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.

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.

[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.

[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.

[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.

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.

[Chordoid meningioma. Two cases]. / Le méningiome chordoïde. A propos de deux cas.

Chordoid meningioma is an uncommon histopathological variant of meningioma frequently confused with chordoma. Recurrence is not uncommon. The aim of our study is to present the clinical and pathological characteristics of this entity and a review of the literature. We report two cases of chordoid meningioma occurring in two women aged 47 and 63 years. Both women presented neurological signs without systemic disease. Radiological findings were temporoparietal and temporofrontal lesions with intense enhancement after contrast injection. Complete surgical resection was performed. Histopathologic findings were consistent with chordoid meningioma.

[Hydatid cyst of the posterior fossa]. / Kyste hydatique de la fosse postérieure.

Hydatidosis is an endemic affection in Tunisia. Cerebral echinococcosis is a relatively rare entity accounting for only 1-2% of all hydatid cysts in humans. Extradural hydatid cyst of the posterior fossa is a very uncommon site for the disease. We report the case of a four-year-old child admitted for high intracranial pressure. Brain CT scan showed an extradural posterior fossa cyst without enhancement after contrast medium injection. Operative finding revealed a hydatid cyst. The histological examination of the tissue sample confirmed the diagnosis. The patient was given albendazole post operatively. She feels well six months later.

[Testicular lymphoma: report of 4 cases]. / Les lymphomes testiculaires: a propos de 4 cas.

BACKGROUND: Primary testicular lymphoma is the most common testicular malignancy in the elderly, account for 1% of all non Hodgkin lymphoma (NHL). The aim was to report from new cases of testicular lymphoma CASES: We report the only four cases of testicular lymphoma observed in a period of 15 years; all these cases were primary diffuse large cell B NHL. Disease course is usually aggressive, with widespread organ involvement. Retrospective analyses show a poor prognosis for this presentation.

[Testicular plasmocytoma: a case report]. / Le plasmocytome testiculaire: a propos d'un cas.

BACKGROUND: Extramedullary plasmocytoma is rare, with less than 50 reported cases and only 8 cases of primary testicular plasmocytoma. AIM: Report of a new case CASE: This report concerns a 65-year-old patient presenting an enlargement of the testis. The diagnosis of testicular plasmocytoma was made on histological examination. There was no evidence of bone marrow involvement or systemic signs of myeloma. This case will be studied with a review of the literature.

[Pseudotumoral gastric amyloidosis]. / Amylose gastrique pseudotumorale.

INTRODUCTION: Localized gastric amyloidosis is rare. It should be considered in differentiation of gastric tumors, in which biopsy is the only means to confirm the diagnosis. CASE REPORT: A 56-year old male patient presented dysphagia and vomiting. A plastic linitis was suggested by endoscopic ultrasonography. Total gastrectomy and clearance of perigastric lymph nodes were performed. The postoperative pathological diagnosis determined the lesion to be localized gastric amyloidosis and no malignant tumor was found. No sign of recurrence has been found one year after surgical resection. DISCUSSION: Currently, surgical resection of pathological tissue and circumambient lymph nodes may be a preferable therapeutic strategy for the localized amyloidosis to prevent possible complications.

[Malignant cerebellar ganglioglioma. A case report and review of the literature]. / Gangliogliome malin cérébelleux. A propos d'une observation, avec revue de la littérature.

Ganglioglioma is usually a well differentiated slowly growing mixed neuronal and glial neoplasm corresponding to WHO grade I or II. However, some gangliogliomas are considered to be WHO grade III because they exhibit anaplastic features in their glial component. Finally there are exceptionally rare cases of newly diagnosed gangliogliomas with grade IV changes in the glial component. We report a case of a 25-year-old woman with a family history of neurofibromatosis who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell multiform tumor glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. Histologically, two cell populations were noted: a predominant glial component consisting in a multiform glioblastoma and ganglion cells supporting a diagnosis of ganglioglioma. Immunohistochemical analysis clearly distinguished the two tumor cell populations. Although other cases of grade III gangliogliomas and twelve cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the second case of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histological findings in a multiform glioblastoma and a ganglioglioma. It also documents the aggressive biologic behavior of this complex neoplasm.

[Bone metastasis from a glioblastoma: An unusual course!]. / Métastase osseuse d'un glioblastome: évolution inhabituelle!

Extraneural diffusion of glioblastoma is very unusual. Extracranial metastases generally occur after craniotomy or a long survival time. Metastases usually involve the lungs, pleura, lymph nodes and occasionally the bone. We report herein a case of bone dissemination occurring 18 month after the initial diagnosis and treatment of supratentorial glioblastoma.

[Renal actinomycosis: a case report]. / Actinomycose rénale: a propos d'une observation.

This report describes the case of a 26-year-old patient hospitalized for spontaneous abdominal pain. Palpation during clinical examination demonstrated a mass in the right abdomino-pelvic cavity. Radiological examination visualized the presence of grains suggestive of actinmycosis. The patient responded favorably to antimicrobial therapy. Actinomyces israeli is a gram-positive anaerobic bacterium that can cause tumor-like lesions usually on the face and neck and more rarely on retroperitoneal structures. Because renal involvement can require nephrectomy, diagnosis of renal actinmycosis must be made during pre-operative work-up. Ultrasound-guided needle biopsy of the lesion can achieve diagnosis and avoid nephrectomy since medical treatment using antimicrobial therapy is effective.

[Rhinocerebral mucormycosis: A case report]. / Mucormycose rhinocérébrale: à propos d'un cas.

Mucormycosis is a rare and acute fungal infection which is frequently lethal, usually observed in non-controlled diabetic patients. The infection usually begins in the nose but it can invade the lung, the digestive tract, and the skin. Rhinocerebral mucormycosis accounts for 40 to 49% of mucormycosis cases. We report the case of a 53-year-old diabetic man, with chronic renal failure, presenting with rhinocerebral mucormycosis. Our patient was treated by an association of amphotericin B and surgical debridement. This observation provides on opportunity to recall clinical, histopathological, and therapeutic aspects of rhinocerebral mucormycosis.

[Unusual presentation of visceral leishmaniasis in an immunocompetent patient]. / Mode de révélation inhabituel d'une leishmaniose viscérale chez un sujet immunocompetent.

Visceral leishmaniasis due to Leishmania infantum is endemic in Tunisia. The incidence in adult patients has increased in recent years; but most of these patients are not HIV positive as in other Mediterranean countries where all the cases are associated with HIV. We present the case of a woman with symptoms suggestive of leishmaniasis but whose bone marrow was sterile and whose serological tests for Leishmania spp. were negative. For this patient, the parasite was only detected in a routine duodenal biopsy. There are few reports of visceral leishmaniasis cases diagnosed by duodenal biopsy and almost all patients were HIV positive in that case. Thus it seems interesting to perform a duodenal biopsy in case of a difficult diagnostic even in the absence of gastrointestinal symptoms and/or HIV infection.

[Three cases of cutaneous blastomycosis]. / La blastomycose cutanée: trois cas.

Blastomycosis is a rare mycotic infection in Africa. Isolated cutaneous disease is extremely rare. We report three cases of cutaneous blastomycosis confirmed by istologic and mycologic examination. No visceral involvement was found. Blastomycosis is rare in Tunisia. The most common site of involvement is the lung. The three cases reported presented with skin involvement only, suggesting a cutaneous inoculation of Blastomyces dermatitidis. The diagnostic criteria of cutaneous blastomycosis are discussed.