scholarly journals Treatment of Hailey–Hailey disease with narrowband phototherapy and acitretin: A case report

2019 ◽  
Vol 7 ◽  
pp. 2050313X1984522 ◽  
Author(s):  
Tatiana Lapa ◽  
Maksym Breslavets

The Hailey–Hailey disease, or familial benign chronic pemphigus, is an autosomal dominant genodermatosis affecting mainly intertriginous areas. It manifests itself in painful blisters, erosions, and cracks and has a chronic course with frequent flares, significantly impacting patients’ quality of life. Presently, there is no cure, but multiple treatment modalities are available. Most evidence supports treatment with topical steroids and antimicrobials. Treatment of recalcitrant disease has been shown to benefit from the addition of oral antibiotics, Naltrexone, systemic retinoids, botulinum toxin A injections, laser treatment, and surgical excision. We describe a case of refractory Hailey–Hailey disease for which most of the abovementioned options failed, but which demonstrated significant improvement following a combination of oral acitretin and narrowband ultraviolet-B phototherapy. To achieve remission, the patient received 30 sessions three times per week with the increment of 20 mJ/cm2 per session and oral acitretin 25 mg PO daily.

2020 ◽  
Vol 12 (3) ◽  
pp. 209-212
Author(s):  
Fahad Al Qooz ◽  
Mohammed Almuharraqi ◽  
Sajjad Salam ◽  
Veena Nagaraj ◽  
Abdullah Darwish

Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare inherited acantholytic dermatosis. It is an autosomal dominant disease affecting the intertriginous areas. HHD has been characterized by flaccid blisters, erosions, and macerations that are limited to flexural (friction-prone) areas. The painful blisters and erosions significantly decrease patients’ quality of life. There are multiple types of therapy related to this disorder. Many of the studies have suggested benefits from steroid therapy in addition to oral antibiotics.


2020 ◽  
pp. 371-377
Author(s):  
Huidi Tchero

AbstractKeloids and hypertrophic scars are abnormal scars, produced by an odd wound-healing response to trauma with a higher incidence in pigmented skin patients. They could be painful or itchy, producing functional and/or cosmetic disability. This chapter focuses on defining scars especially in pigmented skin and the different methods for treating keloids that have been investigated in the literature. Up to the present time, the ideal treatment method has not been defined, although numerous modalities have been designated. We will represent the variances among different scar types and their management methods, concentrating on their indications, modes of action, uses, safety, and efficiency of the following therapies: intralesional steroid, injections of silicone gel/sheet, radiotherapy, photodynamic therapy, electrical stimulation, surgical excision and adjuvant therapy, and cryosurgery. Combination therapies have also shown some value. Still, there is a shortage of randomized clinical trials (RCTs) evaluating such treatment modalities. Management of scar in pigmented (colored) skin is clinically challenging. Therefore, strategic assessment and targeted therapy with focus on deterring recurrence are most needed. The quality of evidence is crucial to select efficient treatments for patients with colored skin, presenting with keloid.


Author(s):  
Sarah Sanad Lotfy ◽  
Yomna Mazid El-Hamd Neinaa ◽  
Nahla El-Sayed Ramzy Ghaly ◽  
Noha Nabil Doghaim

Background: Vitiligo is a chronic cutaneous disease characterized by milky white depigmented patches that leave psychological impact on the patient's quality of life. New treatment modalities have been developed to shorten the duration of treatment of vitiligo with the least side effects. Objective: To evaluate the safety & efficacy of microneedling in combination with narrow band-UVB in the treatment of vitiligo. Patients and Methods: This study included 20 patients with stable vitiligo. They were treated by microneedling (one session every 2 weeks) in combination with narrow band-UVB (3 sessions weekly) for 3 months. Results: The studied patients reported statistically significant degree of clinical improvements as follow;10%reportedgoodimprovement, 25% showe dmoderate improvement, 45% showed mild improvement and 20% showed noimprovement,after3months therapy. The reported side effects were minimal and transient in the form of minor pain, burning sensation and erythema at site of microneedling that disappeared spontaneously within few hours. Conclusion: Microneedling in combination with narrow band-UVB phototherapy could be considered as effective treatment of vitiligo. Microneedling is a tolerable technique, harmless with negligible side effects.


2019 ◽  
Vol 13 (1) ◽  
Author(s):  
Zakir Ali Shah ◽  
Sascha Rausch ◽  
Uzma Arif ◽  
Bilal El Yafawi

Abstract Background Fibrodysplasia ossificans progressiva is an ultrarare autosomal dominant disorder and disabling syndrome characterized by postnatal progressive heterotopic ossification of the connective tissue and congenital malformation of the big toes. Fibrodysplasia ossificans progressiva has worldwide prevalence of about 1 in 2 million births. Nearly 90% of patients with fibrodysplasia ossificans progressiva are misdiagnosed and mismanaged and thus undergo unnecessarily interventions. So far, the number of reported existing cases worldwide is about 700. Clinical examination, radiological evaluation, and genetic analysis for mutation of the ACVR1 gene are considered confirmatory tools for early diagnosis of the disease. Association of fibrodysplasia ossificans progressiva with heterotopic ossification is well documented; however, postsurgical exaggerated response has never been reported previously, to the best of our knowledge. Case presentation We report a case of a 10-year-old Pakistani boy brought by his parents to our institution. He had clinical and radiological features of fibrodysplasia ossificans progressive and presented with multiple painful lumps on his back due to hard masses and stiffness of his shoulders, neck, and left hip. He underwent surgical excision of left hip ossification followed by an exaggerated response in ossification with early disability. Radiological examination revealed widespread heterotopic ossification. All of his laboratory blood test results were normal. Conclusion Fibrodysplasia ossificans progressiva is a very rare and disabling disorder that, if misdiagnosed, can lead to unnecessary surgical intervention and disastrous results of early disability. We need to spread knowledge to physicians and patients’ family members about the disease, as well as its features for early diagnosis and how to prevent flare-up of the disease to promote better quality of life in these patients.


2020 ◽  
Vol 16 ◽  
Author(s):  
Alexander K. C. Leung ◽  
Joseph M. Lam ◽  
Kin Fon Leong ◽  
Kam Lun Hon

Background: Vitiligo is a relatively common acquired pigmentation disorder that can cause significant psychological stress and stigmatism. Objective: This article aims to familiarize physicians with the clinical manifestations, evaluation, diagnosis, and management of vitiligo. Methods: A Pubmed search was conducted in Clinical Queries using the key term "vitiligo". The search included metaanalyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to the English literature. The information retrieved from the above search was used in the compilation of the present article. The information retrieved from the above search was used in the compilation of the present article. Results: Approximately one quarter of patients with vitiligo have the onset before 10 years of age. Genetic, immunological, neurogenic and environmental factors may have a role to play in the pathogenesis. Vitiligo typically presents as acquired depigmented, well-demarcated macules/patches that appear milk- or chalk-white in color. Lesions tend to increase in number and enlarge centrifugally in size with time. Sites of predilection include the face, followed by the neck, lower limbs, trunk, and upper limbs. The clinical course is generally unpredictable. In children with fair skin, no active treatment is usually necessary other than the use of sunscreens and camouflage cosmetics. If treatment is preferred for cosmesis, topical corticosteroids, topical calcineurin inhibitors, and narrowband ultraviolet B phototherapy are the mainstays of treatment. Conclusion: The therapeutic effect of all the treatment modalities varies considerably from individual to individual. As such, treatment must be individualized. In general, the best treatment response is seen in younger patients, recent disease onset, darker skin types, and head and neck lesions. Topical corticosteroids and calcineurin inhibitors are the treatment of choice for those with localized disease. Topical calcineurin inhibitors are generally preferred for lesions on genitalia, intertriginous areas, face, and neck. Narrowband ultraviolet B phototherapy should be considered in patients who have widespread vitiligo or those with localized vitiligo associated with a significant impact on the quality of life who do not respond to treatment with topical corticosteroids and calcineurin inhibitors.


2020 ◽  
Vol 07 ◽  
Author(s):  
Deepika Purohit ◽  
Parijat Pandey

Background:: Cancer is one of the significant causes of morbidity and mortality in patients globally. Lung cancer, among other cancers, remains to be one of the principal causes of deaths in both men and women. The most common type of lung cancer is the non-small-cell lung cancer (NSCLC). Apart from lung cancer, pancreatic cancer is also one of the common cancers currently. Objective:: The assessment of QoL in erlotinib-treated patients can also prove to be very useful in the establishment of this drug as the main treatment option for the patients with pancreatic and lung cancer. Methods:: Therapies that target EGFR-mediated signalling are the latest keystones for treating these two types of cancers. They comprise of two main treatment modalities: firstly, against the extracellular fields, that include monoclonal antibodies and secondly, mechanisms that create interferences in the signalling pathways, primarily the small molecule tyrosine kinase inhibitors. Results:: Quality of life (QoL) is one of the key advantages in erlotinib therapy over chemotherapy. Conclusion:: The present review reports the role of erlotinib in improving the quality of life of cancer patients especially in NSCLC and pancreatic cancers. The studies or trials establishing the relations between erlotinib and QoL are discussed in detail in this review.


Author(s):  
Praveenkumar H. Bagali ◽  
A. S. Prashanth

The unique position of man as a master mechanic of the animal kingdom is because of skilled movements of his hands and when this shoulder joints get obstructed, we call it as Apabahuka (Frozen shoulder), we do not find satisfactory management in modern medical science. Various effective treatment modalities have been mentioned which reverse the pathogenesis, Shodhana is advised initially followed by Shamana therapies. In the present study 30 patients were selected incidentally and placed randomly into two groups A and B, with 15 subjects in each group. Group A received Amapachana with Panchakola Churna, Jambeera Pinda Sweda and Nasya Karma. Group B received Amapachana with Panchakola Churna, Jambeera pinda Sweda and Nasaapana. In both the groups two months follow up was done. Both groups showed significant improvement in the signs and symptoms of Apabahuka as well as the activities of daily livings, thereby improving the quality of life of the patients. Nasya Karma and Nasaapana provided highly significant results in all the symptoms of Apabahuka. In the present study as per the clinical data, Nasaapana is found to be more effective than Nasya Karma.


2020 ◽  
Vol 16 (35) ◽  
pp. 2997-3013
Author(s):  
Kentaro Kogushi ◽  
Michael LoPresti ◽  
Shunya Ikeda

Background: Synovial sarcoma (SS) is a rare, aggressive soft tissue sarcoma with a poor prognosis after metastasis. The objective of this study was to conduct a systematic review of the clinical evidence for therapeutic options for adults with metastatic or advanced SS. Materials & methods: Relevant databases were searched with predefined keywords. Results: Thirty-nine publications reported clinical data for systemic treatment and other interventions. Data on survival outcomes varied but were generally poor (progression-free survival: 1.0–7.7 months; overall survival: 6.7–29.2 months) for adults with metastatic and advanced SS. A high frequency of neutropenia with systemic treatment and low quality of life post-progression were reported. Conclusion: Reported evidence suggests poor outcomes in adults with metastatic and advanced SS and the need for the development of new treatment modalities.


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