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impossiblephilosopherdreamer · 5 years ago

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How Ayurveda Helps to Treat Scleroderma

Scleroderma known as range of conditions in which the connective tissues harden and tighten the skin. It gets worse slowly and gradually. It is a progressive disease which takes long term for progressing as disease. Scleroderma is known as Systemic sclerosis. This disease is known as autoimmune disorder. In this condition immune system of the body attacks the body.

It is considered as rheumatic diseases that are known to affect the connecting and supporting structures of the body like bones, muscles, ligaments and tendons and joints of the body. This result in excessive production of protein collagen, this protein is known as the basis of connective tissue. Due to overproduction of collagen it results as thickening and scarring of the tissue. This disorder is just not restricted to your skin only it can affect other parts of your body also like muscles, heart, lungs, kidneys, blood vessels and digestive system.

Types of Scleroderma

There are two types of Scleroderma

Systemic scleroderma Localized scleroderma

1. Systemic Scleroderma

In Systemic scleroderma the whole body gets affected like kidneys, lungs, esophagus and heart the internal organs of the body including blood also. In Systemic scleroderma it further divided into 2 main types:-Diffuse systemic sclerosis and Limited Cutaneous Systemic Sclerosis Syndrome.

Symptoms of the Systemic scleroderma

In early stages of the disease your skin thickens and shiny areas developing around your bony areas, fingers, mouth, nose and thumb. Joint pain, shortness of breath, white lumps under the skin and calcium deposists. Pain in joint, dry cough, bloating in abdominal after meals and difficulty in swelling are the symptoms of Systemic scleroderma.

Causes of the Systemic Scleroderma

Overproduction of collagen is the main casue behind the condition of Systemic scleroderma, as collagen is the main protein that builds up all of your tissues in the body.

2. Localized Scleroderma

In this type of scleoderma it does not affect the internal organs of the body.It further divides into linear and morphea scleroderma.

Risk factors of Systemic Sclerosis

People who went through radiation and some chemotherapy drugs like bleomycin.

Being Female

Race:-Sometimes it may be the risk factor that you belong to a particular race like being belonging from Native-American or African-American but it’s not necessary

Complications

Complications of Scleroderma vary from person to person their severity ranges from mild to fatal (death).As there is risk of cancer also associated with it.

Following are the some of the complications face by the patient

Skin : The skin becomes hard due that movement in the skin tightens and swelling occurs in both hands,fingers around the face and mouth. Even movements in the muscles and joints of the body also get affected due to scleroderma.

Lungs : Complications in lungs can be the result due Scleroderma. This leads to various problems of lungs. Due to high blood pressure in the artery that carries blood from the heart to the lungs gets affected, hence can cause permanent damage to the lungs. Right ventricle of the heart may lead to get demolished.

Kidneys : Scleroderma can also damage kidneys ,which leads to excess protein in the urine, rise in the levels of blood pressure occurs. In this case symptoms like problem in vision, breathlessness, swelling of the legs and feet, headache and production of urine may get reduced.

Heart : Abnormal heartbeats and congestive heart failure may be the result from damaging the heart tissue. Inflammation or lining around the heart known as pericarditis is the condition prevails due to Scleroderma. This condition causes various ailments in the heart and causes chest pain.

Thyroid : Hyprothyoidism may occur as thyroid becomes under active, hence hormonal change takes place and slow down the metabolism.

Sexual performance: It affects the male sexuality and erectile dysfunction takes place in males and in females by constricting the woman’s vaginal opening sexual lubrication gets decreased in females due to scleroderma.

Digestion :The proper working of intestines get halted as a result bloating ,constipation, indigestion takes place. The esophagus can not work properly as moving liquids and solids become difficult due to Scleroderma.

Some of the above mentioned complications of lung, kidney, heart can be fatal also due to scleroderma.

Ayurvedic Herbs for Scleroderma

Ashwagandha (Withania somnifera) :Vatt and Pitta doshas can be pacified Ashwgandha herb ,this herb contains anti-inflammatory properties that can eradicate many ailments of the body.

Gotukola (Centella asiatica) : Gotukola helps in pascifying the Pitta dosha in our body.

Gandhak (Purified Sulphur) : Gandhak is having like anti-microbial, anti-inflammatory, anti-viral and anti-bacterial properties, these properties helps to treat Scleroderma.

Boswellia Serrata (Shallaki): Shallaki as a herb has great ablity to fight with inflammation by having properties of anti-inflammation.

#scleroderma #skin treatment #skin disorders

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pharmaphorumuk · 5 years ago

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Kadmon preps filing for GVHD drug belumosudil after phase 3 win

New York biotech Kadmon is on course to file for FDA approval of belumosudil for chronic graft versus host disease (GVHD) after it hit the mark in a pivotal trial.

GVHD is a common and often fatal complication that can follow a bone marrow transplant, which occurs when the donated cells mount an immune response against the transplant recipient’s tissues and organs.

In the 132-patient ROCKstar study, almost three quarters of chronic GVHD patients treated with a daily oral dose of belumosudil (KD025) saw an improvement in symptoms that lasted for at least six months, and Kadmon says it will now move ahead with an FDA filing before year-end.

The patients had chronic GVHD symptoms despite prior treatment with at least two lines of systemic therapy.

Current options are steroids and immunosuppressant drugs like cyclosporine, as well as newer therapies like Johnson & Johnson’s Imbruvica (ibrutinib) and Incyte’s Jakafi (ruxolitinib) that are used for the acute stages of GVHD.

There are roughly 5,000 bone marrow transplants carried out every year in the US, often used to treat blood cancers like leukaemias, and it is estimated that between 20% and 80% of people having a donor transplant will develop some degree of GVHD.

Belumosudil – a Rho-associated protein kinase-2 (ROCK2) inhibitor that reduces immune mediators like IL-17 and IL-23 – would be a first-in-class therapy for GVHD if approved by the FDA, says Kadmon.

It could also provide a much needed additional line of treatment for the approximately 14,000 people in the US who live with the disease, and give Kadmon a second product to sit alongside its Clovique (trientine hydrochloride) therapy for genetic disorder Wilson’s disease.

In ROCKstar, a 200mg dose of the drug given once-daily achieved an overall response rate of 73% of patients, not dissimilar from the 74% rate seen in a group taking it at 200mg twice-daily.

For Kadmon, it’s encouraging that the response rate has climbed from an interim analysis reported earlier this year, and that after almost half (49%) of responders have maintained the effect on symptoms for at least 20 weeks.

Taken together, those results suggest belumosudil’s effects are long-lasting and not wearing off over time – important considerations for a drug designed to treat a chronic condition.

The FDA has agreed to review the drug under its Real-Time Oncology Review (RTOR) pilot programme, which aims to speed up access to promising new cancer drugs whilst still ensuring they are safe and effective.

The regulator considers drugs for the RTOR programme if they look likely to be significantly better than approved therapies, have straight forward study designs, and have endpoints that are easily interpreted. One-year follow-up data from ROCKstar will be fed into the rolling review later this year.

Belumosudil has both breakthrough and orphan drug designations for chronic GVHD, and is also being studied in a mid-stage trial in diffuse cutaneous systemic sclerosis, a subtype of rare disease systemic scleroderma that causes skin hardening and organ damage.

UK biotech Redx Pharma also has a ROCK2 inhibitor in development called RXC007, but is focusing on developing it for fibrotic diseases including idiopathic pulmonary fibrosis (IPF) and non-alcoholic steatohepatitis (NASH).

The post Kadmon preps filing for GVHD drug belumosudil after phase 3 win appeared first on .

from https://pharmaphorum.com/news/kadmon-preps-filing-for-gvhd-drug-belumosudil-after-phase-3-win/

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juniperpublishersjojdc · 6 years ago

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Improvement in Scalp Dermatomyositis with Platelet-Rich Plasma-Juniper Publishers

Authored by Hosking AM

Abstract

Dermatomyositis (DM) is an inflammatory myopathy with hallmark cutaneous findings, including heliotrope rash, Gottron’s papules, as well as photodistributed erythema and/or poikiloderma. Scalp DM (SDM) presents as erythematous plaques with overlying scale, associated with severe burning pruritus. Due to inflammation, it may also present with non-scarring alopecia. First-line treatment for SDM includes intralesional corticosteroids; however, to date, no therapy has proven effective. Platelet-rich plasma (PRP) is an emerging treatment modality for a variety of disease entities including arthritis, oral surgery, and skin rejuvenation. Recent reports demonstrate PRP may have a promising role in hair regrowth. Herein, we report a case of refractory SDM with significant clinical improvement after treatment with subdermal PRP injections of the scalp. We also used optical coherence tomography (OCT) as a non-invasive imaging methodology to longitudinally monitor hair growth over the 18-week PRP treatment interval.

Keywords: Dermatomyositis; Scalp; Platelet-rich plasma; Tomography

Abbrevations: DM: Dermatomyositis; OCT: Optical Coherence Tomography; PRP: Platelet-Rich Plasma; SDM: Scalp DM

Introduction

Dermatomyositis (DM) is a systemic, autoimmune, connective tissue disease characterized by chronic muscle inflammation and weakness associated with characteristic cutaneous findings. The disease predominantly affects females compared to males (2:1) and may be associated with occult malignancy [1]. Scalp dermatomyositis (SDM) is seen in 28-82% of patients with dermatomyositis, and presents with diffuse erythema, scale, and poikiloderma of the scalp, with associated non-scarring alopecia [2,3]. Severe, debilitating, burning pruritus is a common symptom, and can help differentiate SDM from other autoimmune causes of hair loss, including lupus and scleroderma [4]. Treatment options include intralesional triamcinolone, as well as systemic corticosteroids, immunomodulators and immunosuppressants to treat the underlying disease. However, SDM is often treatment-resistant, and the above therapies are limited by efficacy, adverse events, and disease recurrence after treatment cessation.

Platelet-rich plasma (PRP) has been used for many years in orthopedics, plastic surgery, and maxillofacial surgery. PRP has recently attracted attention in the field of dermatology for its ability to promote tissue regeneration, wound healing, and potential to stimulate hair growth. PRP consists of the fraction of blood plasma with a higher concentration of platelets (generally two to five-fold higher than baseline) and has been shown to activate human dermal fibroblast proliferation, as well as increase type 1 collagen synthesis [5,6]. Studies have demonstrated an anti-inflammatory effect of PRP, with platelet activation leading to release of inflammatory mediators. Specifically, anti-inflammatory cytokines, such as IL-4 and IL-10, are significantly upregulated in comparison to pro-inflammatory cytokines, such as IL-1β [7]. Herein, we report a case of SDM with significant clinical improvement after treatment with subdermal PRP scalp injections.

Case Report

A 35-year-old, Asian female with a 12-year history of dermatomyositis presents for evaluation of alopecia and scalp dermatitis and pruritus consistent with SDM. For treatment of systemic disease, she has been receiving monthly intravenous immunoglobulin (IVIg) infusions for 11 years, with significant improvement in myalgia and muscle weakness; however, she continues to experience persistent, relapsing alopecia and intractable pruritus with scalp thickening and lichenification. For SDM, the patient was previously treated with intralesional triamcinolone, T/Gel® shampoo, ketoconazole 2% shampoo, topical clobetasol 0.05%, topical calcipotriene 0.005%, topical betamethasone dipropionate 0.064%, and a four-week course of excimer laser, all without significant clinical improvement. The patient’s only current treatment regimen includes monthly intralesional triamcinolone (5mg/mL) injections for the past two years, which have been moderately successful at relieving her symptoms and poorly controlling SDM clinical progression.

Physical examination reveals a well-appearing female with Fitzpatrick type 3 skin with scattered, erythematous, thin plaques with scale over the neck and shoulders, violaceous papules over the extensor aspects of the metacarpophalangeal and interphalangeal joints (Gottron papules) with interphalangeal involvement, ragged cuticles, and trace swelling of all digits. The shawl sign is present with erythema and fine papules with scale over the lateral and posterior neck, as well as the décolletage. She also has faint violaceous patches covering the upper eyelids, consistent with a mild helitrope rash. Examination of the scalp demonstrates diffuse hair thinning with patch-like distribution, atrophic erythematous plaques with hyperkeratotic scale and lichenification, and scattered hyper- and hypopigmented atrophic plaques at the temporal and occipital hairlines. Her eyelashes appear normal; however, there is thinning of bilateral, lateral eyebrows.

In addition to her intralesional corticosteroid injections, we performed three sessions of subdermal injections of PRP (Eclipse Aesthetics, LLC, The Colony, TX, USA) in the scalp over 18 weeks, with an average of 9mL of PRP injected each session. The PRP sample was obtained after centrifuging 22mL of whole blood and extracting the platelet-rich fraction per company protocol. Three weeks after receiving her first session of PRP, there was significant improvement in epidermal thickness, scale, bogginess, and erythema. The patient reported improvement in pruritus and burning sensation as well as decreased shedding.

The patient continued her remarkable improvement after the second session of PRP. However, shortly before her third session the patient experienced a mild flare, with increased pruritus and accompanied by systemic fatigue. Six weeks after her last PRP treatment, the patient appeared “much improved” compared to baseline on a Global Impression of Improvement of Alopecia Scale (from “worse” to “very much improved”) as rated by both patient and physician, and reported persistent, but improved, pruritus and scale over the scalp (Figure 1). The patient was subsequently started on oral plaquenil per rheumatology in an effort to better control systemic disease.

In addition to the above subjective measures, we also used a novel, non-invasive imaging system called optical coherence tomography (OCT) to quantitatively follow our patient’s clinical progress. OCT uses low-coherence interferometry to measure the back-scatter of broad-bandwidth light, producing sub-surface images of the scalp at a depth of approximately 1300 to 1500μm with a resolution of 10μm [8]. This imaging technique may be used to provide quantitative information on hair follicle density and can be used to longitudinally follow the clinical progress of alopecia patients in the outpatient clinical setting.

We used OCT to measure nine different scalp locations, including the frontal hairline, temporal regions, crown and vertex, using a 5mm x 7mm scanning window (Table 1). Although both the investigator and the patient subjectively reported global improvement in hair loss, OCT data was not consistent with these findings. OCT demonstrates increased active hair follicle count on the left parietal (24.5%) with loss in the midline (-9.9%) and on the right (-16.0%). This conflicting data may be consistent with SDM disease course reflecting improvements and flares in disease activity resulting in hair loss in specific scalp areas. Although PRP may improve symptoms of SDM, it does not necessarily induce stable hair regrowth.

Discussion

Involvement of the scalp is a common clinical finding in DM, with one case series reporting scalp involvement in 77.4% patients and alopecia in 87.5% [2]. In the largest histopathologic study of SDM biopsy specimens to date (n=36 specimens from 20 patients), SDM demonstrates interface dermatitis, thickening of the basement membrane, preserved follicular architecture, telangiectasia, and mucin deposition. On horizontal sections, hair counts were most consistent with a diagnosis of chronic telogen effluvium [2]. On trichoscopy, SDM shows enlarged tortuous capillaries, peripilar casts, tufting, and interfollicular scaling. Serologic abnormalities associated with DM include anti-Jo, anti-Ku, and anti-MDA-5 antibodies. The anti-MDA-5 antibody is associated with increased risk of SDM and alopecia [9]. It is possible that in some patients, SDM may be the initial presentation of DM, with the scaly dermatitis often misdiagnosed as sebopsoriasis or seborrheic dermatitis [10].

Systemic treatment of DM often results in a discordance between the response seen in muscle disease and cutaneous disease [11]. Even with remission of muscular disease, cutaneous involvement often persists. Treatment for cutaneous DM may include sun protection, topical or intralesional corticosteroids, topical corticosteroid-sparing agents, such as calcineurininhibitors or retinoid, and/or systemic therapies, such as hydroxychloroquine or low-dose, weekly methotrexate [11]. SDM is particularly challenging to treat and often persists with systemic treatment despite resolution of other cutaneous involvement [12].

Although the mechanism for improvement of SDM’s clinical signs and symptoms with PRP is unknown, the authors feel this topic warrants further investigation. In chronic wound healing, PRP promotes new capillary growth and stimulates epithelization [13]. In orthopedic injuries, PRP has been shown to suppress cytokine release and decrease inflammation, thereby promoting tissue healing [13,14]. We hypothesize that PRP may help attenuate the inflammatory response seen in cutaneous and scalp DM, thus promoting lesion healing. In our experience, autologous PRP injection in the scalp carries little risk with bruising and scalp tenderness being the most commonly reported adverse events. In the literature, rare reports of infection and nerve damage exist, and there is one case of blindness resulting from periocular PRP injections for skin rejuvenation [15]. PRP of the scalp may offer a low-risk complementary or alternative treatment to conventional DM therapy, especially in the case of recalcitrant scalp disease.

Conclusion

SDM can be a debilitating manifestation of DM. Often, conventional therapies do not offer patients relief from the clinical signs and symptoms of disease and are accompanied by unwanted adverse events and disease recurrence after treatment cessation. As evidenced by this case, PRP may be a promising treatment modality for SDM by decreasing inflammation in the scalp and providing transient improvement of clinical signs and symptoms of disease; however, the use of PRP may not necessarily result in hair growth in highly inflammatory scalp conditions.

For more Open Access Journals in Juniper Publishers please click on: https://juniperpublishers.com

For more details JOJ Dermatology & Cosmetics (JOJDC) please click on: https://juniperpublishers.com/jojdc/classification.php

To read more…Full Text in Juniper Publishers click on https://juniperpublishers.com/jojdc/JOJDC.MS.ID.555562.php

#Juniper publishers #Juniper publishers online #Dermatomyositis #Scalp #Platelet-rich plasma

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timclymer · 6 years ago

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Lupus Erythematosus – Skin Disorders

Hyperthyroidism.

Autoimmune thyroid disease of the hypermetabolic type (Graves disease) is reflected by several visible changes. The skin is soft and moist. Scalp hair is thin in diameter, and evidence of diffuse alopecia may be present. Vitiligo occurs in 5% to 10%, and alopecia areata occurs in 1% or 2% of the patients. Onycholysis of the fingernails is sometimes seen. Late in the course of the disease a few patients develop a specific form of clubbing (thyroid acropachy) or pretibial myxedema. The latter consists of thickened, pebbly, skin-colored plaques over the lower anterior shins. These plaques are usually asymptomatic.

Diabetes Mellitus

Patients with diabetes mellitus may develop a number of cutaneous changes. The yellow plaques of necrobiosis lipoidica diabeticorum most often located on the anterior shins, are the most distinct of these changes.Small, hypopigmented, slightly depressed scars (diabetic dermopathy) are also often found on the anterior lower legs. These lesions probably represent obliterative small vessel disease in an area prior to trauma. Bullous lesions somewhat similar in appearance to those of pemphigoid may arise from otherwise normal-appearing skin around the feet and ankles. The cause of these blisters is unknown. Eruptive xanthomas consisting of small, smooth, pink, dome-shaped papules may appear in a sudden shower of lesions in those whose diabetes is grossly out of control. Staphylococcal bacterial infections and candidal yeast infections are seen with increased frequency in diabetic individuals. Diabetes is also associated with a variety of other cutaneous and medical conditions.

Neurofibromatosis

The presence of sharply marginated, light brown patches (cafe-au-lait patches) is often the first clue to the presence of von Recklinghausen’s disease. In late childhood or during the teenage years, axillary freckling and cutaneous neurofibromas begin to develop. The latter are soft, smooth-surfaced, peduncuated papules 0.5 to 2 cm in diameter. They vary in number from several to hundreds and are distributed randomly over the trunk and extremities. Patients with the most severe forms of neurofibromatosis may develop large, grotesque, sack-like plexiform neuromas. A small proportion of these latter lesions undergo sarcomatous degeneration.

Tuberous Sclerosis

The earliest sign of tuberous sclerosis is generally the presence of small, faint white, oval patches (ash leaf spots) scattered randomly on the trunk and extremities. These lesions may be present at birth or may develop in early childhood. One or more thickened skin-colored plaques (shagreen plaques) may appear on the lower back in late childhood. Towards puberty, pinhead-sized, smooth, red, dome-shaped papules (adenoma sebaceum) beginning to emerge on the central portion of the face. The upper lip is spared. Such lesions are easily mistaken for acne papules. Finally, in adult life, small, firm, skin-colored, subungual or periungual fibromas may be noted.

Peutz-Jeghers Syndrome.

This dominantly inherited condition is characterized by the presence of small brown or blackfreckles that appear in clusters on and around the lips and on the fingertips. These pigmentary changes are accompanied by the development of intestinal polyps. Carcinomatous degeneration of these polyps is not common but does occur.

Osler-Weber-Reudu Syndrome

This dominantly inherited condition, also known as hereditary hemorrhagic telangiectasia, is characterized by the presence of small, dusky red, clustered manlles on the fingertips, lips, and mucosal surfaces. These macules are composed of multiple telangiectatic vessels that blanch on pressure. Lesions similar to these may also .occur in patients with the CRST (calcification, Raynaud’s phenomenon, scleroderma, and telangiectasia) variant of scleroderma. Patients with this disease have recurrent episodes of epistaxis and gastrointestinal bleeding. Arteriovenous fistulae are sometimes present in the lungs and liver.

Source by Robin Kumar Lim

from Home Solutions Forev https://homesolutionsforev.com/lupus-erythematosus-skin-disorders/ via Home Solutions on WordPress from Home Solutions FOREV https://homesolutionsforev.tumblr.com/post/185197755610 via Tim Clymer on Wordpress

#Home Solutions FOREV

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homesolutionsforev · 6 years ago

Text

Lupus Erythematosus – Skin Disorders

Hyperthyroidism.

Diabetes Mellitus

Neurofibromatosis

The presence of sharply marginated, light brown patches (cafe-au-lait patches) is often the first clue to the presence of von Recklinghausen's disease. In late childhood or during the teenage years, axillary freckling and cutaneous neurofibromas begin to develop. The latter are soft, smooth-surfaced, peduncuated papules 0.5 to 2 cm in diameter. They vary in number from several to hundreds and are distributed randomly over the trunk and extremities. Patients with the most severe forms of neurofibromatosis may develop large, grotesque, sack-like plexiform neuromas. A small proportion of these latter lesions undergo sarcomatous degeneration.

Tuberous Sclerosis

Peutz-Jeghers Syndrome.

Osler-Weber-Reudu Syndrome

This dominantly inherited condition, also known as hereditary hemorrhagic telangiectasia, is characterized by the presence of small, dusky red, clustered manlles on the fingertips, lips, and mucosal surfaces. These macules are composed of multiple telangiectatic vessels that blanch on pressure. Lesions similar to these may also .occur in patients with the CRST (calcification, Raynaud's phenomenon, scleroderma, and telangiectasia) variant of scleroderma. Patients with this disease have recurrent episodes of epistaxis and gastrointestinal bleeding. Arteriovenous fistulae are sometimes present in the lungs and liver.

Source by Robin Kumar Lim

from Home Solutions Forev https://homesolutionsforev.com/lupus-erythematosus-skin-disorders/ via Home Solutions on WordPress

#Home Solutions Forev

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