[Sweet's syndrome: retrospective case series of 47 patients]. / Syndrome de Sweet : étude rétrospective de 47 cas.

PURPOSE: Also called acute febrile neutrophilic dermatosis, Sweet's syndrome is an inflammatory disorder with a prominent cutaneous expression. It is characterized by a variety of manifestations, clinical and histological findings. The objective of this study was to describe their clinical, pathological and therapeutic characteristics. METHODS: We report on a series of 47 patients who presented a Sweet's syndrome, collected in our institution in Tunis between 1997 and 2011. RESULTS: The patient population consisted of 11 men and 36 women. The mean age was 47 years with extreme ranging from 28 to 74 years. An associated disorder was observed in ten patients: inflammatory disease (three cases), inflammatory bowel disease (two cases), tuberculosis (three cases) and diabetes (three cases). One case of pregnancy was observed. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominantly of neutrophils. Fibrinoid necrosis and intramural inflammation were observed in eight cases. CONCLUSION: The skin disorder can precede, follow, or appear concurrent with the diagnosis of an associated disease which requires careful surveillance.

[Tuberculosis of the breast: a rare often unrecognized diagnosis]. / La tuberculose mammaire: un diagnostic rare, souvent méconnu.

UNLABELLED: Tuberculosis of the breast (BT) is a rare extrapulmonary localization for tuberculosis that mainly affects young women during their child-bearing years. OBJECTIVE: We report eight cases of BT and describe its clinical characteristics, outcomes, and differential diagnoses. PATIENTS AND METHODS: This retrospective study collected all cases of BT diagnosed at our hospital's infectious disease department in Tunisia from 2000 through 2009. We assessed the epidemiologic and clinical findings and reviewed the laboratory, histology, and radiology results, treatment data, and outcomes. RESULTS: Eight women (mean age: 52.5 years, range: 31-75) had BT. Clinical features included retracted erythematous lesions in four cases, a fluctuating abscess in two, and a well-defined nodule in two. The biopsy culture was positive in two cases. Histology results showed a tuberculoid granuloma with caseous necrosis in all cases. Antituberculosis drugs were administered for 8 to 12 months. Two patients underwent surgery. Outcome included total regression of BT for all women. CONCLUSION: Clinical features of BT are often misleading and can easily be mistaken for those of breast cancer. Identification of the Koch bacillus or the presence of a tuberculoid granuloma with caseous necrosis on histology facilitates diagnosis. The treatment consists essentially of antituberculosis drugs. Despite its rarity, BT must not be misjudged, especially in countries where tuberculosis is endemic.

[Rosai-Dorfman disease: therapeutic issues in 2 cases]. / La maladie de Rosai - Dorfman : enjeux thérapeutiques à propos de 2 observations.

Rosai-Dorfman disease (RDD) is a benign lymphoproliferative disorder characterized by cervical lymph node enlargement with a consistent risk of airway compression and esthetic damage. Extranodal localizations are also described. There is no therapeutic consensus for pediatric forms of RDD. Through 2 pediatric cases with nodal involvement in 1 patient and a sinonasal and soft tissue localization in the other, we focus on the management problems of both nodal and extranodal RDD.

Angiolymphoid hyperplasia with eosinophilia: a study of 7 cases.

INTRODUCTION: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasoproliferative disease of undetermined origin. It is characterized by the presence of nodular pseudo-tumors corresponding microscopically to a vascular proliferation within an inflammatory infiltrate made up of lymphocytes, macrophages, and eosinophils. The authors describe 7 cases of ALHE. METHODS: The 7 cases were diagnosed over a period of 19 years (1990-2008). Clinical data and histological slides were brought from the departments of dermatology and pathology of the Rabta Hospital. RESULTS: The 7 patients were 4 women and 3 men with an average age of 34.5 years. The cephalic localization was the most frequent. Lesions were solitary or multiple and formed papules or plaques of variable color. The diagnosis was based in all cases on histological findings. DISCUSSION: The main disease in the differential diagnosis of ALHE is Kimura disease, but the 2 entities have several clinical and histological differences. The pathogenesis remains unclear and there is no consensus on the best treatment.

Spindle cell oncocytoma of the adenohypophysis in a woman: a case report and review of the literature.

INTRODUCTION: Spindle cell oncocytoma of the adenohypophysis is a rare tumour recently reported by Roncaroli et al. in 2002. This tumour is considered a grade I tumour by the World Health Organization. CASE PRESENTATION: We describe what is, to the best of our knowledge, the 14th case of its kind in the literature. A 45-year-old African woman presented clinical and radiological findings related to a nonfunctioning pituitary adenoma. The diagnosis was made on the basis of histological and immunohistochemical findings. CONCLUSION: The purpose of this work is to report a rare pituitary tumour and to describe its histological and immunohistochemical features, which were characterized by the expression of thyroid transcription factor 1 antigen by tumour cells. This fact could support the theory of a possible common origin of these tumours in pituicytomas. In fact, thyroid transcription factor 1 is considered to be a specific marker of pituicytes.

Pemphigus vegetans: a clinical, histological, immunopathological and prognostic study.

BACKGROUND: Pemphigus vegetans (P Veg) is a rare clinical form of pemphigus. Studies on P Veg are rare in the literature and none has so far evaluated the prognostic parameters. OBJECTIVE: In this retrospective study of P Veg, we aimed to analyse epidemiological, clinical, immunopathological and therapeutic data. Study of prognostic factors with accuracy of patient survival was also carried out. METHODS: This is a retrospective study (1981-2009) including 17 cases of P Veg. Statistical analysis was performed with chi-square and Fisher tests looking for a possible relationship between clinical data and prognostic factors. Follow-up time and disease-free survival time were estimated using Kaplan-Meier methods. Clinical data were evaluated in univariate analysis looking for a significant association with survival. Equality of survival distribution was studied using log rank test. RESULTS: The hospital prevalence of P Veg was 0.084‰ with a frequency of 9.1% among pemphigus. The mean age at onset was 47.6 years, with a sex-ratio (F/M) about 4.66. Neumann P Veg was the predominant clinical form (11/17). Clinically, the lesions were multifocal (16/17), prevailed on folds and mucous membranes. Under corticosteroids the mean period for healing was 24 ± 9 days. During the follow-up time, three patients died and 11 patients relapsed. Median of overall relapse-free survival was 13 ± 1.7 months. No significant association between clinical data and prognostic factors was found. LIMITATIONS: This study was a retrospective chart analysis and the number of patients was small. CONCLUSION: The P Veg seems to be more frequent in Tunisia with high rate of mortality.

Leukocytoclastic vasculitis and gastric adenocarcinoma.

BACKGROUND: Cutaneous vasculitis has been mainly associated with hematologic cancer. The association of solid neoplasms with cutaneous paraneoplastic vasculitis is rare. We report a case of cutaneous leucocytoclastic vasculitis that revealed a gastric adenocarcinoma. CASE REPORT: A 72-year-old man presented with an acute diffuse polymorphic eruption, with erythematous, vesicular, and necrotic lesions predominating on the lower limbs, which had developed within the past 8 days. The medical history revealed abdominal pain and weight loss over the previous 6 months. A skin biopsy noted typical leucocytoclastic vasculitis. A workup including GI tract investigations revealed an antral adenocarcinoma. Complete excision of the gastric tumor was followed by resolution of the skin lesions. No tumor recurrence or cutaneous vasculitis was noted during 2 years of follow-up. CONCLUSION: To our knowledge, the association of leucocytoclastic vasculitis with gastric cancer has never been reported previously. The failure of vasculitis to respond to conventional therapy should suggest an underlying malignancy.

[Erythematous type scaly papule on the abdomen: chromoblastomycosis due to Fonsecaea pedrosoi]. / Plaque érythémato-squameuse papuleuse et atrophique de l'abdomen: chromomycose à Fonsecaea pedrosoi.

Chromoblastomycosis is a chronic subcutaneous fungal infection caused by dematiaceous saprophytic moulds. We report a case of chromoblastomycosis due to Fonsecaea pedrosoi observed in man from the Baja region of Tunis. He presented since one year an erythemato-squamous atrophic plaque localised at the abdomen area. Clinical remission was obtained after cryotherapy and terbinafine.

[Paraparesis and fever in a Tunisian woman: cryptococcal spondylitis with spinal involvement]. / Paraparésie fébrile chez une Tunisienne: spondylite à cryptocoque avec atteinte médullaire.

Cryptococcus neoformans is a ubiquitous yeast that causes opportunistic infections mainly involving the central nervous system. Cryptococcoma is a rare entity characterized by a solid, tumor-like mass that is usually located in the cerebral hemispheres or cerebellum. Spinal involvement is rare with only 6 cases reported in literature. Bony involvement is also a rare occurrence that has been observed in only 5 to 10% of reported cases of infection by Cryptococcus neofomans. The purpose of this report is to describe a case of paraplegia due to cryptococcal spondylitis with spinal cord involvement in an HIV-seronegative patient with a history of systemic sarcoidosis. Diagnosis was achieved by histological examination of the surgical specimen.

[Gastrointestinal sarcoidosis]. / Sarcoïdose gastro-intestinale.

INTRODUCTION: Sarcoidosis is a granulomatous disorder of unknown cause, characterised by noncaseating granulomas affecting multiple organs. Gastrointestinal tract involvement in sarcoidosis is rare. The stomach, particularly the antrum is the most common extra-hepatic organ to be involved. We report four cases of gastro-intestinal sarcoidosis. METHODS: Retrospective study of a series of four cases. RESULTS: All patients had gastric sarcoidosis. It involved the duodenum, ileum and gall bladder in a patient with a history of an acute pancreatitis probably due to sarcoidosis. This patient presented with obstructive intestinal manifestations, weight loss and exsudative enteropathy. Two patients presented with mild abdominal pain and the last patient was admitted for upper gastrointestinal bleeding. The endoscopy was normal in one case and showed an antral congestion in another case. Gastric ulcers were found in the patient with a history of upper gastro-intestinal bleeding. A pseudo-linitic aspect was noticed in the patient with obstructive manifestations. The duodenum and the ileum were normal. This patient had an antrectomy and was treated with corticosteroids. Surgery evidenced a perforated duodenal ulcer, which was obstructed by the gall bladder. The patient with gastrointestinal bleeding received proton pump inhibitor and corticosteroids. These two patients improved gradually. The two other patients recovered spontaneously. CONCLUSION: The stomach is the most commonly affected organ in gastrointestinal sarcoidosis. Gastric sarcoidosis can mimic a malignant lesion owing to narrowing of the gastric lumen or can be revealed by upper gastrointestinal bleeding. Duodenum, small bowel and colon involvement is uncommon but may be underestimated in the absence of systematic biopsies.

[Profuse subcutaneous nodules in a Tunisian patient: Kaposi sarcoma revealing AIDS]. / Nodules sous cutanés profus chez un Tunisien: maladie de Kaposi révélatrice de SIDA.

The purpose of this report is to describe a case involving Kaposi sarcoma as the inaugural manifestation of HIV infection at the full-blown AIDS stage. The patient was a 59-year-old Tunisian man who presented with profuse subcutaneous nodules and multiple lymph nodes. Treatment was based on antiretroviral therapy in association with radiotherapy.

Extraventricular neurocytoma in a child mimicking oligodendroglioma: a diagnostic pitfall.

Extraventricular neurocytomas are rare neuronal tumours that have been included in the 2007 WHO classification as a variant of central neurocytoma. They arise outside the ventricles, usually within the cerebral hemisphere,s but also in other regions throughout the neuraxis. The morphological overlap of these tumours with oligodendroglioma often poses diagnostic difficulty. Herein, a case of extraventricular neurocytoma in a 4-year-old girl is reported that mimicked histologically oligodendroglioma. The authors describe the clinicopathological features of this rare entity with special emphasis on differential diagnosis.

[Epidemiology of extrapulmonary hydatid cysts: 265 Tunisian cases]. / Epidémiologie des kystes hydatiques extrapulmonaires : 265 cas en Tunisie.

AIM OF STUDY: The authors wanted to analyze the epidemiological features of extrapulmonary hydatid cysts and compare their results with those reported in literature. METHODS: Two hundred and sixty-five cases of extrapulmonary hydatid cysts collected from 1990 to 2007 were retrospectively studied. RESULTS: One hundred and one male and 164 female patients (sex ratio M/F=0.61 mean age 38.7 years) were included. In our series, hydatid cysts involved mainly the kidney (24.1%), the central nervous system (22.6%), the liver (19.6%) and the spleen (11.3%). CONCLUSION: Contrary to published data, our results show that hydatid cysts of the kidney and of the central nervous system are more frequent than hepatic location which ranks 3rd. Such unusual results may be due to a selection bias.

Cutaneous gamma-delta T-cell lymphoma arising in the setting of Behcet's disease.

Cutaneous gamma-delta T-cell lymphoma (CGD-TCL) is an uncommon lymphoma composed of a clonal proliferation of mature activated gamma-delta T-cells expressing a cytotoxic phenotype. Malignant lymphoma is rarely associated with Behcet's disease, as only 12 cases have been reported in the literature, including a case of cutaneous T-cell lymphoma. In this report, the authors present a new case of CGD-TCL emerging in the course of Behcet's disease in a 40-year-old man. Diagnosis of CGD-TCL was established based on the combination of clinical, histological, immunophenotypical and molecular findings. Through a review of the current literature, the authors analyse the unique clinicopathological, molecular and immunohistochemical features of this rare cutaneous lymphoma.

Extragastrointestinal stromal tumor of the urinary wall bladder: case report and review of the literature.

Most mesenchymal tumours of the gastrointestinal tract are now referred to as gastrointestinal stromal tumours (GISTs). These tumours typically express c-kit (CD117) and CD34; 30-50% are (often focally) positive for alpha-smooth muscle actin, and all are negative for desmin and S100 protein. Recently, mutations in exon 11 of the c-kit gene have been identified as a molecular genetic marker for the subset of GISTs. In this report, we describe a mesenchymal tumour removed from the pelvic cavity of a 34-year-old man. The tumour was strongly attached to the external wall of the urinary bladder. The neoplasm grossly resembled a leiomyoma, and was histologically composed of sheets of spindle cells with a dense collagenous background. The mitotic activity was low (less then 1 per 50 high-power fields). Immunohistochemically, tumour cells were negative for alpha-smooth muscle actin and desmin and positive for CD117 and CD34. This case illustrates that tumours which are phenotypically and genotypically similar to GISTs may present in sites other than the tubular gastrointestinal tract.

Hemangiopericytoma in the central nervous system. A study of eight cases.

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.