[Sclerosing polycystic adenosis of the parotid gland : surgical treatment of an extensive lesion of deep tissue without superficial damage]. / Adénose sclérosante polykystique de la parotide : traitement chirurgical d'une lésion extensive du lobe profond sans atteinte superficielle.

Sclerosing polycystic adenosis (SPA) is a rare benign epithelial lesion of the salivary glands, of unknown etiology, mainly affecting the parotid gland. We report the first clinical case of SPA involving the deep parotid gland with extension in the parapharyngeal space and the masticatory region. It has been resected by an external parotidectomy approach exclusively, despite the median extension of the lesion. The objective of this article is to complete the small series of cases described in the literature, and to update the knowledge of this rare disease.

L'adénose sclérosante polykystique (SPA) est une lésion épithéliale bénigne rare des glandes salivaires, d'étiologie inconnue, atteignant principalement la glande parotide. Nous rapportons le premier cas clinique de SPA dont l'origine est le lobe profond de la parotide et qui envahit la région masticatrice et l'espace parapharyngé. Elle a été réséquée par une voie d'abord externe de parotidectomie, exclusivement, malgré l'extension médiane de la lésion. L'objectif de cet article est de compléter la petite série de cas décrits dans la littérature, et d'actualiser les connaissances de cette pathologie rare.

[Transitory apocrine hidrocystomatosis of the scrotum]. / Hidrocystomatose apocrine transitoire du scrotum.

BACKGROUND: Apocrine hidrocystomas are benign cystic tumors that develop from apocrine gland proliferation. In most cases, they are translucent solitary lesions of the face, generally found in the periorbital region, on the scalp or on the neck. More rarely, apocrine hidrocystomas may be multiple and appear on the ears, trunk, shoulders and genital area. They generally appear in adulthood, with only a few pediatric cases being reported, of which three in the genital area, with a solitary case of multiple hidrocystomas of the scrotum, although no cases of spontaneous involution of hidrocystomas have previously been reported. PATIENTS AND METHODS: Two boys aged 4 and 6 months were seen in consultation for small sub-millimeter size, subcutaneous, black lesions on the scrotum that appeared in the weeks following birth. Histological examination of these lesions resulted in a diagnosis of apocrine hidrocystoma. The children were seen again a few weeks later and the skin lesions had totally disappeared. We report two cases of multiple apocrine hidrocystomas on the scrotum with spontaneous involution diagnosed in a 4- and a 6-month-old boy. DISCUSSION: Apocrine hidrocystomas are rare benign adnexal tumors that develop from apocrine sweat glands. They are considered as cystic proliferations of the apocrine glands rather than simple retention cysts. The main differential diagnosis of the rare cases of multiple apocrine hidrocystomas are eccrine hidrocystomas. The treatment of such lesions is based on surgical excision if they are isolated, daily application of topical atropine 1%, or CO2 laser for multiple apocrine hidrocystomas.

[A case of Sweet syndrome in a patient receiving erlotinib for lung adenocarcinoma]. / Syndrome de Sweet chez une patiente traitée par erlotinib pour un adénocarcinome bronchique.

INTRODUCTION: Sweet syndrome is a neutrophilic dermatosis and is often idiopathic, although its onset may be drug-induced or paraneoplastic. The purpose of this case report is to describe the very first occurrence of Sweet syndrome following erlotinib intake in a patient diagnosed with lung adenocarcinoma. PATIENTS AND METHODS: We observed Sweet syndrome, as assessed by clinical, laboratory and histological examination, in a middle-aged female patient presenting lung adenocarcinoma diagnosed three years prior to her cutaneous symptoms. DISCUSSION: Given the extremely long time between the diagnosis of lung cancer and the onset of Sweet syndrome, as well as the occurrence of skin lesions during administration of the medication and their subsidence after drug withdrawal, we suggest a possible link between this particular EGFR tyrosine kinase inhibitor and the patient's neutrophilic dermatological signs. To our knowledge this association has not previously been described in the medical literature.

[Annular lichenoid dermatitis of youth: A case report and literature review]. / Dermatite lichénoïde annulaire du sujet jeune : un cas et revue de la littérature.

BACKGROUND: Annular lichenoid dermatitis of youth (ALDY) is a rare form of dermatitis mainly affecting children and young people. All cases reported show a consistent clinical and histological picture. This is the first case described in the French literature. PATIENTS AND METHODS: A 5-year-old girl presented an annular isolated patch of the lower abdomen with an erythematosquamous border and central hypopigmentation for one year. Topical corticosteroids and pimecrolimus proved effective but relapse occurred after treatment withdrawal. DISCUSSION: Over sixty cases of ALDY are described in the English-language medical literature. The main differential diagnosis is childhood mycosis fungoides, particularly the hypopigmented variant. Biopsy is necessary for diagnosis since it can reveal typical histological features. Histopathology in all cases shows lichenoid reaction with CD4+ and CD8+ polyclonal lymphocytes. It is limited to the tips of rete ridges and associated with apoptosis of keratinocytes resulting in quadrangular-shaped rete ridges. Our case does not demonstrate either epidermotropism or atypical lymphocytes. CONCLUSION: Annular lichenoid dermatitis of youth (ALDY) is a poorly known distinctive entity within the lichenoid dermatitis family. Clinical-histological correlation is essential to diagnosis. The etiology is still unknown and the course is mostly chronic.

Multiple myeloma presenting as hepatic nodular lesion.

The diffuse infiltration by plasma cells in the liver is not uncommon in multiple myeloma (MM). However, a MM with hepatic mass is very unusual. We report a case of a 75-year-old male with hepatomegaly and a lesion occupying a voluminous space in the liver. A lambda light chain multiple myeloma was found in the check-up of this hepatic mass. We also provide a literature review.

Chronic diarrhea among adults in Kigali, Rwanda: association with bacterial enteropathogens, rectocolonic inflammation, and human immunodeficiency virus infection.

One hundred patients with chronic diarrhea were seen in the Department of Internal Medicine at the Centre Hospitalier de Kigali, Rwanda; stool and/or rectal swab culture was performed for these patients, and they underwent rectoscopy and serological testing for human immunodeficiency virus type 1 (HIV-1). Enteropathogenic bacteria were isolated from 39 (39%) of the patients: Shigella species (22 of 100 patients tested), non-typhi Salmonella (11/100), Aeromonas species (5/60), and Campylobacter species (4/60). Rectocolitis was seen in 70 (70%) of the patients. HIV-1 antibodies were detected in 82 (94%) of 87 patients tested. Cytomegalovirus was not found in rectal biopsy specimens from 29 patients. Entamoeba histolytica was detected in two of 31 rectal smears. Idiopathic ulcerative colitis was diagnosed for two HIV-1-seropositive patients. One or more AIDS-defining diseases were found in 32 (32%) of the patients, and 72 (72%) fulfilled the World Health Organization's clinical case definition criteria for AIDS. Chronic diarrhea, as seen in a hospital setting in a region highly endemic for HIV-1 infection, is strongly associated with HIV-1 infection, with rectocolonic inflammation, and with infection due to enteropathogenic bacteria.

Hepatitis C virus infection in African patients with liver cirrhosis or primary hepatocellular carcinoma.

We have studied the prevalence of hepatitis C virus (HCV) infection in Rwandan patients with histologically proven liver cirrhosis (LC) or primary hepatocellular carcinoma (HCC). Anti-HCV antibodies were determined by using a second-generation test, with a line immunoassay for structural and non-structural antigens as confirmation. Seventy-nine patients with LC, 26 with HCC, and 54 voluntary blood donors as controls were evaluated. Anti-HCV antibodies were more prevalent in LC patients (48%) and in HCC patients (38%) than in the controls (17%; difference, p = 0.0001 and p = 0.03, respectively). Eighty-four per cent of LC patients and 54% of HCC patients were HBsAg-negative. The prevalence of anti-HCV antibodies was significantly higher for LC and HCC patients who had been in contact with HBV but who had no persistent HBV infection (p < 0.05). We conclude that HCV infection is common in Rwanda and is linked to LC and HCC.

Pattern of malignant lymphomas in Rwanda.

The pattern of 115 cases of malignant lymphomas diagnosed in the years 1979-1987 is presented using the updated Kiel's classification for non Hodgkin's lymphomas and after application of monoclonal antibodies L 26 and UCHL1 for the identification of B and T lymphocytes. Ninety-six cases (83.5%) were non Hodgkin's lymphomas and 19 cases (16.5%) Hodgkin's disease. Among non Hodgkin's lymphomas, B-cell lymphomas were predominant with a total number of 91 cases (94.8%). An analysis of B-cell lymphomas showed the following relevant features: a high frequency of lymphoplasmacytic/oid immunocytoma, a low frequency of Burkitt's lymphoma with a predominance of abdominal localisations, a high frequency of extranodal lymphomas (47.3%) and high grade lymphomas (48.4%); a 65% increase in high grade and extranodal lymphomas and a 76% increase in Burkitt's lymphomas and immunoblastic lymphomas in the period 1984-87 compared to the period 1979-83. For T-cell lymphomas, relevant features were the presence of one case of pleomorphic T-large cell lymphoma or adult T-cell leukemia-lymphoma and the fact that four out of five cases were large cell (high grade) lymphomas. More than 50% (57.9%) of Hodgkin's disease were of mixed cellularity type, only 15.8% being of nodular sclerosing type. The disease seemed to affect adolescents and young adults, more than 80% (83.3%) being aged 15-40 years. These features are compared to those observed by other authors and possible pathogenetic mechanisms are discussed.

[Kaposi's sarcoma in Rwanda: clinico-pathological and epidemiological aspects]. / Le sarcome de Kaposi au Rwanda: aspects clinico-pathologiques et épidémiologiques.

The clinico-pathological and epidemiological features of 119 cases of Kaposi's sarcoma diagnosed during the years 1979-1986 in the main pathology department of Rwanda are presented. Skin involvement (89%) was predominant with almost 70% of cutaneous lesions localised on the lower limbs; 11% of cases presented with extracutaneous localisations, 77% (10 cases out of 13) in the lymph nodes. Incidence rises progressively with age, and males are more affected than females with a sex ratio (m:f) of 6.4:1. The highest frequencies were observed in the western prefectures which border the province of Kivu in eastern Zaire. Histologically, three types were encountered: a mixed type (84%), a spindle cell-predominant type (12.6%) and an anaplastic type (3.4%). The factor VIII-related antigen was present in all 40 cases tested by the PAP method. Antihuman immunodeficiency virus antibodies were looked for in 18 cases: 10 cases with localised Kaposi's sarcoma, all of whom were seronegative, and 8 cases with aggressive generalised Kaposi's sarcoma, all of whom were seropositive. The results are compared with those of other authors, and the histogenesis and pathogenesis, particularly the relationship with the acquired immunodeficiency syndrome, are discussed.

[Cancer in Rwanda. Study of relative frequency]. / Le cancer au Rwanda. Etude de fréquence relative.

The relative frequency by age, sex and site of the malignant tumours diagnosed during the years 1982-84 in the main pathology department of Rwanda (serving approximately 80% of the population) is presented. Excluding skin, the numerically most important cancers in males were Kaposi's sarcoma (11.5%), stomach cancer (9.9%), non-Hodgkin's lymphoma (8.1%) and primary carcinoma of liver (6.6%), and in females cancers of the uterine cervix (21.4%), breast (16.8%) and stomach (6.5%). The possible sources of bias are discussed, and the results are compared with those from previous studies in this part of central Africa.

Sinus histiocytosis with massive lymphadenopathy in Rwanda: report of eight cases with immunohistochemical and ultrastructural studies.

The clinico-pathological, immunohistochemical and ultrastructural features of eight cases of sinus histiocytosis with massive lymphadenopathy (SHML) recorded in Rwanda from 1975 to 1980 are reported. The main histopathological features were a massive enlargement of the lymph node sinuses which were filled with large histiocytes, a great number of which phagocytosed blood cells, especially lymphocytes, and an important proliferation of plasma cells in the medullary cords. The pathogenesis of this syndrome is discussed, particularly the role of immunological disorders challenged by a chronic, but non-specific, infectious state. The ultrastructural study revealed no micro-organisms and no Langerhans' granules. Immunohistochemical staining revealed two interesting features: the presence of immunoglobulins in the cytoplasm of the histiocytes indicating the presence of an immune phagocytosis and the absence of lysozyme and alpha 1-antichymotrypsin (alpha 1-AC) from the histiocytes. The significance of the latter phenomenon is still unknown.

[Familial erythrophagocytic lymphohistiocytosis. Report of five cases with cytophotometric study of nuclear DNA (author's transl)]. / La lymphohistiocytose familiale érythrophagocytaire. A propos de cinq observations avec étude cytophotométrique de l'A.D.N. nucléaire.

The authors report five cases of familial erythrophagocytic lymphohistiocytosis (F.E.L.H.). Two patients are brothers. One case is associated with a striking extramedullary hematopoiesis. A cytophotometric study of nuclear DNA in the histiocytes was performed in all cases. The histiocytes are diploid and there is no aneuploidy. These findings allow the authors to exclude the hypothesis of the tumoral nature of F.E.L.H. The data collected from the literature about the familial incidence of F.E.L.H. militate for an autosomal recessive transmission. The extramedullary hematopoiesis, which has not yet been reported in association with F.E.L.H., is probably due to the intense lymphohistiocytic infiltration of the bone marrow.

Wernicke's encephalopathy in patients with tumors of the lymphoid-hemopoietic systems.

In three treated patients with a generalized invasion by a tumor of the lymphoid-hemopoietic systems, the neuropathologic findings were consistent with Wernicke's encephalopathy. The clinical picture was atypical, but thiamine deficiency by severe malabsorption was the probable cause of this neurologic complication. It is postulated that the chronic form of Wernicke's encephalopathy must occur more frequently than previously shown in treated and long-standing cases of such kinds of tumors.