Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.3.16 (calcineurin)
17,112 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present a small review of lichen sclerosus in women and an update on the newest knowledge, e.g. on calcineurin inhibitors as a choice of treatment. The goal is to put more focus on the disease in Denmark because it is so often diagnosed only with a great delay. We further emphasize the importance of follow-up on these patients, due to the risk of cancer, the great influence of the disease on quality of life and the risk of structural changes in the vulva region.
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PMID:[Lichen sclerosus--a neglected disease]. 2209 15

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.
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PMID:Diagnosis and treatment of lichen sclerosus: an update. 2332 78

The topical calcineurin antagonist tacrolimus plays an important role in the treatment of different forms of eczema because of its favorable risk profile. In addition, different off-label indications have been clinically tested where tacrolimus ointment has achieved clinical improvement. This article discusses off-label treatment of vitiligo, seborrheic dermatitis, steroid rosacea, perioral dermatitis, rosacea and lichen sclerosus.
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PMID:[Off-label indications for topical tacrolimus]. 2415 Aug 24

Lichen sclerosus (LS) is an inflammatory dermatosis with a predilection for the anogential skin. Vulvar LS can be a debilitating disease, causing pruritus and pain, and it carries the potential for atrophy, scarring, and significant functional impairment. Recently, many advances have been made regarding the etiology and natural history of the disease process; however, much debate still exists regarding the most advantageous medical and surgical management of this disorder. In an effort to provide a comprehensive review on current vulvar LS literature, the following three controversies will be discussed: (1) optimal disease treatment, (2) theories behind LS's oncogenicity and treatments for minimizing malignancy, and (3) the value of surgical treatment for LS. Ultra-potent topical corticosteroids (TCSs) are the first-line treatment for vulvar LS, while topical calcineurin inhibitors (TCIs) remain second-line agents for patients for whom TCS treatment resulted in incomplete resolution of symptoms or adverse events. Due to the relapsing nature of the disease, long-term maintenance therapy is often required. In addition, recent advances have contributed to the understanding of the association between LS and squamous cell carcinoma (SCC). While the exact mechanism responsible for LS-associated SCC is not known, immune dysregulation and inflammation may play an important role; therefore, successful treatment of LS should be directed towards alleviation of symptoms and reversal of the underlying histopathologic changes. Patients with LS-associated malignancy, as well as patients who need correction of functionally restrictive, scarring processes, can successfully undergo surgical intervention with tissue conservation.
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PMID:Influence of treatments on prognosis for vulvar lichen sclerosus: facts and controversies. 2416 Feb 87

Lichen sclerosus (LS) is a lymphocyte-mediated inflammatory dermatosis with a characteristic location (85-98%) in the anogenital region. The authors point out the main features in the epidemiology and clinical presentation of the disease and the possible approach to neoplastic development. Expanded differential diagnosis of LS sparked not only dermatologists but also gynecologists, urologists and GPs. Points are the chronic course of the disease and resistance to therapy. Discuss the results of the treatment of LS with less potent topical corticosteroids, calcineurin inhibitors, phototherapy and photodynamic therapy, surgery.
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PMID:[Lichen sclerosus--clinical and therapeutic aspects]. 2491 40

Chronic graft-versus-host disease (cGVHD) is one of the main late complications of allogeneic hematopoietic stem cell transplant and a major contributor to the mortality and morbidity in surviving recipients. Skin is the most common involved organ in cGVHD and may mimic a wide spectrum of dermatological conditions in its clinical and histopathologic manifestations. Some of the commonly simulated diseases are scleroderma, morphea, and lichen sclerosus. Chronic GVHD simulating eosinophilic fasciitis (EF) is relatively rare, frequently presenting with skin induration, a typical "peau d'orange" appearance, peripheral blood eosinophilia, myalgia, arthralgia, and arthritis leading to joint contractures in severe cases.Diagnosis is based on clinical manifestations and histopathology. Treatment is challenging because most cases are refractory to first-line therapy of glucocorticoids and calcineurin inhibitors (CNIs), and there is no standard second-line therapy.We report a comprehensive review of literature on all reported cases of CGVHD presenting as EF. We also describe an additional interesting case of cGVHD presenting as EF that was resistant to traditional therapy of high-dose glucocorticoids and cyclosporin A, but showed complete resolution of skin manifestations after addition of imatinib.Chronic GVHD presenting as EF is a rare variant of sclerodermatous cGVHD. Diagnosis is difficult, and treatment of cGVHD mimicking EF remains a therapeutic challenge because of obscure pathogenesis and poor response to traditional immunosuppressive medications. Emerging insights into the pathogenesis of cGVHD have resulted in the development of novel targeted therapies, which may improve outcomes and should be attempted in this subset of the disease. Larger studies are warranted to substantiate these preliminary findings.
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PMID:Chronic graft-versus-host disease presenting as eosinophilic fasciitis: therapeutic challenges and an additional case. 2571 Aug 60

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis of unknown etiology. Extragenital involvement is uncommon and commonly affects the neck, shoulders, and upper portion of the trunk. It is predominant in women with a male-to-female ratio of 1:6 and occurs at any age. Linear pattern along the lines of Blaschko are seen. There is no cure for LSA. Topical corticosteroids and calcineurin inhibitors, such as tacrolimus, pimecrolimus, PUVA antimalarial agents, and topical retinoids have been tried with varying results. A case of a 33-year-old man with LSA over right lower limb along the lines of Blasckho is reported here.
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PMID:Extragenital lichen sclerosus et atrophicus along the lines of Blaschko. 2650 Aug 67

Female lichen sclerosus is a chronic inflammatory dermatitis, with a predilection for the anogenital area, which in some cases can become seriously distorted (atrophy of the labia minora, phimosis, introital stenosis, etc.). Most cases are diagnosed in postmenopausal women, but it can affect women of any age. Lichen sclerosus is usually a pruriginous condition, although it can also be asymptomatic. It is associated with an increased risk of vulvar cancer, even though it is not a premalignant condition itself. The true precursor of cancer associated with lichen sclerosus is vulvar intraepithelial neoplasia, differentiated type. The diagnosis is usually clinical, but in some cases a biopsy can be performed, especially to exclude vulvar intraepithelial neoplasia or cancer. The treatment of lichen sclerosus aims at controlling the symptoms, stopping further scarring and distortion and reducing the risk of cancer. The gold standard in treatment is ultra-potent topical steroids (clobetasol propionate). Second-line treatments include calcineurin inhibitors, retinoids, and immunosuppressors. Surgery is used only for the treatment of complications associated with lichen sclerosus. Follow-up must be kept indefinitely.
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PMID:Lichen sclerosus in women: a review. 2866 5

Noninfectious penile lesions are classified by clinical presentation as papulosquamous (e.g., psoriasis), inflammatory (e.g., lichen sclerosus, lichen nitidus, lichen planus), vascular (e.g., angiokeratomas), or neoplastic (e.g., carcinoma in situ, invasive squamous cell carcinoma). Psoriasis presents as red or salmon-colored plaques with overlying silvery scales, often with extragenital cutaneous lesions. Lichen sclerosus presents as a phimotic, hypopigmented prepuce or glans penis with a cellophane-like texture. Lichen nitidus usually produces asymptomatic pinhead-sized, hypopigmented papules. The lesions of lichen planus are pruritic, violaceous, polygonal papules that are typically systemic. Angiokeratomas are typically asymptomatic, well-circumscribed, red or blue papules, often with annular or figurate configurations. Carcinoma in situ should be suspected if there are velvety red or keratotic plaques on the glans penis or prepuce, whereas invasive squamous cell carcinoma presents as a painless lump, ulcer, or fungating mass. Some benign lesions, such as psoriasis and lichen planus, may mimic carcinoma in situ or invasive squamous cell carcinoma. Biopsy is indicated if the diagnosis is in doubt or neoplasm cannot be excluded. The management of benign noninfectious penile lesions usually involves observation, topical corticosteroids, or topical calcineurin inhibitors. Neoplastic lesions generally warrant organ-sparing surgery.
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PMID:Noninfectious Penile Lesions. 2936 26

Off-label prescribing is a common practice in dermatology, particularly when uncommon dermatologic diseases have limited or no approved treatment options. Topical calcineurin inhibitors are approved for the treatment of eczema, and their anti-inflammatory, immunomodulatory, and steroid-sparing effects make them an attractive therapeutic option for a wide variety of other dermatologic diseases. This review summarizes and qualifies the available evidence supporting the clinical effectiveness of tacrolimus ointment and pimecrolimus cream in non-eczema indications. There is high-quality evidence supporting the effectiveness of topical calcineurin inhibitors in multiple dermatological disorders including vitiligo; psoriasis of the face, folds, and genitals; seborrheic dermatitis; chronic hand dermatitis; contact dermatitis; oral lichen planus; lichen sclerosus; morphea; and cutaneous lupus erythematosus. Lower-quality evidence suggests they may be considered as an option in many other cutaneous disorders.
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PMID:Off-Label Use of Topical Calcineurin Inhibitors in Dermatologic Disorders. 3147 36


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