calcipotriene and Scleroderma--Localized

Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud's phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.

Topics: Administration, Cutaneous; Administration, Oral; Algorithms; Biopsy; Calcitriol; Dermatologic Agents; Diagnosis, Differential; Disease Progression; Drug Therapy, Combination; Eosinophilia; Evidence-Based Medicine; Fasciitis; Glucocorticoids; Humans; Methotrexate; Phototherapy; Practice Guidelines as Topic; Randomized Controlled Trials as Topic; Scleroderma, Localized; Skin; Tacrolimus; Treatment Outcome; United States

Solitary morphea profunda (SMP) is a variant of localized scleroderma (LS). We report the case of a 50-year-old white woman with a history of trauma sustained in an automobile accident who presented with SMP on the right upper arm. We also provide a review of the classification, epidemiology, etiology, diagnostic studies, pathogenesis, physical findings, histopathology, treatment, and prognosis of SMP, along with other important details pertaining to the disease. We also provide a brief overview of LS and morphea profunda (MP).

Topics: Accidents, Traffic; Anti-Inflammatory Agents; Antirheumatic Agents; Arm Injuries; Betamethasone; Calcitriol; Dermatologic Agents; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Hydroxychloroquine; Middle Aged; Prednisone; Scleroderma, Localized

Morphea (localized scleroderma) is a skin disorder with significant morbidity. No consistent recommendations exist for therapy, impeding patient care.. We sought to create an evidence-based therapeutic algorithm.. We reviewed English-language literature using search engines and hand searches for therapeutic interventions in morphea. Results were summarized.. Narrowband ultraviolet B is appropriate for progressive or widespread superficial dermal lesions; broadband ultraviolet A/ultraviolet A-1 is appropriate for widespread or progressive deeper dermal lesions. Systemic treatment with methotrexate, corticosteroids, or both is indicated for deep or function-impairing lesions and rapidly progressive or widespread (severe) disease. Topical treatment with calcipotriene or tacrolimus is supported for limited, superficial, inflammatory lesions. Use of oral calcipotriol, D-penicillamine, interferon gamma, and antimalarials is not supported.. Limitations are publication bias; lack of adequately powered, controlled trials; and no validated outcome measures.. Phototherapy, methotrexate/systemic corticosteroids, calcipotriene, and topical tacrolimus have the most evidence for efficacy in morphea. Treatment works best in inflammatory disease. Disease activity, severity, progression, and depth should play a role in therapeutic decision making.

Topics: Adrenal Cortex Hormones; Algorithms; Anti-Bacterial Agents; Anti-Inflammatory Agents; Calcitriol; Clinical Trials as Topic; Evidence-Based Medicine; Humans; Immunologic Factors; Immunosuppressive Agents; Methotrexate; PUVA Therapy; Scleroderma, Localized; Tacrolimus; Treatment Outcome; Ultraviolet Therapy; Vitamin D

Calcipotriene offers a safe and effective option in the treatment of plaque psoriasis. It helps regulate the abnormal growth and production of keratinocytes, as well as has a number of effects on inflammation seen with psoriasis. When used as monotherapy or in combination with corticosteroids, it may help reduce the adverse effects seen with chronic steroid use. Calcipotriene is currently only indicated for plaque psoriasis; however, it has shown promise for use in a wide range of dermatologic conditions.

Topics: Administration, Cutaneous; Calcitriol; Dermatologic Agents; Humans; Keratinocytes; Lichen Planus; Ointments; Patient Education as Topic; Patient Selection; Psoriasis; Scleroderma, Localized; United States; United States Food and Drug Administration; Vitamin D; Vitiligo

Localized scleroderma refers to a diverse spectrum of disorders that involve fibrosis of the skin. Children are more likely than adults to develop localized forms of scleroderma. This condition may have devastating effects on growth and development such as limb asymmetry, flexion contractures, and psychological disability. The pathogenesis of localized scleroderma is unknown but its possible relation to Borrelial infection is discussed. This article reviews associated laboratory and radiologic abnormalities, and discusses implications for monitoring disease activity. There is no universally effective therapy for this idiopathic condition and therapy is limited. A rationale for treatment based on disease subtype and severity is provided.

Topics: Adrenal Cortex Hormones; Biomarkers; Calcitriol; Child; Dermatologic Agents; Humans; Immunosuppressive Agents; Methotrexate; Scleroderma, Localized; Scleroderma, Systemic; Skin; Ultraviolet Therapy

Morphea and linear scleroderma are characterized by erythema, induration, telangiectasia, and dyspigmentation. There is no universally effective treatment. Oral calcitriol has been beneficial in the treatment of localized and extensive morphea/scleroderma, but the use of topical calcipotriene has not been reported.. The purpose of this study was to evaluate the efficacy and safety of topical calcipotriene 0.005% ointment in the treatment of localized scleroderma.. In a 3-month open-label study, 12 patients aged 12 to 38 years with biopsy-documented active morphea or linear scleroderma applied calcipotriene ointment under occlusion twice daily to plaques for 3 months. The condition of each patient had previously failed to respond to potent topical corticosteroids and, for some patients, systemic medications. Efficacy was assessed at baseline, 1 month, and 3 months. Levels of serum ionized calcium, intact parathyroid hormone, and 1,25-dihydroxyvitamin D and of random urinary calcium excretion were measured.. During the 3-month trial, the condition of all 12 patients showed statistically significant improvement in all studied features. No adverse effects were reported or detected through laboratory monitoring of mineral metabolism.. Topical calcipotriene 0.005% ointment may be an effective treatment for localized scleroderma, but double-blind placebo controlled studies are needed for confirmation.

Topics: Administration, Topical; Adolescent; Calcitriol; Child; Dermatologic Agents; Female; Humans; Male; Ointments; Scleroderma, Localized; Time Factors

Linear morphoea (LM) is a rare fibrosing disorder of the limbs or the face that may cause functional disability and severe aesthetic sequelae. Despite a wide range of therapeutics reported for LM, there is currently a lack of consensus on the optimal therapy. Little is known about the long-term outcome of this disease.. To describe the short- and long-term outcome of a large series of patients with LM acquired in childhood.. A retrospective chart review of 52 paediatric patients with LM seen in our centre during a 20-year span (1990-2010) and a telephone survey in 2011 to assess the long-term outcome of these patients.. Limbs were affected twice as often as the face, with a higher proportion of female patients. Stabilization was obtained after a mean disease duration of 5·4 years. Patients sometimes experienced long stretches of disease quiescence followed by reactivation; 31% of patients reported active disease after 10 years. All but one patient had aesthetic sequelae, and 38% had functional limitations. The effectiveness of methotrexate and systemic corticosteroids was apparent in the short term.. LM needs prolonged monitoring as the disease can have very long periods of quiescence followed by reactivation. The combination of methotrexate and systemic corticosteroids was effective in the early stages of the disease but did not seem to prevent long-standing active disease or relapse in the long term.

Topics: Adolescent; Adrenal Cortex Hormones; Age of Onset; Aminoquinolines; Calcitriol; Child; Dermatologic Agents; Drug Therapy, Combination; Female; Humans; Imiquimod; Male; Methotrexate; Ointments; Phototherapy; Retrospective Studies; Scleroderma, Localized; Tacrolimus; Treatment Outcome; Vitamin A; Vitamin E

Generalized morphea is a subtype of localized scleroderma that lacks systemic manifestations. It is a rare condition in which idiopathic sclerosis of the skin occurs in a widespread manner. We report a 37-year old Thai female who presented with multiple hypopigmented lesions on right leg and right arm with a skin biopsy showing the typical sclerotic change. The patient responded to treatment with topical calcipotriol.

Topics: Administration, Topical; Adult; Biopsy; Calcitriol; Dermatologic Agents; Female; Humans; Scleroderma, Localized; Skin; Treatment Outcome

A 15-year-old patient developed scleroderma en coup de sabre on right temple at 5 years of age. Multiple treatments (3 cycles of intravenous penicillin, topical glucocorticosteroids, topical calcipotriol, and cream PUVA phototherapy combined with topical calcipotriol) produced no improvement. The patient suffered greatly from the psychosocial stigmatization, so that the entire lesion was resected at 14 years of age. One year after the operation a thin non-sclerotic scar was present; tiny lateral areas of sclerosis not included in the operative field were unchanged. The operation greatly improved the patient's daily life. The surgical therapy of scleroderma en coup de sabre offers an interesting therapeutic alternative.

Topics: Administration, Topical; Adolescent; Calcitriol; Dermatologic Agents; Follow-Up Studies; Glucocorticoids; Humans; Injections, Intravenous; Male; Penicillins; PUVA Therapy; Scleroderma, Localized; Time Factors; Treatment Outcome

Topics: Adolescent; Adult; Anti-Inflammatory Agents; Betamethasone; Calcitriol; Dermatologic Agents; Female; Humans; Male; Middle Aged; Scleroderma, Localized; Treatment Outcome

Superficial morphea is a recently described condition with distinct clinical and histologic features that distinguish it from classic morphea. To date this disease has been reported only in females. We present a report of this condition in a man.

Topics: Adult; Antigens, CD34; Calcitriol; Coloring Agents; Dermatologic Agents; Humans; Male; Scleroderma, Localized; Skin

Topics: Administration, Topical; Calcitriol; Child; Combined Modality Therapy; Emollients; Facial Hemiatrophy; Female; Follow-Up Studies; Humans; Hyperpigmentation; Male; PUVA Therapy; Scleroderma, Localized; Treatment Outcome; Ultrasonography

A 5-year-old girl presented with a 2-month history of an indurated hypopigmented, atrophic plaque of biopsy-documented morphea over the right hip area. Previous treatment with 0,1% betamethasone valerate cream twice a day for 3 months failed to improve the lesion. She was treated with calcipotriol ointment twice daily, with nightly occlusion to the plaque for 9 months, and this resulted in resolution. No side effects were noted.

Topics: Administration, Topical; Biopsy, Needle; Calcitriol; Child, Preschool; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Follow-Up Studies; Humans; Immunohistochemistry; Scleroderma, Localized; Severity of Illness Index; Treatment Outcome

We examined the responsiveness of cultured dermal fibroblasts from biopsies of 6 patients with active morphoea and a similar number of matched controls to the cell proliferation inhibition activity of calcipotriol. Cultured fibroblasts from controls showed no significant response to calcipotriol (at concentrations of 1 x 10(-8) to 1 x 10(-4) M). However, calcipotriol did inhibit the proliferation response of morphoea fibroblasts at all concentrations when compared with controls. There was 4- to 20-fold inhibition in 2 of the morphoea patients when compared with control samples. Four other morphoea samples showed inhibition but to a lesser extent compared with controls.

Topics: Adolescent; Adult; Biopsy; Calcitriol; Case-Control Studies; Cell Division; Cells, Cultured; Dermatologic Agents; Female; Fibroblasts; Humans; Male; Middle Aged; Scleroderma, Localized