[Rieger-Marchac flaps: Complications and patient satisfaction]. / Le lambeau de Rieger-Marchac : complications et satisfaction des patients.

PURPOSE: Like all surgical procedures, dorsal nasal flaps may be followed by both early and late complications. The aim of this study was to evaluate the surgical complications and cosmetic outcome of dorsal nasal flaps over a 7-year period in an academic dermatologic surgery unit. PATIENTS AND METHODS: Data were collected retrospectively for all patients undergoing dorsal nasal flap between 1 January 2006 and 31 December 2013. Early and late complications were recorded. Patients were contacted by phone to assess long-term outcomes. RESULTS: A total of 35 patients were included. Early complications included bleeding (n=2), local infection (n=2) and focal flap necrosis (n=1). Late complications comprised flap thickening (n=7), restriction of the medial canthus (n=2), opening of the labionasal angle (n=1), stitch granuloma (n=1) and telangiectasia on the flap (n=1). Regarding the aesthetic result, seven patients were very satisfied with the flap. Four patients underwent corrective surgery and one patient had laser treatment for telangiectasia on the flap. CONCLUSION: Two thirds of patients were satisfied with the aesthetic results and one third had late complications of the flap. Consequently, patients undergoing Rieger-Marchac procedures must be informed of the potential need for further corrective measures following nasal dorsal flap repair.

AFAST - Adult Female Acne Scoring Tool: an easy-to-use tool for scoring acne in adult females.

BACKGROUND: Acne is a concern in adults, especially in women. The specifications in current acne grading systems are not applicable to this particular population. OBJECTIVE: To develop and validate a measurement tool (AFAST: adult female acne scoring tool) for acne in women by taking into account the specific locations of adult female acne, and to evaluate the impact of the photographic modalities on rating reproducibility. METHODS: Six experts in dermatology rated pictures of 54 women with a phototype from I to IV during two sessions, with an interval of 24 h. They rated the acne severity on the face using the GEA scale (Score 1) together with a new scale to assess acne on the mandibular zone (Score 2). Pictures of 30 women were taken using a standardized photographic device; pictures of the other 24 women were taken by their own dermatologists during daily practice. RESULTS: At session 1, the inter-rater's reproducibility was good for Score 1 with an ICC of 0.77 [0.72-0.83], and excellent for Score 2 with an ICC of 0.87 [0.82-0.91]. Between sessions 1 and 2, the mean intra-rater's reproducibility was excellent for both scores with an ICC of 0.88 [0.84-0.92] for Score 1, and an ICC of 0.87 [0.78-0.92] for Score 2. Photographic modalities had no significant effect on the inter- and intra-rater's reproducibility. CONCLUSION: For the first time, it has been demonstrated that AFAST can accurately rate acne severity in women. It is a promising, easy-to-use tool for both daily practice and clinical investigation.

Evaluation of a PCR test for detection of treponema pallidum in swabs and blood.

Syphilis diagnosis is based on clinical observation, serological analysis, and dark-field microscopy (DFM) detection of Treponema pallidum subsp. pallidum, the etiological agent of syphilis, in skin ulcers. We performed a nested PCR (nPCR) assay specifically amplifying the tpp47 gene of T. pallidum from swab and blood specimens. We studied a cohort of 294 patients with suspected syphilis and 35 healthy volunteers. Eighty-seven of the 294 patients had primary syphilis, 103 had secondary syphilis, 40 had latent syphilis, and 64 were found not to have syphilis. The T. pallidum nPCR results for swab specimens were highly concordant with syphilis diagnosis, with a sensitivity of 82% and a specificity of 95%. Reasonable agreement was observed between the results obtained with the nPCR and DFM methods (kappa = 0.53). No agreement was found between the nPCR detection of T. pallidum in blood and the diagnosis of syphilis, with sensitivities of 29, 18, 14.7, and 24% and specificities of 96, 92, 93, and 97% for peripheral blood mononuclear cell (PBMC), plasma, serum, and whole-blood fractions, respectively. HIV status did not affect the frequency of T. pallidum detection in any of the specimens tested. Swab specimens from mucosal or skin lesions seemed to be more useful than blood for the efficient detection of the T. pallidum genome and, thus, for the diagnosis of syphilis.

Factors associated with severe skin infections in patients treated with biologic therapies for inflammatory rheumatic diseases.

BACKGROUND: The incidence of severe infections is increased under biologic therapies and the skin is the second localization. OBJECTIVE: To appraise the factors associated with severe skin infections (SSI) in patients under biologic therapies for inflammatory rheumatic diseases (IRD). METHODS: We performed a case-control (ratio 1:3) study nested in a prospective cohort of patients with IRD. SSI was defined as requiring hospitalization or intravenous anti-infectious therapy. We defined two imbedded periods: period A was the time window between the first biologic therapy and the SSI; period B was the last 3 or 12 months (for tumor necrosis factor blockers or rituximab, respectively) before the SSI. RESULTS: Among 4,361 patients with IRD, 29 had a SSI under biologic therapy. In multivariate analyses, SSI were significantly associated with smoking, baseline C-reactive protein and gammaglobulinemia, non-steroidal anti-inflammatory drugs before biologic therapy, cumulative dose of steroids, concomitant steroids during period A, number of different biologic therapies during period A, treatment with infliximab during period A, period B or as first biologic therapy and treatment at high dose during period B. CONCLUSION: In patients under biologic therapies for IRD, the risk of SSI is associated with several factors including tobacco, treatment with infliximab or high dose range.

[Measles in adults: an emerging disease not sparing medical staff]. / Rougeole de l'adulte : une maladie émergente n'épargnant pas le personnel médical.

BACKGROUND: A large outbreak of measles is taking place in Europe and is related to a low vaccination coverage. Measles is observed in adults. METHODS: We retrospectively studied all the consecutive cases of measles seen in adults between the 1/1/2007 and the 30/4/2009 in four Parisian hospitals. RESULTS: Twenty-one patients were included. Six patients (29%) were health care workers (HCW) including five (83%) who were vaccinated. Twenty (95%) patients were hospitalized. All patients presented with febrile exanthema, cough and rhinitis in association with hepatic involvement in 71%. Neither death nor sequelae were reported. CONCLUSION: Measles may occur in HCW, most of them being insufficiently covered by the vaccination. Therefore, since 2010, one injection of measles vaccine is now recommended in France, for HCW without history of measles or vaccination with two doses. Furthermore, adequate respiratory precautions should be taken when seeing patients with febrile exanthema and cough.

[Using PubMed subject headings to enhance reference searches]. / Utiliser les descripteurs dans PubMed pour améliorer une recherche bibliographique.

The PubMed search engine is an essential tool to stay abreast of the latest medical literature on specific topics. While the basic search techniques are common knowledge, the ability to use medical subject headings properly is an essential in obtaining valuable references. The purpose of this article is to explain what medical subject headings are and how they can be used to improve the results of reference searches in PubMed.

Fulminant respiratory failure and death in a patient with idiopathic bronchiolitis obliterans.

A middle-aged man with fulminant respiratory failure was found to have idiopathic bronchiolitis obliterans with organizing pneumonia on lung biopsy specimen. His course was atypical in that it was not altered by steroid therapy and led to fulminant respiratory failure and death.

Disseminated infection with Mycobacterium avium-intracellulare. A report of 13 cases and a review of the literature.

Thirteen cases of disseminated infection with Mycobacterium avium-intracellulare (MAI) seen at the National Jewish Hospital and Research Center and 24 cases from the literature were analyzed to define clinical and therapeutic features of the disease. Disseminated MAI infection was a disease of immunocompromised and apparently normal hosts. It was acquired from the environment by unknown mechanisms, usually entering the body through the lungs and spreading to include the reticuloendothelial system, bones, and less commonly, the skin. Diagnosis was often delayed and required culture of tissue or secretions. Medical personnel must maintain a high index of suspicion for MAI disease, especially in immunocompromised hosts. These patients should be monitored carefully for evidence of MAI with frequent cultures of blood and bone marrow. Blood culture systems able to recover MAI promptly and reliably should be employed (52, 64). New diagnostic aids, such as the standardized preparation of PPD-B currently being prepared or tests for antibody to MAI, will help in differentiating MAI from other processes. If MAI is recovered, broad-spectrum therapy should be instituted. Response to combination antimicrobial chemotherapy in the patients surveyed in this report was gratifying. Over two-thirds of treated patients responded to therapy. New antimycobacterial agents such as ansamycin and thienamycin have been shown to have activity against MAI in vitro (40, 81, 92) and may further improve therapeutic efficacy. Studies of in vitro synergy, currently in progress in our laboratory, will also help define the optimal therapeutic regimen for each individual patient. While the patients presented in this report had a reassuring response to therapy, those who had many bacilli in the tissues had a poorer outcome. Patients with AIDS often have this lepromatous histology (37) and thus may respond more poorly than the patients in this report even when optimal therapy is employed. Careful monitoring of AIDS patients for MAI infection may permit earlier institution of therapy and improve the chances for control of the infection. Studies to assess the relationship of in vitro sensitivity to therapeutic response in these patients are currently underway in our laboratory. It is hoped that early institution of therapy and optimization of regimens according to in vitro sensitivity data will lead to decreased morbidity and mortality in all patients with MAI infection.

Splenic pathology in thrombotic thrombocytopenic purpura.

To determine splenic pathology in thrombotic thrombocytopenic purpura (TTP), 10 spleens and two accessory spleens were studied. The eight women and two men ranged from 20 to 66 years of age (mean age, 39 years). Three spleens were enlarged. Thrombi were noted in arteries and arterioles in nine specimens: no associated inflammation was seen. Periodic acid-Schiff-positive diastase-resistant hyaline subendothelial deposits (SEDs) were present in all cases. Some arterioles showed a transition between thrombi and SEDs. The presence of platelets or platelet-related material in SEDs and thrombi was documented by factor VIII staining. Hyperplasia of B cells and germinal centers was present in 67%, and periarteriolar concentric fibrosis ("onion-skinning") in 58%. Histiocytes showed prominent iron deposits in 92% and hemophagocytosis in 83% of cases. Extramedullary hematopoiesis was present in 42%. Blood lakes, infarcts, and endothelial hyperplasia were rarely noted; microaneurysms were not seen. Ten spleens from patients with idiopathic thrombocytopenic purpura and 10 age-matched control spleens rarely showed SEDs or hemosiderosis and did not show hemophagocytosis or thrombi. We conclude that subendothelial deposits may be related to platelet thrombi incorporated into vessel walls. Germinal centers and periarteriolar concentric fibrosis may indicate an immunologic role in TTP, as in systemic lupus erythematosus.

Hemoglobin spherulosis in the vitreous cavity.

A 50-year-old woman was evaluated for a subretinal hemorrhage that extended into the vitreous of the right eye. Examination showed multiple refractile brown spherules in the vitreous. A vitrectomy was performed, and pathologic examination of the spherules showed them to be composed of free hemoglobin. Free-hemoglobin spherulosis in the vitreous, like cholesterosis bulbi, is a manifestation of vitreous hemorrhage.

Bone marrow findings in connective tissue disease.

The authors studied 35 marrow biopsies from 32 patients with rheumatoid arthritis, systemic lupus erythematosus, mixed connective tissue disease, polymyositis, and psoriatic arthritis. Reasons for biopsy included cytopenia, fever of unknown origin, and malignancy. Cellularity was abnormal in 71%. Plasma cells were increased in 60% and associated with lymphoid aggregates. Immunoperoxidase stains showed polyclonal perivascular plasma cells and increased T-cells forming lymphoid aggregates. Two patients had granulomas without documented infection. Anemic patients had findings consistent with anemia of chronic disease, erythroid aplasia, hemolysis, and iron deficiency. Iron stores were variable. Platelet and granulocyte precursors were variably altered and did not predictably correlate with the presence, absence, or cause of thrombocytopenia and neutropenia. Myelodysplastic syndromes were present in two patients with rheumatoid arthritis. Osteomalacia and osteoporosis were seen, resulting from renal failure and steroids. Marrow findings are unpredictable and reflect the diverse causes of cytopenias in patients with connective tissue disorders.

Acute monocytic leukemia with chloroacetate esterase positivity. FAB M4 or M5?

French-American-British criteria for the diagnosis of acute monocytic leukemia (M5) require that 80% of nonerythroid bone marrow cells consist of monoblasts, promonocytes, and/or monocytes. Monocytic differentiation is demonstrated by fluoride-sensitive nonspecific esterase positivity. Chloroacetate esterase positivity is accepted as a marker of granulocytic differentiation. Three cases fulfilling French-American-British criteria for M5 showed fluoride-sensitive nonspecific esterase positivity in up to 100% of nonerythroid marrow cells but also exhibited strong chloroacetate esterase positivity in 20% to 90% of the same population. Less than 5% of blasts stained for Sudan black B and peroxidase. These cases may be viewed as chloroacetic esterase-positive acute monocytic leukemia or as acute myelomonocytic leukemia. The authors favor the former because the cases were myeloperoxidase negative; however, these cases indicate that chloroacetate esterase may not be a specific marker for granulocytic differentiation.

Prospective gene rearrangement studies and multiparameter analysis of acute myeloid leukemia.

Twenty-six cases of acute myeloid leukemia (AML) with cytochemical and immunophenotypic data were studied prospectively for immunoglobulin and T-cell receptor gene rearrangement. Dysmyelopoiesis was seen in 100% and Auer rods in 18%. Sudan black B was positive in 83% of the cases, peroxidase in 76%, nonspecific esterase in 74% (fluoride-inhibited in 82%), chloroacetate in 70%, acid phosphatase and PAS in 100%, and immunoperoxidase stains for platelet glycoprotein IIIa and factor VIII in 0% of the cases studied. Flow cytometry revealed myeloid phenotype in 19 of 20 cases. In four cases 5-86% of cells were TdT positive. Heavy-chain gene rearrangement was demonstrated in three cases (12%) and kappa light chain gene rearrangement in one; clinically significant rearrangement of the T-cell receptor gene was not found. Rearrangements of immunoglobulin genes are found occasionally in AML; these may represent nonspecific findings or coexistent lymphoid differentiation in AML.

Dysmegakaryopoiesis resembling acute megakaryoblastic leukemia in treated acute myeloid leukemia.

Acute myeloid leukemia (AML) is characterized by trilineage dysplasia, including atypical megakaryocytes. Acute megakaryoblastic leukemia (FAB M7) is particularly associated with atypical megakaryocytic hyperplasia (AMH). Fifteen patients with nonmegakaryoblastic AML developed AMH after therapy, comprising 12.6% of cases of AML diagnosed from 1986 to 1989. Platelet counts were normal in nine patients and decreased in six. Blasts comprised less than 5% of cells in 40% of the biopsies, ranged from 5-15% in 53%, and comprised more than 30% of cells in 7%. Numerous small hypo- and hyperlobated megakaryocytes were seen in all specimens, often occurring in clusters, and were more easily seen in sections than in smears. Subsequent biopsies in 13 patients showed a remission marrow in seven, increased blasts in three, and AML in three; none showed AMH. AMH resembling acute megakaryoblastic leukemia may be seen transiently after treatment of AML.

Myelodysplastic syndromes and acute myeloid leukemia in connective tissue disease after single-agent chemotherapy.

Cytopenias are typical of patients with connective tissue disease (CTD) and are usually related to autoimmune phenomena. In some cases, cytopenia may be the result of treatment with cytotoxic agents. Although multi-drug therapy is known to produce myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML) in patients with CTD, treatment with single-agent therapy, particularly methotrexate, has rarely been associated with secondary MDS or AML. Blood and marrow samples were studied from 3 men and 5 women with rheumatoid arthritis (5 cases), Behcet's disease (2 cases), and systemic lupus erythematosus (1 case) developing MDS or AML after methotrexate (5 cases), chlorambucil (2 cases), and cytoxan (1 case). The durations of CTD ranged from less than 6 months to more than 10 years. Five patients (63%) presented with MDS including refractory anemia (RA), refractory thrombocytopenia (RT), refractory anemia with excess blasts (RAEB), chronic myelomonocytic leukemia (CMML), and RAEB in transformation. Patients with RT, CMML, and RAEB in transformation developed AML. Of six patients presenting with or developing AML, four had AML with differentiation (FAB M2), one acute myelomonocytic leukemia (FAB M4), and one M4Eo. Inv 16 was seen in the M4Eo and t(8;21) in one case of M2. Four of six patients are alive up to 6 years after diagnosis of AML. One of three patients with MDS is alive 6 months after diagnosis of MDS. Cytopenias in patients with CTD may be due to therapy-related MDS or AML occurring in a setting of single-agent chemotherapy, including methotrexate.

Failure to engraft after bone marrow transplantation: bone marrow morphologic findings.

Few studies have explored bone marrow findings in patients with graft failure or delayed engraftment after bone marrow transplantation (BMT). The authors retrospectively identified 4 patients of 165 transplant recipients who underwent bone marrow examination after BMT because peripheral blood counts had not recovered to expected levels. All patients were women who were 21- to 49-years old (mean 37 years). Three patients underwent autologous BMT; the fourth received peripheral stem cell infusion. Transplants were performed for treatment of Hodgkin's disease, breast carcinoma, and follicular small cleaved cell lymphoma. Three patients received GM-CSF after marrow infusion. The time between transplant and biopsy ranged from 19 to 40 days (mean 22 days). White cell counts ranged from 0.1 to 0.6 x 10(9)/L, hematocrits from .25 to .41, and platelet counts from 10 x 10(9)/L to 39 x 10(9)/L. Aspirate smears were markedly hypocellular in all cases, and markedly hypocellular, and all contained histiocytes with foamy eosinophilic cytoplasm diffusely throughout the biopsy. Acid-fast and Gomori's methenamine-silver (GMS) stains were negative. Serous fat atrophy and marrow fibrosis were not seen. Delayed engraftment after BMT may be associated with a profuse histiocytic proliferation similar to that seen in immunodeficiency, some hematologic disorders, and storage diseases.

Splenic pathology after traumatic injury.

Little is known concerning the pathology of spleens removed for traumatic injury. The authors studied the gross and microscopic features of 44 spleens removed for trauma and received at the Surgical Pathology Division of Parkland Memorial Hospital and 10 normal control spleens from the Medical Examiner's Office, Dallas County, Texas. The mean age of patients undergoing post-traumatic splenectomy was 29.6 years with a male:female ratio of 6:1. The most common procedure done for traumatic splenic rupture was splenectomy (39 of 44 cases); wedge resection or partial splenectomy was done in 5 cases. The mean weight of the spleens was 167 g (181 g in males, 93 g in females, P = .056). Capsular laceration or rupture were noted in 86% of post-trauma spleens, usually involving the superior pole and/or hilum. Subcapsular neutrophilic infiltrates were seen in 7%. Gross evidence of parenchymal hemorrhage was seen in 25%, and microscopic evidence in 68%. Control spleens showed none of these findings. Germinal centers were present in 77% of spleens with germinal center hyperplasia in 55% (including patients 16-59 years old), numerous primary follicles in 45%, mantle zone hyperplasia in 10%, and marginal zone hyperplasia in 41% of patient spleens. Control spleens showed few or none of these findings. No patient spleens had histologic features suggestive of Epstein-Barr virus (EBV) or other infection, granulomas (other than lipogranulomas), or infarct. The findings suggest that splenic rupture after trauma may be related to prior immunologic stimulation of the spleen, and that spleens removed for trauma are not equivalent to normal controls.

Mantle cell lymphoma. Morphologic findings in bone marrow involvement.

Although mantle cell lymphoma (MCL), has been well described in lymph nodes, involvement of blood and bone marrow has not been well defined. The authors reviewed involved blood and marrow specimens from 13 patients with MCL to determine patterns of infiltration. These findings were compared to marrow involvement by follicular small cleaved cell lymphoma (SCCL) and small lymphocytic lymphoma (SLL). Peripheral blood involvement by MCL was present in 5 patients (38%). The circulating lymphoma cells were small (7-10 mu) with slightly folded nuclei. Marrow involvement ranged from 5% to 90% of the marrow space and was predominantly intertrabecular, including nodules and interstitial infiltrates (9 cases each; 68%). Paratrabecular aggregates (6 cases; 46%) and diffuse replacement by lymphoma (3 cases; 23%) were also seen. In SCCL, paratrabecular involvement was seen as were interstitial nodules. Cases of SLL showed diffuse, interstitial or nodular involvement without paratrabecular localization. Cytologic comparison showed nuclei that were angulated in SCCL, round in SLL, and slightly irregular in MCL, with considerable overlap among the groups. The architectural and cytologic findings in marrow involved by MCL show features of both SCCL and SLL, and cannot be used to definitively diagnose MCL.