[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.

[Pyoderma gangrenosum associated with anti-proteinase 3 antineutrophil cytoplasmic antibodies (PR3-ANCA) induced by propylthiouracil]. / Pyoderma gangrenosum avec anticorps anti-cytoplasme des neutrophiles de type anti-protéinase 3 (PR3-ANCA) induits par le propylthiouracile.

BACKGROUND: Synthetic antithyroid drugs are often used in the treatment of hyperthyroidism, regardless of aetiology. They may cause various side effects, including the development of anti-neutrophil cytoplasmic antibodies (ANCA), ANCA-associated vasculitis, and neutrophilic dermatoses. Propylthiouracil (PTU) is the antithyroid drug most frequently implicated in ANCA-associated diseases specifically involving anti-myeloperoxidase ANCA (MPO-ANCA). To our knowledge, there are no clinical reports describing the association of pyoderma gangrenosum (PG) and anti-proteinase3-ANCA (PR3-ANCA) induced by PTU, with ANCA levels decreasing after antithyroid drug withdrawal. PATIENTS AND METHODS: A 68-year-old woman was treated with propylthiouracil (PTU) for toxic multinodular goitre. She presented necrotic ulceration of the lower abdomen. The patient's history, physical examination, and bacteriological and histological samples led to a diagnosis of pyoderma gangrenosum. This pyoderma involved ANCA with antigenic specificity for proteinase 3. Withdrawal of PTU and a short course of corticosteroids and cyclosporine resulted in rapid and complete resolution of the pyoderma gangrenosum as well as a decrease in ANCA. No relapse was observed one year after cessation of treatment. DISCUSSION: We report a case of PG associated with PR3-ANCA induced by PTU, without any demonstrable vasculitis.

Loss of tumorigenicity and increased immunogenicity induced by interleukin-10 gene transfer in B16 melanoma cells.

Because interleukin-10 (IL-10) has potent immunosuppressive and anti-inflammatory properties and is produced by some cancers, we hypothesized that its production might play a role in carcinogenesis by inhibiting adequate antitumoral immune responses. To test this hypothesis, retroviral vectors containing the IL-10 cDNA were generated and used to infect B16F1 melanoma cells that were injected subcutaneously in syngeneic mice. Surprisingly, IL-10 gene transfer resulted in a loss of tumorigenicity that was proportional to the amount of IL-10 secreted. Histological analysis showed massive area of necrosis of these tumor cells, with infiltration of polymorphic inflammatory cells. Parental cells simultaneously implanted had decreased tumorigenicity only when mixed with IL10-producing cells, but not when injected contralaterally, suggesting that their eradication is mediated mostly by a local phenomenon. Host T lymphocytes and natural killer (NK) cells were involved in this eradication because IL-10-producing cells grew in nude mice and in CD8+ or NK-depleted mice. Finally, mice injected with IL-10-secreting cells developed an antitumoral systemic immune response able to protect them against a subsequent challenge with parental cells. These results demonstrate that, in some settings, IL10 may have in vivo immunostimulating and proinflammatory properties that need to be considered in its therapeutic development.

Curative potential of herpes simplex virus thymidine kinase gene transfer in rats with 9L gliosarcoma.

The transfer of the gene coding for the thymidine kinase of the herpes simplex virus (HSV-tk), followed by ganciclovir (GCV) administration, has been described for the treatment of several types of cancer, especially brain tumors. We further studied the efficacy of this approach by using the 9L rat gliosarcoma model, and cells producing 5 x 10(3), 9 x 10(4), 3 x 10(5) HSV-tk retroviral particles per milliliter. Their stereotactic injection in 9L brain tumors and GCV treatment did not result in any increase of survival. To study a model of optimal in vivo transduction, we examined the survival of rats with tumors growing from 9L cells that had been previously transduced in vitro with the HSV-tk vectors (9LTk cells). We observed that GCV administration cured 26% (n = 42) of the animals with 9LTk brain tumors, with most of the relapsing tumors remaining HSV-tk positive. The increase of either the dose or the duration of GCV treatment did not improve the survival rate. But the cerebral localization of the tumor played an important role, because this survival rate reached 67% (n = 12) when similar tumors were growing subcutaneously. No or only marginal antitumoral responses were induced by the presence of a selectable marker gene in the HSV-tk vectors. These results demonstrate that in vitro HSV-tk gene transfer in 9L tumor cells, but not in vivo gene transfer, followed by GCV treatment, is able to cure rats at a rate that is higher for subcutaneous than for intracerebral tumors.

Leukaemia and lymphoma of the appendix presenting as acute appendicitis or acute abdomen. Four case reports with a review of the literature.

Leukaemic and lymphomatous infiltration of the appendix is rare and even rarer is acute appendicitis as the initial manifestation. From our routine biopsy material we collected four cases of haematological malignancies presenting as acute appendicitis or acute abdomen, caused or accompanied by tumoral infiltration of the appendix. Appendicitis was the initial manifestation that allowed diagnosis of the underlying disease. The clinical histories and histological examinations of the appendices and of one autopsy are described. We report the first detailed description of acute myeloid leukaemia involving the appendix, and three cases of lymphomatous infiltration of the appendix presenting with appendicitis, and give an overview of the literature. In these days of budgetary cuts in national health services, where one may be tempted not to have seemingly commonplace cases of appendicitis histologically verified, our cases emphasize that careful histopathological examination of all appendectomy specimens should be mandatory. Despite the fact that leukaemia and lymphoma of the appendix are rare, our cases illustrate that these must be included in the differential diagnosis of acute appendicitis and that physicians and surgeons have to be aware of these conditions.

Relationship between DNA ploidy level, nuclear size, and survival in large cell lymphoma.

Intermediate and high grade subtypes of non-Hodgkin's large cell (LCL) and immunoblastic lymphomas exhibit considerable variability, and histologic morphology alone may not adequately characterize those features important for prognosis. The relationship between nuclear morphology and survival was assessed in a series of 50 cases of large cell lymphomas in which ploidy, proliferation, and nuclear area (NA) were measured. Ploidy was calculated by both DNA index (DI) and DNA histogram type (DHT). Proliferation was calculated from the proportion of S phase (SPF) cells present in the DHT. These four parameters were measured using image cytometry of Feulgen-stained nuclei from fine-needle aspirations. To characterize the relationship with survival, these parameters were associated with the clinical follow-up of the patients. The results show that of the 50 LCL cases, only 5 were clearly aneuploid, whereas the remaining 45 were either diploid (29 cases), tetraploid/hypotetraploid (13 cases), or weakly aneuploid (hyperdiploid, 3 cases). Of the 34 patients who died from their disease, both smaller NA and DI correlated with longer survival in an equivalent fashion; neither conferred greater sensitivity when combined with the other. The SPF did not correlate with survival. In LCL, aneuploidy seems to be a relatively uncommon event, but when present ploidy measurement appears useful to define prognosis.

Development of a calcifying fibrous pseudotumour within a lesion of Castleman disease, hyaline-vascular subtype.

A nine year old boy with localised Castleman disease of the hyaline-vascular subtype developed a calcifying fibrous pseudotumour. This pathological association does not appear to have been described before. In this case, the development of this very unusual soft tissue tumour-like process was thought to be related to a previous fine needle aspiration biopsy, which was performed because of lymphadenopathy localised to the right inguinal area. This case provides further evidence of the reactive nature of calcifying fibrous pseudotumour and also broadens the pathological spectrum of the stromal cell proliferation that occasionally supervenes within lesions of Castleman disease, hyaline-vascular type.

Subcutaneous lymphoid hyperplasia arising at site of ethnic scarifications and mimicking subcutaneous panniculitis-like T-cell lymphoma.

The case of a 40-year-old black man, who developed a very unusual tumour-like lymphoid hyperplasia involving primarily the subcutaneous tissue, is reported. The lesion, which arose at a site of tribal scarifications, displayed a deceptive morphology that closely resembled subcutaneous panniculitis-like T-cell lymphoma (SPTCL). An accurate diagnosis could only be made following detailed immunohistochemical and molecular studies. Although SPTCL has been thought to represent a very specific clinicopathologic entity, the present case illustrates that its histological appearance can, however, be closely mimicked by reactive and benign conditions.

Trisomy 21 as the sole abnormality in a refractory anemia with ring sideroblasts.

Numerous chromosome abnormalities have been described in myelodysplastic syndromes, but single karyotypic aberrations are much less frequent. We report the case of a 65-year-old woman who presented a trisomy 21 as the sole karyotypic anomaly for a refractory anemia with ring sideroblasts. The nature of such an anomaly is discussed in regard to pathogenesis and prognosis.

Nature of the T lymphocytes in lymphocyte predominance Hodgkin's disease.

Little is known about the function of the T lymphocytes in lymphocyte predominance Hodgkin's disease. We report here the case of a 37-year-old man with a diffuse LPHD, featuring a similar increase in T lymphocytes in both the peripheral blood and the tumor, thus allowing for their characterization by functional assays. These cells were CD4+CD45RO+ and produced high amounts of IL-2 and IFN-gamma, consistent with a TH1-type profile. This subset of T helper cells is involved in cellular immunity and could reflect a cytotoxic reaction directed against the neoplastic cells.

A case of pleomorphic T-cell lymphoma with a high content of reactive histiocytes presented with hypereosinophilia.

A case of peripheral T-cell lymphoma classified, according to the updated Kiel classification, as a large pleomorphic T-cell lymphoma with a high content of reactive histiocytes and blood hypereosinophilia is reported. Light microscopic examination revealed a diffuse effacement of the lymph node structure by large pleomorphic lymphoma cells mixed with eosinophils and many histiocytes, some of them presenting discrete features of hemophagocytosis. The neoplastic cells were CD3, CD5, CD8 and HLA-DR positive but failed to show CD30 antigen. DNA molecular analysis displayed simultaneous rearrangements of the genes coding for the delta chain of the T-cell receptor and for the Ig heavy chain. Increased serum levels of angiotensin converting enzyme and ferritin were found and probably induced by the reactive histiocytes. Immunoassays (ELISA) with antibodies directed against some cytokines and against the Tac peptide (sIL-2R) were performed. They demonstrated high serum levels of sIL-2R and a slight increase in GM-CSF, but neither IL-5 nor IL-3. The association of blood hypereosinophilia and histiocytic hyperplasia with a peripheral T-cell lymphoma is discussed.

Lethal cutaneous verrucous carcinoma.

Verrucous carcinoma is a rare subtype of well-differentiated squamous cell carcinoma that may occur anywhere on the skin. It is slow growing, enlarging relentlessly and invading locally. Some cases were reported to occur on chronic scar tissue but demonstrated the same clinical course than in other locations. Herein, we report on a very unusual case of cutaneous verrucous carcinoma arisen from a long-standing (40 years old) burn scar and which involved the pleura five years after the initial surgical resection. To our best knowledge, this is the first case of pleural involvement by a verrucous carcinoma originating from the skin.

[Sympathetic ophthalmia versus Harada disease]. / Ophtalmie sympathique versus maladie de Harada.

An algerian woman presents with a panuveitis of the right eye. Main features of this uveitis are exudative retinal detachments. That happened several months after a corneal ulcer of unknown aetiology on the left eye. Immunologic, neurologic and infections workup associated with previous ocular injury points to the diagnosis of sympathetic ophthalmia. However, the diagnosis of Harada disease can also be considered in this case because of similarities regarding the clinical and the histologic aspects which are exposed. If corticosteroids are the drug of choice, enucleation is still indicated for sympathetic ophthalmia.

[Cryptococcal chorioretinitis and acquired immunodeficiency syndrome: apropos of a case]. / Choriorétinite à cryptocoques et syndrome d'immunodéficience acquise: à propos d'un cas.

A patient with acquired immune deficiency and antecedents of pancreatitis presented with headaches, fever, dyspnea and bilateral decrease of vision. A diagnosis of disseminated cryptococcosis was made by lumbar puncture, alveolar washing and elevated cryptococcal antigen in blood, urine and stool. Bilateral chorioretinitis with ischaemic maculopathy was responsible of the low vision and attributed to cryptococcal infection after vitreous puncture and isolation of yeast from the vitreous. Systemic treatment with Amphotericin led to resolution of the chorioretinitis. A retinitis due to cytomegalovirus was associated short time before the patient died. Post-mortem anatomopathologic analysis revealed cryptococcus in high number in the choriocapillaris.

[Sebaceous cutaneous carcinoma. Value of early cytological test]. / Carcinome sébacé cutané. Intérêt de l'examen cytologique précoce.

Sebaceous carcinoma is a skin tumour which frequently metastases to the visceral organs. Needle biopsy is needed for rapid diagnosis. We observed a case in a 65-year-old patient who had a tumour formation below the right maxillary angle and homolateral justamandibular lymph node enlargement. Cytology of the needle biopsy showed a double cell population: small anaplastic cells and an agglutinated cell mass with peripheral maturation and sebaceous differenciation. Treatment was surgical with homolateral node dissection and secondary plasty. The tumour was large, non-encapsulated with local infiltration. On light microscopy, two cell populations were seen. Evident sebaceous differentiation was confirmed by histochemical staining. Immunolabelling confirmed the epithelial nature of the tumour. The clinical diagnosis of sebaceous carcinoma is difficult. Early cytology is needed to identify the two cell components when other cytological signs do not allow a positive diagnosis.

[A bizarre excrescence of the umbilicus in a 1-month-old child]. / Une excroissance bizarre de l'ombilic chez un enfant de un mois.

We report the case of an umbilical polyp, derived from omphalo-mesenteric remnants in an one-month-old female child. This rare abnormality results from a closure defect of the vitelline duct. The vitelline duct normally closes between the 5th and the 7th weeks of intra embryonic development but can lead to several pathologies in case of closure defects, giving rise to abdominal (Meckel diverticulum, vitelline cyst) or umbilical symptoms (umbilical fistula, umbilical sinus and umbilical polyp). These disorders have a 2% incidence, and may induce clinical symptoms of varied gravity ranging from clinical silence to acute abdomen. We seized the opportunity of this rare clinical observation to review the nosology of vitelline duct defects at the light of embryologic data.

[Adenopathy and eosinophilia]. / Adénopathies et eosinophilie.

The exercise topic is the persistence of adenopathies for many years in a young marocan boy who also presents with peripheral blood eosinophilia.

[Disseminated intravascular coagulation syndrome as a manifestation of breast adenocarcinoma metastasis]. / Syndrome de coagulation intravasculaire disséminée révélateur de la généralisation d'un adénocarcinome mammaire.

Disseminated intravascular coagulation is a well known complication of malignancies especially of mucin-secreting cancers. However, it rarely occurs as the first clinical manifestation of a neoplasm. We report the case of a subacute disseminated intravascular coagulation syndrome revealing a metastatic breast carcinoma.

[Pulmonary nodules and arachnophobia]. / Nodules pulmonaires et arachnophobie.

Pulmonary nodules are a common reason for consultation and their investigation must always exclude a possible neoplastic cause. This means that, in addition to a thorough history, investigations may be necessary which are sometimes invasive and therefore potentially a cause of iatrogenic harm. The toxic aetiologies for pulmonary nodules are rare. We report a case of a patient with pulmonary nodules occurring predominantly in the right lung, about 1cm in diameter, non-cavitating without calcification, and sometimes surrounded by a peripheral halo. The nodules were a chance finding during preoperative evaluation. After a comprehensive review, a reaction to an inhaled irritant was the preferred hypothesis, specifically overuse of a compound insecticide containing, in addition to the propellant gas and solvent type hydrocarbon - a mixture of piperonyl butoxide, of esbiothrine and permethrin. Removal of this led to the complete disappearance of nodules. Pathological examination identified bronchiolitis obliterans with organising pneumonia accompanied by non-necrotizing granulomas and lipid vacuoles.

Mycotic aneurysm of the right iliac artery: imaging diagnosis and correlation with pathological findings.

We report the case of a 55-year-old woman who presented at the emergency department with hypogastric pain, fever, clinical signs of sepsis and a critical inflammation syndrome in her blood test values. CT-scan of the abdomen demonstrated an infected aneurysm of the right iliac artery. The patient underwent surgery with a favorable outcome. Histological examination of resected artery was performed and compared to the CT features. The authors review the literature and stress the importance of early diagnosis and treatement.