Pseudosystemic sclerosis as a complication of recombinant human interleukin 2 (aldesleukin) therapy.
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Biomedical subjects
Publications and source records attributed to P Courville.
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AIM: Thirty-five cases of lymphadenectomy carried out in the context of positive sentinel lymph node for malignant melanoma have been reviewed to assess the prognostic value of certain metastatic charachteristics. We have checked wether the type (macro or micrometastasis) and localisation (subcapsular or intraparenchymal) in the sentinel lymph node had predictive value for the lymphadenectomy outcome and evolution of the case. MATERIAL AND METHODS: The retrospective study relates to 35 cases (with an average 2 years history) taken from a total of 87 sentinel lymph node protocols; average age 46.5 years, Breslow 2.5 mm with an history of 25 months. RESULTS: Among the 35 positive sentinel lymph nodes we have 19 cases (54.2%) of micrometastasis. Among the 35 lymphadenectomy 5 cases (14.28%) turned out positive, 3 of which concerned micrometastatic sentinel lymph nodes. In our cohort the micrometastatic nature of sentinel lymph nodes did not have statistically significant impact upon the lymphadenectomy result but showed more favourable short-term evolution with 68.42% metastatic free evolution as against 43.75% in case of initial macrometastasis. The subcapsular localisation of micrometastasis equally represents a factor of improved prognosis (69.2% of metastatic free evolution against of 30.8% in the case of intraparenchymal localistion). CONCLUSION: Unfortunately, none of the studied criteria justifies a modification of our present clinical attitude whereby a systematic lymphadenectomy in cases of positive sentinel lymph nodes is performed, whatever the type or localisation of the relevant metastases.
INTRODUCTION: The prevalence of cutaneous leishmaniasis is increasing, especially in immunocompromized subjects. CASE RECORD: We report a case of particular interest, where a patient with steroid refractory polymyositis developed cutaneous leishmaniasis. Clinical outcome was favorable after institution of intralesional antimony therapy. DISCUSSION: Cutaneous leishmaniasis is still recognized to be an opportunistic infection. Its frequency is indeed higher in immunocompromized subjects, mainly HIV-infected patients. In our patient, both polymyositis and steroid therapy were predisposing factors of cutaneous leishmaniasis onset; prednisone therapy has been postulated to be associated with immune dysfunction leading to: reduced blood T cells' levels (CD4 et CD8) as well as decreased cytokine synthesis (e.g. interferon gamma).
BACKGROUND: Dyspareunia is frequently associated with a psychiatric origin, particularly in patients with no obvious vulvovaginal or pelvic disease. The aim of this study was to assess the frequency of dry syndrome in patients with dyspareunia and to evaluate the main clinical and biological features and follow-up data for women with dyspareunia and dry syndrome. PATIENTS AND METHODS: Twenty-two patients presenting chronic idiopathic dyspareunia (without clear vulvovaginal dermatosis or infection) were included in this retrospective study. All patients underwent history-taking, gynecological examination, a Schirmer tear test, a sugar test, labial salivary gland biopsy assessment and immunological examination. A diagnosis of Sjögren's syndrome was considered where histological examination of the salivary glands showed a lymphocyte infiltration corresponding to stage 3 or 4 in the Chisholm classification. Diagnosis of dry syndrome without Sjögren's syndrome was made in patients with xerostomia and/or xerophthalmia without a specific histological picture of Sjögren's syndrome or immunological abnormalities at salivary gland biopsy. RESULTS: Based on our criteria, 10 patients (45%) had dry syndrome, including 4 with Sjögren's syndrome and 6 with dry syndrome without Sjögren's syndrome. 9 of these 10 patients presented either xerostomia (7 cases) and/or xerophthalmia (7 cases). Vaginal dryness was reported by 3 of the 10 women with dry syndrome but also by 4 of 12 women without dry syndrome. Examination of the vulva showed no particular clinical features and treatment with an emollient was not effective in all cases. DISCUSSION: This study showed a high frequency of dry syndrome in patients with chronic "idiopathic" dyspareunia. The incidence of the condition was even greater in women with functional conditions evocative of dry syndrome. Women presenting dyspareunia with no clearly related clinical causes should thus be carefully assessed for dry syndrome.
INTRODUCTION: Anti-TNFalpha has occasionally been used in the treatment of recalcitrant forms of systemic vasculitis such as Behçet's disease, Wegener's granulomatosis and Churg-Strauss syndrome. We report on the outcome of treatment in rheumatoid arthritis patients with cutaneous vasculitis lesions on anti-TNFalpha. OBSERVATIONS: Two patients with rheumatoid arthritis present for several years had necrotic ulcers of the lower limbs due to cutaneous vasculitis. After the failure of various immunosuppressive drugs (cyclophosphamide, azathioprine, methotrexate), the two patients were treated with anti-TNFalpha: infliximab in the first case and adalimumab in the second. Cutaneous ulcers healed within two to four months of the start of anti-TNFalpha treatment. Despite ongoing anti-TNFalpha treatment, these cutaneous ulcers relapsed four to six months after complete healing. CONCLUSION: Initially spectacular healing of cutaneous vasculitis ulcers under anti-TNF alpha treatment followed by relapse after several months of treatment is suggestive of an escape mechanism.
BACKGROUND: Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy is often poorly specific for maculopapular eruptions. We report an atypical case of neutrophilic disorder associating subcorneal pustules and hypodermal nodules in a male patient with angioimmunoblastic lymphadenopathy. CASE REPORT: A 62 year-old man with angioimmunoblastic lymphadenopathy was treated with oral prednisone (15 mg/day) and cyclophosphamide. Pustules and deep nodules occurred simultaneously on the arms and trunk, the abdomen and the back respectively. Biopsy of these lesions showed an infiltration of neutrophil polymorphonuclears in the median and superficial dermis with a subcorneal pustule and nodular infiltration of neutrophil polymorphonuclears in the deep dermis and hypodermis respectively. Increasing the dose of prednisone to 30 mg/day led to rapid and complete healing of the cutaneous lesions. DISCUSSION: This atypical case of neutrophilic disorder associating acute subcorneal pustules and deep dermal-hypodermal nodules seems to correspond to a unique type of neutrophilic disorder that differs from those previously reported in the literature. Neutrophilic disorders have been reported only rarely in patients with angioimmunoblastic lymphadenopathy. This case illustrates the wide range of clinical and histological lesions found in neutrophilic disorders, a number of which may coexist in a single patient.
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The natural resistance-associated macrophage protein (Nramp) homologs form a family of proton-coupled transporters that facilitate the cellular absorption of divalent metal ions (Me2+, including Mn2+, Fe2+, Co2+, and Cd2+). The Nramp, or solute carrier 11 (SLC11), family is conserved in eukaryotes and bacteria. Humans and rodents express 2 parologous genes that are associated with iron disorders and immune diseases. The NRAMP1 (SLC11A1) protein is specific to professional phagocytes and extrudes Me2+ from the phagosome to defend against ingested microbes; polymorphisms in the NRAMP1 gene are associated with various immune diseases. Several isoforms of NRAMP2 (SLC11A2, DMT1, DCT1) are expressed ubiquitously in recycling endosomes or specifically at the apical membrane of epithelial cells in intestine and kidneys, and can contribute to iron overload, whereas mutations impairing NRAMP2 function cause a form of congenital microcytic hypochromic anemia. Structure-function studies, using various experimental models, and mutagenesis approaches have begun to reveal the overall transmembrane organization of Nramp, some of the transmembrane segments (TMS) that are functionally important, and an unusual mechanism coupling Me2+ and proton H+ transport. The approaches used include functional complementation of yeast knockout strains, electrophysiology analyses in Xenopus oocytes, and transport assays that use mammalian and bacterial cells and direct and indirect measurements of SLC11 transporter properties. These complementary studies enabled the identification of TMS1 and 6 as crucial structural segments for Me2+ and H+ symport, and will help develop a deeper understanding of the Nramp transport mechanism and its contribution to Me2+ homeostasis in human health and diseases.
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Paclitaxel is a recent antineoplastic agent that belongs to the taxane family. Its activity has been demonstrated in advanced and refractory ovarian, breast, lung, and head and neck cancer. Adverse cutaneous reactions to paclitaxel have been reported, namely bullous fixed drug eruption, onycholysis, acral erythema, erythema multiforme, and pustular eruption. We report the first case of scleroderma-like changes after paclitaxel administration. A 63-year-old patient presented with an edematous and infiltrated erythema of the head, neck, axillae, and left hand 10 days after administration of paclitaxel and paraplatin for primitive peritoneal cancer. Cutaneous lesions improved after a change from paclitaxel to cyclophosphamide. Cutaneous lesions recurred 3 months later, after reintroduction of paclitaxel, and progressively evolved to cutaneous sclerosis. Skin biopsy showed a dermal fibrosis. Biologic tests revealed no autoimmunity. Scleroderma-like lesions of this patient were reminiscent of previously reported cases that occurred after administration of docetaxel, which also belongs to the taxan family. Thus, scleroderma-like syndromes seem to represent a unique cutaneous adverse event caused by taxanes.
PURPOSE: To evaluate the prognostic value of DNA ploidy and proliferative activity in metastasis occurrence after enucleation for choroidal melanomas. METHODS: This retrospective study investigated 14 patients (eight males, six females) using flow cytometry and immunohistochemistry with the Ki67 antibody. RESULTS: Two of the tumors were aneuploid, 12 were diploid. The proliferation index was between 0% and 16%. Two tumors progressed to metastatic disease; both were diploid and had a low proliferation index. They were mixed cell type. The median observation period was 3 years and 9 months. CONCLUSION: DNA content and proliferative activity are not correlated with metastasis occurrence. Sclera invasion is the best prognostic factor.
BACKGROUND: Cryptococcosis and alternariosis are rare opportunistic infections often observed in immunocompromised patients. Because Cryptococcus and Alternaria are ubiquitous fungi found in soil, the presence of fungi in the dermis has to be observed on histological examination to confirm a real cutaneous, invasive, infection. PATIENT: We report the first case of concomitant cutaneous cryptococcosis and cutaneous alternariosis, in an immunocompromised patient treated for a metastatic thymoma. CONCLUSION: This observation underlines the fact that the possible co-existence of several rare infections in immunocompromised patients should take into consideration pathogen identification in order to adapt the therapy to individual patient requirements.
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INTRODUCTION: Eczematiform eruptions in the elderly represent a relatively frequent motive for consultation and may lead to repeated hospitalization. Their etiologic diagnosis is often difficult and explains the frequent relapses. The frequent relapses can be explained by the difficulty in determining their etiologic diagnosis. The aims of this study were: 1) to specify the evolving characteristics of these eruptions in elderly patients and 2) determine their etiology. PATIENTS AND METHODS: The inclusion criteria in this retrospective study were: patients aged over 65 at the time of diagnosis, presenting with extensive eczematiform eruption (> 20 p. 100 of body surface) and lasting for more than one month. Eczema on stasis dermatitis of the lower limbs and generalized contact eczema were excluded. Eighty-three patients followed between January 1990 and January 1999 were included. The clinical, biological, histological and evolving characteristics were analyzed. RESULTS: Mean age of patients was 77 +/- 8 years and the male female sex ratio was 2.4. Patients received a mean of 4.0 +/- 2.6 drugs/patient, consisting essentially of cardiovascular and psychotropic agents. The cutaneous eruption had evolved a mean of 12.5 months (1 to 48 months) before diagnosis. Eczema was pruriginous in 92 p. 100 of cases. Frequent relapses were observed in 68 p. 100 of cases. Precise etiologic diagnosis was retained in 48 patients (58 p. 100). This was disseminated contact eczema (n=19), lymphoma cutis (n=10), atopic eczema (n=7), scabies acariasis (n=6) and pemphigoid (n=6). No etiologic diagnosis was retained in the remaining 35 patients (42 p. 100). Comparison of the characteristics in the 2 groups showed excessive consummation of medicinal products (p=0.024), predominant eruption of sun-exposed areas (p=0.004) and a greater frequency of histological images of keratinocyte necrosis (p=0.0072) in patients presenting eczematiform eruptions of unknown etiology. DISCUSSION: These observations suggest the eventual responsibility of medicinal products in the occurrence of certain extensive and chronic eczematiform eruptions in the elderly. However, the delays of imputability of various causal drugs were often longer than those currently admitted for toxidermia, and the withdrawal of potentially imputable agents rarely led to spectacular improvement in the lesions. A case test report is in progress to specify this hypothesis.
Neutrophilic dermatoses are characterized histologically by a sterile infiltration of neutrophils throughout the dermis. The condition may affect various internal organs, notably the lungs, the digestive tract, and the joints, whereas muscle impairment is considered to be extremely rare. We describe the case of a patient with pyoderma gangrenosum in whom severe sterile neutrophilic myositis developed as the first manifestation of an acute myelogenous leukemia.
A 46-year-old patient was referred for a right mammary nodule associated with a chronic mastopathy. Cytological examination suggested an inflammatory process but frozen sections concluded to carcinoma with lymphoid stroma. Histological examination found characteristic features of MALT lymphoma: reactive lymphoid follicles, lymphoplasmocytoid lymphoid proliferation and lymphoepithelial lesions. Immunohistochemistry and in situ hybridation revealed a monotypic IgGk immunoglobulin. Molecular biology techniques found a rearrangement of IgH locus. Lymphocytic perigalactophoritis was found in the remaining breast tissue. After 33 months, no tumoral recurrence occurred. Primary MALT lymphoma of the breast is exceptional and its histogenesis is unclear. The role of pre-existent inflammatory lesions in the genesis of this tumour is not established. Our observation illustrates the diagnostic difficulty and the usefulness of complementary techniques in this diagnosis. The existence of inflammation close to tumour is interesting to emphasize.