scholarly journals Atypical Presentation of Lichen Planopilaris: Presentation of Two Cases and Review

2020 ◽  
Vol 7 (1) ◽  
pp. 1-5
Author(s):  
Nwanneka Okwundu ◽  
Felicia Ekpo ◽  
Jessica Ghaferri ◽  
David Fivenson
Keyword(s):  
Mycosis Fungoides ◽  
Lupus Erythematosus ◽  
Hair Follicles ◽  
Unknown Etiology ◽  
Dermoepidermal Junction ◽  
Lichen Planopilaris ◽  
Interfollicular Epidermis ◽  
History Of ◽  
Caucasian Patients ◽  
Immunomodulatory Therapies

Lichen Planopilaris (LPP) is an uncommon scalp disorder of unknown etiology and prevalence. It is thought to be an autoimmune process triggered by unknown genetic and/or environmental factors that attack hair follicles of the scalp. LPP has been reported to mimic or present in association with various autoimmune diseases and immunomodulatory therapies. We present two atypical case of LPP in Caucasian patients; the first is a patient with generalized pruritus, skin eruptions, and scalp hair loss. Biopsy of the lesions revealed exocytosis of atypical lymphocytes at the Dermo-Epidermal Junction (DEJ) and formation of small Pautrier’s microabscesses in the interfollicular epidermis as well as a robust lymphocytic inflammatory infiltrate with destruction of perifollicular appendages. This led us to the diagnosis of LPP-like Folliculotropic Mycosis Fungoides (FMF). The second case is a patient with a history of Systemic Lupus Erythematosus (SLE) who presented clinically with perifollicular erythema and alopecic patches, with loss of follicular ostia on the frontal and vertex scalp and evidence of follicular tufting. Histologically, she had decreased number of follicles with a peri-infundibular lymphocytic infiltrate and vacuolization at the Dermoepidermal Junction (DEJ) and there was also thickening of the basement membrane highlighted with a periodic acidic Schiff stain. This led to a diagnosis of SLE/LPP overlap. Keywords: Lichen Planopilaris (LPP); Cicatricial; Alopecia; Frontal Fibrosing Alopecia (FFA), Folliculotropic Mycosis Fungoides (FMF), Lichen Planus (LP).

scholarly journals Clinical Patterns of Polyneuropathy Attending in a Tertiary Level of Hospital

2014 ◽  
Vol 15 (1) ◽  
pp. 03-08
Author(s):  
Makhan Lal Paul ◽  
Rezaul Karim Khan ◽  
Md. Zulfiqar Hossain Khan ◽  
Md. Mahabubur Rahman ◽  
Monoj Sinha ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Current Knowledge ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Cross Sectional Study ◽  
Muscle Cramp ◽  
Unknown Etiology ◽  
Cross Sectional ◽  
Female Ratio ◽  
Clinical Pattern ◽  
History Of

A cross sectional study was carried out in the department of Neurology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Shahbag, Dhaka to explore the different clinical pattern of polyneuropathy. A total of 60 subjects were included in this study and mean age of the entire patient was 42.25 year and male and female ratio was 3:1. Out of all patients 26.7% were service holder, 20.0% were student, 20.0% were housewife, 11.7% were unemployed and 33.3% were engaged in some other professions. Maximum 41.7% patients were educated up to graduate and above level followed by 20.0% secondary, 18.3% primary, 16.7% higher secondary and 3.3% were illiterate. Out of all patients 31.7% were smoker, 48.3% were non smoker and 20.0% past smoker. About 35.0% patients had history of betel leaf; nut chewing and no patient had history of alcohol intake. Out of all patients of polyneuropathy 33.3% had diabetic neuropathy, 11.7% had Guillain-Barré syndrome (GBS), 10.0% had chronic inflammatory demyelinating polyneuropathy, similar number had unknown etiology, 6.7% had renal failure, 5.0% had leprosy, Vitamin B 12 deficiency and chronic liver disease(CLD) of each, 3.3% had history of INH drug intake and similar had systemic lupus erythematosus (SLE). Regarding clinical presentation, out of all respondents 83.3% had muscle weakness, similar number had problems with object handling, 66.3% had muscle cramp, 63.3% had impaired standing or gait, 55.0% had distal paresthesia, 41.7% had burning feet, 8.3% had restless legs and 5.0% had stiffness. In the light of this study we conclude that polyneuropathy has wide variety of clinical pattern. The study will enrich our current knowledge and will improve the quality of management of polyneuropathy among the Bangladeshi population.DOI: http://dx.doi.org/10.3329/jom.v15i1.19851 J Medicine 2014; 15: 3-8

scholarly journals Folliculotropic mycosis fungoides treated with topical corticosteroids: A case report and a review of its trichoscopic features

2022 ◽  
Vol 13 (1) ◽  
pp. 111-113
Author(s):  
Linda Manaa ◽  
Yosra Soua ◽  
Marwa Thabouti ◽  
Laila Njim ◽  
Monia Youssef ◽  
...  
Keyword(s):  
Mycosis Fungoides ◽  
Histopathological Examination ◽  
Hair Follicles ◽  
Full Blood Count ◽  
Positive Staining ◽  
Close Monitoring ◽  
Cutaneous Lesions ◽  
Liver And Kidney ◽  
History Of ◽  
Stage Ia

Sir, Folliculotropic mycosis fungoides (FMF) represents 5% of cutaneous lymphomas. It is a rare variant of mycosis fungoides that differs not only by its clinical and histological presentation but also by its prognosis. It is characterized by an infiltrate of atypical lymphocytes in the perifollicular dermis and hair follicles, with or without mucinosis, while epidermotropism may be completely absent. Dermoscopic and trichoscopic features in FMF are variable and not well defined. Herein, we present a unique case of FMF in a female patient with scalp alopecia, which evolved well under topical treatment. We review its trichoscopic findings. A 64-year-old female presented to our dermatology department with a seven-month history of an alopecic plaque on the scalp. No complaints of itching or burning were made. There was a history of arterial hypertension and dyslipidemia, which has been treated by oral medication. A physical examination revealed an erythematous, non-infiltrated, circumscribed alopecia in the frontal region of the scalp 8 cm in size associated with alopecia of the eyebrows (Fig. 1a and 1b). No other cutaneous lesions were evident. There were no other alopecic or infiltrated plaques, no hyperkeratosis or follicular papules, no acneiform lesions. A histopathological examination of a biopsy specimen revealed an epidermis covered with focally parakeratotic hyperkeratosis. The dermis contained a lymphocytic infiltrate in the follicles and the perifollicular areas. The hair follicles were dissociated by Alcian blue-positive edema. Folliculotropic infiltrate showed positive staining for CD3 and CD4. Some lymphocytes were CD20+ (Fig. 3a – 3c). The clinical, histological, and immunohistochemical appearance was consistent with the diagnosis of folliculotropic MF. Further examination showed no extracutaneous involvement. A full blood count and liver and kidney parameters were found to be in the normal range. A thoraco-abdomino-pelvic CT scan was without abnormality. Referring to the WHO/EORTC classification, the patient’s disease was stage IA. We initiated treatment with a high-potency topical corticosteroid with close monitoring. After two months of treatment, improvement was observed (Fig. 1c). No other cutaneous lesions were evident. There were no other alopecic or infiltrated plaques, no hyperkeratosis or follicular papules, no acneiform lesions. A histopathological examination of a biopsy specimen revealed an epidermis covered with focally parakeratotic hyperkeratosis. The dermis contained a lymphocytic infiltrate in the follicles and the perifollicular areas. The hair follicles were dissociated by Alcian blue-positive edema. Folliculotropic infiltrate showed positive staining for CD3 and CD4. Some lymphocytes were CD20+ (Fig. 3a – 3c). The clinical, histological, and immunohistochemical appearance was consistent with the diagnosis of folliculotropic MF. Further examination showed no extracutaneous involvement. A full blood count and liver and kidney parameters were found to be in the normal range. A thoraco-abdomino-pelvic CT scan was without abnormality. Referring to the WHO/EORTC classification, the patient’s disease was stage IA. We initiated treatment with a high-potency topical corticosteroid with close monitoring. After two months of treatment, improvement was observed (Fig. 1c).

PCR-RFLP Detection od PAI-2 Gene Variants: Prevelence in Ethnic Groups and Disease Relationship in patients Undergoing corony Angiography

1997 ◽  
Vol 77 (05) ◽  
pp. 0955-0958 ◽  
Author(s):  
Carole A Foy ◽  
Peter J Grant
Keyword(s):  
Gene Variants ◽  
Genotype Distribution ◽  
Pima Indians ◽  
Significant Difference ◽  
Pcr Rflp ◽  
History Of ◽  
Caucasian Patients ◽  
Clinical Conditions ◽  
Rflp Assay ◽  
Coronary Atheroma

SummaryPAI-2 is a fibrinolytic inhibitor produced predominantly by monocytes. Most PAI-2 is intracellular making study in clinical conditions difficult. Abnormalities in production may be associated with inflammation and fibrinolysis at sites of tissue damage such as the atherosclerotic plaque.PAI-2 gene variants have been described: variant A consists of Asn120, Asn404 and Ser413 and variant B consists of Asp120, Lys404 and Cys413. We designed a PCR-RFLP assay using primers spanning the region containing Asn/Lys404 and Ser/Cys413. Variant B contains an Mwol restriction site. We analysed 302 Pima Indians and 286 healthy Caucasian volunteers. To investigate relationships between genotype and vascular disease we analysed 333 Caucasian patients undergoing coronary angiography.Gene variant B was more common in the Pimas than in Caucasians (p <0.0001). There was no significant difference in genotype distribution between the volunteers and patients. In the patients there was no association between genotype and either a history of MI or extent of coronary atheroma.

Corticosteroids in Inflammatory Bowel Disease patients: a practical guide for physicians.

2020 ◽  
Vol 15 ◽  
Author(s):  
Maria Carla Di Paolo ◽  
Cristiano Pagnini ◽  
Maria Giovanna Graziani
Keyword(s):  
Severe Disease ◽  
Unknown Etiology ◽  
History Of Disease ◽  
Bowel Diseases ◽  
Literature Evidence ◽  
History Of ◽  
Inflammatory Bowel ◽  
Favorable Safety ◽  
Pass Metabolism

: Inflammatory bowel diseases (IBDs) are chronic conditions characterized by unknown etiology and pathogenesis with deregulation of mucosal immunity. Among possible treatments, corticosteroids, already available from the 50’, are still the mainstay of treatment for moderate-severe disease. Nonetheless, the use of steroids is still largely empirical and solid evidence about therapeutic schemes are lacking. Moreover, due to the important side-effects and for the unsatisfactory impact on long-term natural history of disease, the steroid sparing has become an important therapeutic goal in IBD management. Besides conventional steroids, the so called “low bioavailability” steroids, which are steroids with high affinity for peripheral receptors and elevated hepatic first-pass metabolism, have demonstrated efficacy and more favorable safety profile. In the present review of the literature evidence of efficacy and safety of conventional and low bioavailability steroids in IBD patients are evaluated, and practical suggestions for a correct use in clinical practice are presented according to the current clinical guidelines.

scholarly journals AB0420 RISK OF DEVELOPING TYPE 2 DIABETES MELLITUS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1510.2-1510
Author(s):  
L. Kondrateva ◽  
T. Popkova ◽  
E. Nasonov ◽  
A. Lila
Keyword(s):  
Abdominal Obesity ◽  
Lupus Erythematosus ◽  
Antihypertensive Drugs ◽  
Control Group ◽  
Systemic Lupus ◽  
Type 2 Dm ◽  
History Of ◽  
Systemic Rheumatic Diseases ◽  
Very High

Background:Patients with systemic lupus erythematosus (SLE) have higher than in general population prevalence of diabetes mellitus (DM). Hyperinsulinemia is a predictor of developing type 2 DM, however routine measurement of insulin levels for DM risk assessment is uncomfortable in daily clinical practice. International Diabetes Federation recommends the use of patient questionnaires to quickly identify people who may be at a higher risk of DM development.Objectives:To determine the 10-years risk of developing type 2 DM in SLE patients using dedicated questionnaire - Finnish Type 2 Diabetes Risk Assessment Form (FINDRISK) data.Methods:The study included 92 SLE patients without DM (83 women, 9 men, 39 [34; 47] years old). The median disease duration was 6 [2,14] years, SLEDAI-2K was 4[2;8]. SLE pts were treated with glucocorticoids (GC) (89%) and hydroxychloroquine (78%), immunosuppressive drugs (28%) and biological agents (10%). The control group consisted of 88 subjects without systemic rheumatic diseases, inflammatory arthritis or DM, matched by age and sex with SLE patients. Eight items of FINDRISK questionnaire (age, overweight, abdominal obesity, family history of diabetes, physical inactivity, eating habits, history of antihypertensive drugs treatment, history of hyperglycemia) were taken into account to calculate the total risk score (TS). The risk of developing DM within following 10 years is regarded as low (1%) or slightly elevated (4%) with TS ≤11 points, as moderate (17%), high (33%) or very high (50%) with TS ≥12 points.Results:The risk of developing DM was low or slightly elevated in 65 (71%) SLE pts and moderate, high or very high in 27 (29%) pts. The difference was significant compared with the control group, in which 76 (86%) subjects had a low or slightly elevated risk and 12 (14%) had a moderate, high or very high risk (p=0,01). The number of risk factors (4[2;5]) and the median TS of SLE pts (9[5;12] points) were higher than values in control subjects (3[2,4] factors and 6[3;9] points, respectively) (p<0,01 for both). DM risk factors profiles were similar in two groups, except for higher prevalence of abdominal obesity (66% vs 41%, p<0,01) and history of antihypertensive drugs treatment (57% vs 17%, p<0,01) in SLE. There were positive correlations between TS and CRP levels (r=0,25, p=0,02), SLICC (r=0,36, p<0,01), HAQ (r=0,29, p<0,01), and negative correlations between TS and SLEDAI-2K (r= -0,32, p<0,01), glomerular filtration rate by CKD-EPI (r=-0,23, p=0,03). Current GC use had no influence on TS values in SLE.Conclusion:Patients with SLE were more likely than individuals without systemic rheumatic diseases to have a moderate, high and very high risk of developing DM, and therefore, required interventions to prevent the metabolic disease. Increased risk of developing DM was associated with most common traditional factors, especially by abdominal obesity and regular use of antihypertensive drugs that can be considered a kind of equivalent to the presence of hypertension. Curtain contribution of inflammation, lupus activity and irreversible damage index can’t be ignored. Clarification of SLE-specific phenomena in DM pathogenesis requires further research.Disclosure of Interests: :None declared

scholarly journals “It’s not lupus”. A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case

Lupus ◽  
2021 ◽  
pp. 096120332098176
Author(s):  
Sarah J van der Lely ◽  
Jeffrey Boorsma ◽  
Marc Hilhorst ◽  
Jesper Kers ◽  
Joris Roelofs ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Gestational Trophoblastic Disease ◽  
Grand Round ◽  
Placental Site Trophoblastic Tumor ◽  
Trophoblastic Tumor ◽  
Anchoring Bias ◽  
Placental Site ◽  
History Of

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.

“Protenuria in SLE: Is it always lupus?”

Lupus ◽  
2021 ◽  
pp. 096120332098345
Author(s):  
Alessandra Ida Celia ◽  
Roberta Priori ◽  
Bruna Cerbelli ◽  
Francesca Diomedi-Camassei ◽  
Vincenzo Leuzzi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Fabry Disease ◽  
Histological Examination ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Kidney Involvement ◽  
History Of ◽  
Genetic Assay

Proteinuria is one of the most typical manifestations of kidney involvement in Systemic Lupus Erythematosus (SLE). We report the case of a 23-year-old woman with a 6-year-long history of SLE presenting with proteinuria after a three-year remission on hydroxychloroquine. Kidney histological examination showed alterations inconsistent with lupus nephritis and suggestive of hydroxychloroquine toxicity or Fabry disease. The latter was confirmed by genetic assay.

scholarly journals Semiquantified Noncalcified Coronary Plaque in Systemic Lupus Erythematosus

2012 ◽  
Vol 39 (12) ◽  
pp. 2286-2293 ◽  
Author(s):  
ADNAN N. KIANI ◽  
JENS VOGEL-CLAUSSEN ◽  
ARMIN ARBAB-ZADEH ◽  
LAURENCE S. MAGDER ◽  
JOAO LIMA ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Cardiovascular Risk ◽  
Cardiovascular Risk Factors ◽  
Lupus Erythematosus ◽  
Univariate Analysis ◽  
Coronary Plaque ◽  
Systemic Lupus ◽  
History Of ◽  
Noncalcified Plaque

Objective.A major cause of morbidity and mortality in systemic lupus erythematosus (SLE) is accelerated coronary atherosclerosis. New technology (computed tomographic angiography) can measure noncalcified coronary plaque (NCP), which is more prone to rupture. We report on a study of semiquantified NCP in SLE.Methods.Patients with SLE (n = 147) with no history of cardiovascular disease underwent 64-slice coronary multidetector computed tomography (MDCT). The MDCT scans were evaluated quantitatively by a radiologist, using dedicated software.Results.The group of 147 patients with SLE was 86% female, 70% white, 29% African American, and 3% other ethnicity. The mean age was 51 years. In our univariate analysis, the major traditional cardiovascular risk factors associated with noncalcified plaque were age (p = 0.007), obesity (p = 0.03; measured as body mass index), homocysteine (p = 0.05), and hypertension (p = 0.04). Anticardiolipin (p = 0.026; but not lupus anticoagulant) and anti-dsDNA (p = 0.03) were associated with higher noncalcified plaque. Prednisone and hydroxychloroquine therapy had no effect, but methotrexate (MTX) use was associated with higher noncalcified plaque (p = 0.0001). In the best multivariate model, age, current MTX use, and history of anti-dsDNA remained significant.Conclusion.Our results suggest that serologic SLE (anti-dsDNA) and traditional cardiovascular risk factors contribute to semiquantified noncalcified plaque in SLE. The association with MTX is not understood, but should be replicated in larger studies and in multiple centers.

Comorbid depression and ulcerative colitis – is there a connection?

2017 ◽  
Vol 41 (S1) ◽  
pp. S534-S534
Author(s):  
A. Melada ◽  
I. Krišto-Mađura ◽  
A. Vidović
Keyword(s):  
Ulcerative Colitis ◽  
Psychiatric Treatment ◽  
Psychiatric Symptoms ◽  
Specific Treatment ◽  
Unknown Etiology ◽  
Suicidal Thoughts ◽  
Symptoms Of Depression ◽  
Bidirectional Communication ◽  
History Of ◽  
Competing Interest

Ulcerative colitis (UC) is a subset disorder of inflammatory bowel disease (IBD) with chronic course and symptoms such as fatigue, gastrointestinal pain, fever, etc. IBD is associated with psychological manifestations including depression and anxiety. There is an increased number of studies trying to link these comorbidities. The gut-brain axis is regulated by intestinal microbiota and this bidirectional communication including immune, neural, endocrine and metabolic mechanisms may bring us closer to the answer. The following case concerns a 56-year-old patient with history of major depressive disorder who was in continuous psychiatric care and treated with antidepressants. Several years after the beginning of psychiatric treatment, he was hospitalized for diagnostic examination due to subfebrility of unknown etiology, but with no final somatic diagnosis. After two years he was referred to our department and at administration the patient showed symptoms of depression, anxiety, lack of motivation and suicidal thoughts and tendencies. Subfebrility was still present at that time. His psychopharmacotherapy was revised and there was a slight improvement in mood and behaviour. During outpatient follow-ups the symptoms of depression were still prominent and remission was not achieved even with modulation of antidepressant pharmacotherapy. The following year the patient was diagnosed with UC and started specific treatment after he presented with diarrhea in addition to subfebrility. Subsequently his mood improved, suicidal thoughts were diminished and ultimately remission was achieved. This case suggests that only after UC was being treated the psychiatric symptoms also withdrew which implicates that inflammatory mediators were involved in pathogenesis of depression.Disclosure of interestThe authors have not supplied their declaration of competing interest.

scholarly journals An Early Unexpected Immune Thrombotic Thrombocytopenic Purpura Relapse Associated with SARS-CoV-2 Infection: A Case Report and Literature Review

2021 ◽  
pp. 1-5
Author(s):  
Maya Kornowski Cohen ◽  
Liron Sheena ◽  
Yair Shafir ◽  
Vered Yahalom ◽  
Anat Gafter-Gvili ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Response To Therapy ◽  
Intravascular Hemolysis ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
History Of ◽  
Triggering Factor

SARS-CoV-2 has been reported as a possible triggering factor for the development of several autoimmune diseases and inflammatory dysregulation. Here, we present a case report of a woman with a history of systemic lupus erythematosus and antiphospholipid syndrome, presenting with concurrent COVID-19 infection and immune thrombotic thrombocytopenic purpura (TTP). The patient was treated with plasma exchange, steroids, and caplacizumab with initial good response to therapy. The course of both TTP and COVID-19 disease was mild. However, after ADAMTS-13 activity was normalized, the patient experienced an early unexpected TTP relapse manifested by intravascular hemolysis with stable platelet counts requiring further treatment. Only 3 cases of COVID-19 associated TTP were reported in the literature thus far. We summarize the literature and suggest that COVID-19 could act as a trigger for TTP, with good outcomes if recognized and treated early.

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