scholarly journals Management Of Childhood Lichen Planus

2016 ◽  
Vol 12 (1) ◽  
pp. 1-6
Author(s):  
DM Thapa ◽  
M Malathi
Keyword(s):  
Lichen Planus ◽  
Treatment Options ◽  
Poor Response ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Direct Immunofluorescence ◽  
Topical Steroids ◽  
Indian Children ◽  
Lichen Planopilaris ◽  
Oral Steroids

Childhood lichen planus (LP) is a rare entity, with less than 2–3% of all cases seen in patients under 20 years of age. LP in childhood is common in subtropical countries such as India. The most common clinical type of LP in Indian children is the classic form. Approximately 1–15% of patients with LP demonstrate nail involvement, but disease of the nails without skin involvement is rare. LP is diagnosed by historical and physical findings, biopsy results, and, in some cases, features on direct immunofluorescence (DIF). LP tends to have a chronic course. Depending on disease severity, however, LP may respond to a combination of topical or systemic therapies. The response to therapy may be similar to that seen in adults. Moderately potent or super potent steroids are the treatment of choice. Topical steroids can be combined with oral steroids in tapering doses over 2-12 weeks period. This is useful for children with widespread involvement or cutaneous LP lesions associated with significant morbidity. Intralesional steroid is effective for hypertrophic LP unresponsive to topical steroids. Topical steroids in adhesive base used several times a day for several months is a treatment of choice for symptomatic oral LP. Topical steroids in combination with systemic steroids can be given in a tapering dose over 3-6 weeks in very symptomatic cases in early stages. In severe unresponsive cases of both cutaneous and oral LP, oral retnoids are the preferred option. Treatment options for the nail LP in young children are oral steroids given as tapering dose over 4-12 weeks and oral retinoids. Intralesional steroids as nail matrix injection are the third option for older children. Most pediatric patients with LP respond to treatment with full clearance over 1-6 months. Poor response to treatment is a feature of hypertrophic LP and lichen planopilaris. DOI: http://dx.doi.org/10.3126/njdvl.v12i1.10588 Nepal Journal of Dermatology, Venereology & Leprology Vol.12(1) 2014 pp.1-6

scholarly journals Recognizing Presentations of Pemphigoid Gestationis: A Case Study

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Sadie Henry
Keyword(s):  
Case Reports ◽  
Well Being ◽  
Direct Immunofluorescence ◽  
Topical Steroids ◽  
Pemphigoid Gestationis ◽  
Adequate Treatment ◽  
Autoimmune Blistering Disease ◽  
Oral Steroids ◽  
Blistering Disease ◽  
Dermatoses Of Pregnancy

Introduction. Pemphigoid gestationis (PG) is an autoimmune blistering disease that occurs in approximately 1 in 50,000 pregnancies. Failing to recognize PG may lead to inadequate maternal treatment and possible neonatal complications.Case Report. At 18 weeks of gestation, a 36-year-old otherwise healthy Caucasian G4P1 presented with pruritic papules on her anterior thighs, initially treated with topical steroids. At 31 weeks of gestation, she was switched to oral steroids after her rash and pruritus worsened. The patient had an uncomplicated SVD of a healthy female infant at 37 weeks of gestation and was immediately tapered off steroid treatment, resulting in a severe postpartum flare of her disease.Discussion. This case was similar to reported cases of pruritic urticarial papules followed by blisters; however, this patient had palm, sole, and mucous membrane involvement, which is rare. Biopsy for direct immunofluorescence or ELISA is the preferred test for diagnosis. Previous case reports describe severe postdelivery flares that require higher steroid doses. Obstetrical providers need to be familiar with this disease although it is rare, as this condition can be easily confused with other dermatoses of pregnancy. Adequate treatment is imperative for the physical and psychological well-being of the mother and infant.

scholarly journals Primary Biliary Cholangitis: Predictors of Poor Response to Ursodeoxycholic Acid after 1 Year of Treatment in Moroccan Patients

2021 ◽  
pp. 990-995
Author(s):  
Hakima Abid ◽  
Inssaf Akoch ◽  
Maria Lahlali ◽  
Nada Lahmidani ◽  
Mounia El Yousfi ◽  
...  
Keyword(s):  
Ursodeoxycholic Acid ◽  
Biological Response ◽  
Poor Response ◽  
Overlap Syndrome ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Decompensated Cirrhosis ◽  
Primary Biliary Cholangitis ◽  
Biliary Cirrhosis ◽  
Average Value

Introduction: Primary biliary cholangitis (PBC), the new dominance of primary biliary cirrhosis, is a cholestatic disease of autoimmune etiology and represents the leading cause of intra-hepatic cholestasis. Treatment is mainly based on ursodeoxycholic acid. The biological response to treatment is the main predictor of survival without liver transplantation. The Globe-score has been recently validated as the main prognostic factor. Materials and methods: This is a retrospective study carried out in our department collating all cases of PBC followed in consultation. The aim of our work is to research the predictors of poor response to UDCA. Results: 46 patients were collected. The mean age of the patients was 58.82 years, with a predominance of women (n = 43, 93.5%). 34.78% of patients were in the stage of cirrhosis. Anti-M2 mitochondria antibodies were positive in 44 patients (95.65%). An overlap syndrome was found in 11 patients (23.9%). Treatment was based on UDCA combined with corticosteroid therapy and immunosuppressant for overlap syndrome. A biochemical response at 1 year of treatment according to the Paris II criteria was found in 47.8%. The average value of the globe score was 1.35. A score greater than 0.30 was objectified in 20 cases (43.47%). Nineteen cirrhotic patients (41.30%) had a globe score> 0.30. Factors associated with poor response to therapy were: stage of decompensated cirrhosis, elevated pre-therapy total bilirubin greater than 30 g / l and hypoalbunemia less than 35 g / l. The study of the correlation between Globe score and Paris II showed a strong and significant association with a correlation coefficient estimated at 67%. The Paris II score was significantly correlated with the response to treatment (p = 0.001). Conclusion: In accordance with the data in the literature, the globe-score and Paris II are two similar predictive means for evaluating the response at 1 year of treatment in Moroccan context. Keywords: Morocco, Predictors of response, Primary biliary cholangitis, Ursodeoxycholic acid

Indian Dermoscopic Study of Forty Six Cases of Lichen Planus Pigmentosus - New Dermoscopy Signs Discovered

2018 ◽  
Vol 3 (02) ◽  
Author(s):  
Rahul Kumar Sharma, Rajendra Kumar Sharma
Keyword(s):  
Lichen Planus ◽  
Smart Phone ◽  
Poor Response ◽  
Response To Treatment ◽  
Dermatology Clinic ◽  
Single Marker ◽  
Brownish Black ◽  
Brown Pigmentation ◽  
Black Pigmentation ◽  
Very High

Lichen planus pigmentosus (LPP) is a atypical pigmented variety of lichen planus. Lichen planus pigmentosus is an unpredictable relapsing idiopathic dermatosis with periods of activation and remission with poor response to treatment and may leads to cosmetically disfiguring post inflammatory pigmentation. Aim - To study dermoscopic features of untreated cases of Lichen planus pigmentosus. Study subjects - All the patients who attended the dermatology clinic from November 2015 to November 2017 with the clinical diagnosis of LPP and who fulfilled the inclusion and exclusion criteria. Methodology - All the patients who attended the dermatology clinic with the diagnosis of LPP were examined by a dermatoscope. Dermoscopy was performed with DL4 dermatoscope. The images were further magnified with smart phone. Results - Our study showed various dermoscopic signs in cases of LPP like annular granular pattern (35 cases), annular globular pattern (5 cases), homogeneous brown pigmentation (12 cases), homogeneous brownish black pigmentation (8 cases), brownish ovoid nests (3 cases), bluish blackish fine dots (4 cases), Wickham’s striae(2 cases) and pigmented targetoid globules(3 cases). Discussion - Dermatoscope is an indispensible valuable tool in clinical practice which helps in making early lucid diagnosis of LPP with very high accuracy. Our study showed that annular granular pattern is the commonest pattern in Indian LPP cases followed by homogeneous brown pigmentation. In our Indian LPP dermoscopy study we discovered three novel dermoscopic signs which includes brownish globular nests, pigmented targetoid globules and bluish blackish fine dots. In our study we got few unique cases where Wickham’s striae was also present with other dermoscopic signs which supports the link of LPP to lichen planus. Dermatoscopic diagnosis of LPP is made by combination of various signs and should not be dependent on the presence of single marker.

Anaplastic thyroid cancer: A report of six consecutive cases treated successfully with nab-paclitaxel.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e17586-e17586 ◽  
Author(s):  
Nicholas Travers ◽  
Eleanor Cronin ◽  
Mira Marinova ◽  
Peter Smith ◽  
Andrew Peter Dean
Keyword(s):  
Thyroid Cancer ◽  
Primary Tumour ◽  
Treatment Options ◽  
Poor Response ◽  
Anaplastic Thyroid Cancer ◽  
Response To Treatment ◽  
Phase 2 Trial ◽  
Local Disease ◽  
Radiological Response ◽  
Number Of Cycles

e17586 Background: Advanced anaplastic thyroid cancer is a disease with very few treatment options. Poor response rates have been reported with cisplatin / doxorubicin and modest response noted in a single phase 2 trial with 24 hour paclitaxel infusion. The 50% response rate has not been reproduced by other investigators but raises questions about the possible utility of taxanes. Although paclitaxel is not approved for this indication in Australia, nab-Paclitaxel is available on a compassionate access scheme. We report 6 consecutive cases treated with nab-paclitaxel (as an alternative to paclitaxel infusion) who all exhibited a response to treatment. Methods: 6 consecutive patients with histologically proven advanced anaplastic thyroid cancer were assessed and imaged to document extent of disease. They were all treated with nab-paclitaxel 100mg/M2 given weekly either continuously or for 3 weeks out of every 4. Clinical and radiological response to treatment was documented. Results: The number of cycles given ranged from 3 to 8. Two patients required dose reductions due to neuropathy. Of the 6 patients; 5 had measurable metastatic disease and 5 had measurable local disease. Four patients showed clinical response to treatment with shrinkage of measurable local disease. One patient showed response to treatment with shrinkage of the primary tumour. Three patients with stridor developed considerable relief from this distressing symptom. Three patients with inoperable disease subsequently became operable and were resected to obtain local disease control. One patient with no evaluable disease after resection (Stage 4B) who received adjuvant nab-paclitaxel remains free of recurrence at 4 years. Conclusions: Anaplastic thyroid cancer is generally regarded as malignancy untreatable by chemotherapy. Our pilot series suggests nab-Paclitaxel warrants further evaluation and should be considered as a chemotherapy backline for combination with targeted agents.

scholarly journals Treatment of bone pain secondary to metastases using samarium-153-EDTMP

2004 ◽  
Vol 122 (5) ◽  
pp. 208-212 ◽  
Author(s):  
Elba Cristina Sá de Camargo Etchebehere ◽  
Carlos Araújo Cunha Pereira Neto ◽  
Mariana Cunha Lopes de Lima ◽  
Allan de Oliveira Santos ◽  
Celso Darío Ramos ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Prostate Cancer ◽  
Lung Cancer ◽  
Bone Metastases ◽  
Bone Pain ◽  
Poor Response ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Unknown Primary ◽  
Breast And Prostate Cancer

CONTEXT: More than 50% of patients with prostate, breast or lung cancer will develop painful bone metastases. The purpose of treating bone metastases is to relieve pain, reduce the use of steroids and to maintain motion. OBJECTIVE: To evaluate the use of samarium-153-EDTMP (153Sm-EDTMP) for the treatment of bone pain secondary to metastases that is refractory to clinical management. TYPE OF STUDY: Retrospective. SETTING: Division of Nuclear Medicine, Universidade Estadual de Campinas (Unicamp). METHODS: Fifty-eight patients were studied (34 males) with mean age 62 years; 31 patients had prostate cancer, 20 had breast cancer, three had lung cancer, one had lung hemangioendothelioma, one had parathyroid adenocarcinoma, one had osteosarcoma and one had an unknown primary tumor. All patients had multiple bone metastases demonstrated by bone scintigraphy using 99mTc-MDP,and were treated with 153Sm-EDTMP. Response to treatment was graded as good (pain reduction of 50-100%), intermediate (25-49%) and poor (0-24%). RESULTS: All patients showed good uptake of 153Sm-EDTMP by bone metastases. Among the patients with prostate cancer, intermediate or good response to therapy occurred in 80.6% (25 patients) and poor response in 19.4% (6). Among the patients with breast cancer, 85% (17) showed intermediate or good response to therapy while 15% (3) showed poor response. All three patients with lung cancer showed poor response to treatment. The lung hemangioendothelioma and unknown primary lesion patients showed intermediate response to treatment; the osteosarcoma and parathyroid adenocarcinoma patients showed good response to treatment. No significant myelotoxicity occurred. DISCUSSION: Pain control is important for improving the quality of life of patients with advanced cancers. The mechanism by which pain is relieved with the use of radionuclides is still not yet completely understood, however, the treatment is simple and provides a low risk of mielotoxicity. CONCLUSION: Treatment with 153Sm-EDTMP can control the pain secondary to bone metastases effectively in most patients with breast and prostate cancer without significant side effects.

Molecular Profiling of Carcinoma of Unknown Primary and Correlation With Clinical Evaluation

2008 ◽  
Vol 26 (27) ◽  
pp. 4442-4448 ◽  
Author(s):  
Gauri R. Varadhachary ◽  
Dmitri Talantov ◽  
Martin N. Raber ◽  
Christina Meng ◽  
Kenneth R. Hess ◽  
...  
Keyword(s):  
Colon Cancer ◽  
Poor Response ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Unknown Primary ◽  
Cancer Center ◽  
Carcinoma Of Unknown Primary ◽  
University Of Texas ◽  
Pathologic Features ◽  
Tissue Of Origin

Purpose To evaluate the feasibility of a 10-gene reverse transcriptase polymerase chain reaction assay to identify the tissue of origin in patients with carcinoma of unknown primary (CUP) site. Patients and Methods Diagnostic biopsy formalin-fixed, paraffin-embedded (FFPE) specimens from 120 patients with CUP were collected retrospectively from Sarah Cannon Research Institute, Nashville, TN, and prospectively from The University of Texas M. D. Anderson Cancer Center, Houston, TX. Tissue of origin assignments by the assay were correlated with clinical and pathologic features and with response to therapy. Results The assay was successfully performed in 104 patients (87%), and a tissue of origin was assigned in 63 patients (61%). In the remaining 41 patients (39%), the molecular profiles were not specific for the six tumor types detectable by this assay. The tissues of origin most commonly identified were lung, pancreas, and colon; most of these patients had clinical and pathologic features consistent with these diagnoses. Patients with lung and pancreas profiles had poor response to treatment. Patients with colon cancer profiles had better response to colon cancer–specific therapies than they did to empiric CUP therapy with taxane/platinum regimens. Patients with ovarian cancer profiles were atypical, with widespread visceral metastases and a paucity of overt peritoneal involvement. Conclusion This gene expression profiling assay was feasible using FFPE biopsy specimens and identified a putative tissue of origin in 61% of patients with CUP. In most patients, the assigned tissue of origin was compatible with clinicopathologic features and response to treatment. Prospective studies in which assay results are used to direct therapy are indicated.

scholarly journals Novel biomarkers for the detection of prostate cancer

2016 ◽  
Vol 9 (2_suppl) ◽  
pp. 3-10 ◽  
Author(s):  
Niels Asger Jakobsen ◽  
Freddie Charles Hamdy ◽  
Richard John Bryant
Keyword(s):  
Prostate Cancer ◽  
Disease Burden ◽  
Treatment Options ◽  
Specific Antigen ◽  
Response To Therapy ◽  
Response To Treatment ◽  
The Novel ◽  
Novel Biomarkers ◽  
Clinically Significant

Prostate-specific antigen (PSA) is widely used as a biomarker in the detection of prostate cancer and for decision making regarding treatment options, response to therapy, and clinical follow-up. Despite its widespread use, it is well recognised that PSA has suboptimal performance as a screening tool due to poor specificity, resulting in high negative biopsy rates and potential ‘over-diagnosis’ and ‘over-treatment’ of clinically insignificant cancers. In particular, PSA does not reliably distinguish either cancer from benign prostatic conditions, or ‘clinically significant’ from ‘indolent cancers’, and it is inaccurate in predicting disease burden and response to treatment. There is an urgent demand for novel biomarkers to address these clinical needs. This article provides an update on the novel candidate biomarkers in development, which have shown potential for improving the detection of clinically significant cases of this malignancy.

Vulval lichen planus–lichen sclerosus overlap

2018 ◽  
Vol 29 (10) ◽  
pp. 1017-1023 ◽  
Author(s):  
Matthew Howard ◽  
Anthony Hall
Keyword(s):  
Lichen Planus ◽  
Lichen Sclerosus ◽  
Poor Response ◽  
Clinical Findings ◽  
Response To Treatment ◽  
Prolonged Treatment ◽  
Dramatic Improvement ◽  
Labia Majora ◽  
Erosive Lichen Planus ◽  
Oral Corticosteroids

Vulval lichen planus–lichen sclerosus overlap is an emerging observation. Few clinical reports exist with no reviews of literature. We present a focused update of this phenomenon and discuss a clinical case. We report a 63-year-old woman with a 20-year history of ulcerative vulvo-vaginitis, initially diagnosed as benign mucous membrane (cicatricial) pemphigoid. This led to prolonged treatment with oral corticosteroids with minimal improvement in symptoms. Subsequent complications of long-term use of systemic corticosteroid ensued. A clinico-pathological diagnosis of severe erosive lichen planus was made on clinical findings and on non-specific biopsy changes of ulceration and inflammation. Treatment with topical clobetasol propionate 0.05% ointment twice daily led to dramatic improvement of ulceration, easing of discomfort and marked improvement in quality of life. Clinical examination revealed Wickham’s striae on the labia majora supporting the diagnosis. Six years after commencement of topical clobetasol, white plaques were noticed on the labia majora, perineum and peri-anal region consistent with lichen sclerosus, confirmed by repeat vulval skin biopsy and on vulvectomy. This case highlights the challenge of diagnosis of extensive vulvo-vaginal ulceration and the necessity to re-examine a previous diagnosis if there is poor response to treatment.

scholarly journals Pharmacogenetic Aspects of Type 2 Diabetes Treatment

2020 ◽  
Vol 5 (3) ◽  
pp. 13-23
Author(s):  
N. O. Pozdnyakov ◽  
I. N. Kagarmanyan ◽  
A. E. Miroshnikov ◽  
E. S. Emelyanov ◽  
A. A. Gruzdeva ◽  
...  
Keyword(s):  
Gene Polymorphism ◽  
Poor Response ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Pharmacokinetic Parameters ◽  
Average Decrease ◽  
Kcnj11 Gene ◽  
Personalized Approach ◽  
A Minor ◽  
Pparγ Gene

In this article, we analyze the role of different variants of the KCNJ11, TCF7L2, SLC22A1, SLC22A3, CYP2C9, CYP2C8, PPARγ genes polymorphisms in efficacy of diabetes mellitus pharmacotherapy. T allele of the KCNJ11 rs2285676 gene polymorphism and G allele of KCNJ11 rs5218 gene polymorphism are associated with the response to IDPP-4 therapy; the presence of KCNJ11 gene rs5210 polymorphism A allele is a predictor of poor response. The effect of rs7903146 polymorphism of TCF7L2 gene was evaluated on the response to treatment of patients taking linagliptin. Linagliptin significantly reduced HbA1c levels for all three rs7903146 genotypes (CC: –0.82 %; CT: –0.77 %; TT: –0.57 %). A significantly smaller effect of therapy was observed with the genotype with ТТ. The rs622342 polymorphism of SLC22A1 gene was studied in effectiveness of metformin. The researches demonstrated that carriers of variant AA had an average decrease of HbA1c of 0.53 %, heterozygous – decrease of 0.32 %, and carriers of a minor variant of SS had an increase of 0.2 % in the level of HbA1c. A significant effect of CYP2C9 polymorphisms on the pharmacokinetic parameters of PSM was noted. When studying the kinetics of glibenclamide, it was found that carriage of the allele *2 significantly reduces glibenclamide metabolism: homozygous carriers had clearance 90 % lower than homozygous carriers of the wild variant. The studies confirmed the association of the allelic variants of Thr394Thr and Gly482Ser of PPARγ gene with higher efficacy of the rosiglitazone. The data obtained from the analysis of the association of the Pro12Ala polymorphism of PPARγ gene and the response to therapy is contradictory. Thus the personalized approach, based on the knowledge of polymorphism options, will allow choosing the most effective drug with transparent kinetics for each individual patient.

Polymorphisms in the Promoter Region of the Mcl-1 Gene Are Associated with Ex Vivo Cytotoxicity but Not with Response to Treatment in Advanced Chronic Lymphocytic Leukemia (CLL).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4991-4991
Author(s):  
Neus Villamor ◽  
Silvia Marce ◽  
Francesc Bosch ◽  
Anna Ferrer ◽  
Patricia Pérez-Galán ◽  
...  
Keyword(s):  
Promoter Region ◽  
Ex Vivo ◽  
Poor Response ◽  
Allelic Frequency ◽  
Response To Therapy ◽  
Lymphocytic Leukemia ◽  
Response To Treatment ◽  
Rna Expression ◽  
Wild Type

Abstract High levels of Mcl-1 have been related to a poor response to therapy in patients with CLL. Genetic polymorphisms in the promoter region of the Mcl-1 gene consisting in insertions of 6 (+6) or 18 (+18) bp have been described in 30% to 60% of patients with CLL. The biological and clinical translation of such polymorphisms is controversial. We have analysed this issue in a cohort of newly diagnosed patients with CLL (14 F/ 46 M; 80% in B or C Binet’s stage), treated with a combination of fludarabine (F), cyclophospamide (C) and mitoxantrone (M). Polymorphisms were correlated with: Mcl-1 RNA expression as assessed by quantitative PCR, ex vivo cytotoxicity against F, C, and M, alone or in combination, using the MTT assay, and clinical response to treatment. The frequency of the wild type (wt), +6, and +18 allel was 56%, 12%, and 32 %, respectively (table). Twenty-one of 60 (35%) patients were homozygous for wt allel. Quantification of Mcl-1 RNA expression was performed in 18 patients. Wt/wt patients (n=6) tended to have lower Mcl-1 gene expression than the remaining patients (1.0 AU ±0.4 vs 2.2 AU ±2.8). Ex vivo cytotoxicity was different according to the Mcl-1 genotypes for F, M, F+M, and C+F, but not for FCM. The highest cytotoxicity was observed in patients with +18/+18 genotype (n=7) while those with wt/wt (n=11) presented intermediate cytotoxicity and those with other genotypes (n=17) had the lowest cytotoxicity. Twenty-eight patients achieved CR, 22 PR, and 5 failed to respond. No relationship was found between Mcl-1 polymorphisms and response to therapy (table). Interestingly, however, the ex vivo response to F (58.2% ±16 vs 45.7% ±16, p=0.05) and F+M (87% ±9 vs 76% ±15.5, p=0.04) was greater in the 13 patients who achieved MRD-negative CR. After a median follow up of 28 months (range: 6–53), 16 of 38 patients have progressed. No relationship was found between Mcl-1 polymorphism and progression after treatment. In summary, in this series of patients with advanced CLL homogeneously treated the frequency of insertions in the promoter region of Mcl-1 gene was high. Although Mcl-1 polymorphisms were associated with the ex vivo response to antileukemic drugs, they did not correlate with response to treatment and disease progression. Distribution of Mcl-1 Polymorphisms and Response to Treatment Allel Allelic Frequency Genotype Patients CR (MRD- CR) wt/wt 21 (35%) 9 ( 4 ) wt 68 (56%) +6/+6 2 ( 3%) 0 ( 0 ) +18/+18 7 (12%) 4 ( 3 ) +6 14 (12%) wt/+6 6 (10%) 4 ( 2 ) wt/+18 20 (33%) 9 ( 3 ) +18 38 (32%) +6/+18 4 ( 7%) 2 ( 1 )

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