the second case, developed at the age of 38 and 28, respectively, nephrotic syndrome. The diagnosis of secondary renal amyloidosis was confirmed by renal biopsy in the first case and by minor salivary gland biopsy in the second case. Death occurred 2 months after diagnosis in both cases. CONCLUSION: Renal involvement is quite common in AA amyloidosis in patients with recessive dystrophic epidermolysis bullosa. Nephrotic syndrome and rapid decline in renal function renal are characteristic features. The prognosis is poor due to underlying conditions and the lack of an etiological treatment.

Mammary Myofibroblastoma with Unusual Morphological and Immunohistochemical Features.

Mammary myofibroblastoma (MFB) is a rare mesenchymal tumor, derived from mammary stromal fibro/myofibroblasts, which has various morphological features and characteristic immunohistochemical staining. The epithelioid morphologic variant is defined, accordingly, as a proliferation of exclusively or predominantly (>50%) epithelioid cells, variably embedded in a myxoid to fibrous stroma. These histological and cytological features may pose a diagnostic challenge mainly with metaplastic carcinoma and invasive lobular carcinoma of the breast. Thus, immunohistochemical staining by myofibroblastic markers is helpful for confirming diagnosis. Herein, we present a case of MFB in a 43-year-old female. This case report emphasizes the role of immunohistochemistry as gold standard in the diagnosis of MFB. This case is also being presented because of its unusual radiologic findings, its epithelioid histologic variant mimicking malignancy, and its uncommon immunohistochemical phenotype.

Oncocytome de la surrénale : Étude de cas.

RéSUMé: Nous rapportons ici un nouveau cas de cette entité afin de souligner les caractéristiques anatomocliniques utiles à son identification et ceci, en vue de la distinguer des corticosurrénalomes, qui sont de pronostic nettement plus sombre.

[Uterine leiomyoma with massive lymphoid infiltration: case report]. / Léiomyome utérin avec infiltration lymphocytaire massive: une observation.

Uterine leiomyoma with massive lymphoid infiltration is a rare and unusual pathological finding; only 20 cases have been reported in the literature. We report a case of this unusual lesion in a 35-year-old woman who underwent a myomectomy. On gross examination, the tumor was of white color and firm consistency. Histological sections showed interlacing bundles of spindle shaped cells of low density with moderate to severe lymphocytic infiltrate associated to lymphoid follicles and few plasma cells. Immunohistochemically, the diffusely infiltrating lymphoid cells were predominantly of T cell phenotype. The interspersed spindle shaped cells were positive with alpha smooth muscle actin, desmin and h-caldesmon. The cause of this unusual lesion is not clear, but the recognition of its distinct histological features is important to avoid possible confusion with differential diagnosis including malignant lymphoma, inflammatory pseudotumor and pyomyoma.

Mucinous tubular and spindle cell carcinoma of the kidney associated with tuberculosis.

Mucinous tubular and spindle cell carcinomas (MTSCC) are low-grade renal epi-thelial neoplasms with approximately 100 documented cases reported in the literature. We report a case of MTSCC in a 79-year-old patient in association with a renal tuberculosis infection that has never been reported. Further investigations are needed to determine the frequency and true prognosis of these tumors.

[Ewing sarcoma osseous and extraosseous : a clinicopathologic study of 29 cases]. / Sarcome d'Ewing Osseux et Extra Osseux : Etude anatomo-clinique de 29 cas.

BACKGROUND: Ewing's sarcoma (ES) is a rare tumour accounting for 10% of primary malignant bone tumours in children and 3% of all childhood malignancies. ES belongs to a group of small round cell tumours. AIM: In this review, we will describe the main clinicopathological features of this rare tumour and discuss its prognosis. METHODS: We report a retrospective study of 29 cases of ES, of which 4 were extraosseous, diagnosed over a period 11 years (January 1989 - December 1999). Clinicopathological data were described. Hematoxylin-eosin staining and immunohistochemical study were reviewed. RESULTS: 12 patients were male and 17 were female (ratio: 0,8) with a median age of 16 years. 62,5% of tumours were located in flat bone and 33,3% in long bone. The medium size of the tumor was 10,6 cm (range:3-25cm). 27,5% of patients presented with metastatic disease at time of diagnosis. Microscopically, tumour tissue was composed of round, small, blue cells with fine granular chromatin. Tumour cells strongly coexpressed CD99 and vimentin (100%). Systemic treatment consisted of adjuvant chemotherapy (84,2%). Local control was based on and surgery (57,9%) or radiation therapy (36,8%). A good response to chemotherapy was obtained in 37.5%; 13,7% of patients were alive without disease (medium follow up: 169 mois); 34,5% of patients developed metastases (medium follow up : 23 months) and 10,3% developed recurrences (medium follow up :13 months). CONCLUSION: Our study emphasizes two points : the great size of the tumor and the frequent location in flat bone which may explain the poor prognosis of Ewing sarcoma in our series despite the multidisciplinary treatment.

[A rare congenital gingival tumor]. / Une tumeur gingivale congénitale rare.

INTRODUCTION: Congenital epulis (CE) of the newborn also known as congenital granular cell tumor or Neumann tumor is an uncommun benign tumour occuring in the anterior alveolar ridge of the jaws. It may interfer with breathing and feeding. In our study, we discuss the clinicopathologic and evolutive caracteristics and the diagnosis problems of this entity. OBSERVATION: A newborn girl, two months old, presented at birth a nodular mass in the upper alveolar crete of the maxilla. The mass measured 1.5 cm and exhibited a smooth surface. Histologically, it was composed of diffuse sheets and clusters of granular cells with abundant coarsely granular cytoplasm. The evolution was good without recurrence after five months. DISCUSSION: CE is a rare tumor; its etiopathogenesis is still unclear. The diagnosis suspected clinically is confirmed by the histopathological study. The prognosis is exellent without recurrence.

[Histological features of celiac disease in south Tunisia: a study of 114 pediatric cases]. / Aspects histologiques de la maladie coeliaque dans le sud Tunisien: etude de 114 cas pédiatriques.

AIM: To report the histological features of celiac disease in a paediatric population originating from south Tunisia. METHODS: A retrospective study of a series of duodenal biopsies from 114 children with celiac disease diagnosed over a period of 6 years (from January 1999 to December 2004). The diagnosis was confirmed by histological results, serological studies and clinical response to gluten free diet. RESULTS: The average age of patients was of 6.2 years (range 6 months-15 years). Sex ratio was 0.71. Symptoms were dominated by chronic diarrhea (48%), weight loss (50%) and anemia (20.1%). Histological findings showed an intraepithelial lymphocytosis (Marsh type 1) in 12.2% of cases, type 2 was present in 1.7% of cases and type 3 (villous atrophy) in 86% of cases. A treatment with a gluten-free diet was indicated for all patients, only the cases who haven't presented a clinical amelioration (11 cases) have beneficed a control biopsie; a villous atrophy was persistent in 80% of this patients. CONCLUSION: Histological features in duodenal biopsies for the diagnosis and the follow-up of patients with coeliac disease. This allows an appropriate treatment and prevents further complications.