Cutaneous Connective Tissue Diseases: Epidemiology, Diagnosis, and Treatment

Reddy, Bobby Y.; Hantash, Basil M.

doi:10.2174/1874372200903010022

Cited by 12 publications

(9 citation statements)

References 55 publications

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“…This manifestation is usually triggered by UV irradiation although not exclusively. This so called “butterfly eruption” typically lasts from days to weeks and heals without scarring ( 24 , 32 ). The rare, generalized form is presented as a morbilliform widespread eruption ( 24 , 33 ).…”

Section: Classification Of Subtypes Of Clementioning

confidence: 99%

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Clinical aspects of cutaneous lupus erythematosus

Elmgren

Nyberg²

2023

Front. Med.

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Lupus erythematosus (LE) is an autoimmune inflammatory disease with a wide clinical spectrum from life-threatening multi-organ inflammation in systemic lupus erythematosus (SLE) to limited skin disease in cutaneous LE (CLE). The etiology of CLE is still not fully understood but a multifactorial genesis with genetic predisposition and certain environmental factors as triggers for the development are generally accepted features. Lesions can be induced and aggravated by UV-irradiation and smoking is linked to more severe forms of skin disease and to co-morbidity. Drugs, including many common medicines like antihypertensives, are known to induce subacute CLE (SCLE). The mechanisms involved have recently been shown to be part of the IFN-I pathway and new, specific treatments are currently in clinical trials. CLE is currently classified in subtypes based on clinical presentation and duration into acute CLE (ACLE), SCLE, and chronic CLE (CCLE). Distinct subtypes can be seen in individual patients or coexist within the same patient. Because of the confluent and overlapping picture between these subsets, serology, and histopathology constitute an important role guiding towards correct diagnose and there is ongoing work to update the classification. The Cutaneous Lupus Area Severity Index (CLASI) is a validated tool to measure activity and damage both in clinical trials but also for the clinician to evaluate treatment and follow the course of the disease among patients. CLE is known to have substantial impact on the life of those affected. Several tools have been proposed to measure QoL in these patients, currently Skindex-29 is probably the most used. Patient education is an important part of prevention of flares, including UV-protection and smoking cessation. First-line treatment includes topical corticosteroids as well as topical calcineurin inhibitors with the addition of systemic treatment with antimalarials in more severe or therapy resistant cases. Treatment specifically targeting CLE has been lacking, however novel potential therapies are in later phase clinical trials. In this review we aim to describe the different subsets of the cutaneous form in LE with focus on clinical aspects.

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Section: Classification Of Subtypes Of Clementioning

confidence: 99%

“…It presents as a widespread erythema with epidermal detachment ( 20 , 35 ). Other manifestations appearing with ACLE are telangiectasias, oral ulcerations, poikiloderma, scales, and erosions ( 24 , 32 ).…”

Section: Classification Of Subtypes Of Clementioning

confidence: 99%

Clinical aspects of cutaneous lupus erythematosus

Elmgren

Nyberg²

2023

Front. Med.

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“…This symptom is pathognomonic for dermatomyositis. (10) 2. The heliotrope rash is a macular, reddish-purple rash that appears on the eyelids and is named after the small purple flower Heliotrope peruvianum, which grows in Peru.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Periungual telangiectasias are visible blood vessels that appear as irregular splinters on the proximal nail folds, and they are frequently associated with ragged, dystrophic cuticles. (10) 5. Scalp alopecia with scaly, reddish violet lesions is a condition that can occur.…”

Section: Clinical Featuresmentioning

confidence: 99%

Summarized Review on Dermatomyositis: Demonstrating Modes of Diagnosis & Current Therapeutic Managements

Ghosh

2021

Int J Res Rev

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Dermatomyositis (DM) is an autoimmune idiopathic inflammatory myopathy characterized by distinctive skin lesions, specific autoantibodies, and, most importantly, subacute symmetrical proximal muscle weakness and inflammation. Clinically amyopathic DM is a type of DM that presents with only characteristic skin lesions and no muscle disease. Heliotrope rash, erythematous rash over the face, erythematous scaly papules over the interphalangeal and metacarpophalangeal joints (Gottron's papules), erythema over the same joints (Gottron's sign), photosensitive pruritic erythema over the anterior chest and neck (V-sign), erythema extending to the shoulders and malignancy, respiratory disease, and cardiac disease are all causes of mortality and morbidity in dermatomyositis. Depending on the patient's parameters, management may include photoprotection and medications such as systemic corticosteroids, antimalarials, mycophenolate mofetil, biologicals, and IV immunoglobulins. Keywords: dermatomyositis, Gottron’s papules, heliotrope rash, inflammatory myopathy.

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“…Of these antinuclear antibodies, anti-double-stranded make up 50%, antihistone 47%, anti-topoisomerase 76%, and anti-centromere 12%. In generalized morphea, eosinophilia has been reported and may correlate with the severity of the disease [4]. Rheumatoid factor can also be elevated in 60% of those with scleroderma.…”

Section: Diagnosismentioning

confidence: 99%

A Case of Extensive Debilitating Generalized Morphea

Sapra

Dix

Bhandari

et al. 2020

Cureus

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Morphea, also known as localized scleroderma, is an uncommon idiopathic inflammatory disorder leading to the development of sclerotic plaques in the skin. The disorder preferentially affects females. The pathogenesis of morphea is not well-understood. The disorder is likely to have an autoimmune basis; environmental and genetic factors may also play a role in its etiology. Morphea has a variety of clinical presentations. Lesions of morphea typically begin as inflammatory plaques or patches that evolve into firm sclerotic lesions. Involvement may be limited to the dermis or may extend to underlying subcutaneous fat, muscle, or bone. The identification of characteristic clinical findings is often sufficient for the diagnosis of morphea. A biopsy can be a useful tool when the diagnosis is in question or to obtain information on the depth and intensity of the disease, and it should always extend at least into the subcutaneous fat. Morphea may cause joint contractures and other impairments secondary to tissue sclerosis and can be very debilitating cosmetically and functionally.

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Cutaneous Connective Tissue Diseases: Epidemiology, Diagnosis, and Treatment

Cited by 12 publications

References 55 publications

Clinical aspects of cutaneous lupus erythematosus

Clinical aspects of cutaneous lupus erythematosus

Summarized Review on Dermatomyositis: Demonstrating Modes of Diagnosis & Current Therapeutic Managements

A Case of Extensive Debilitating Generalized Morphea

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