Lupus erythematosus primary care dermatology society uk. Topical druginduced subacute cutaneous lupus erythematosus. Scle can occur in patients with sle, be induced by drugs, or may be associated with other diseases such as sjogrens syndrome or complement deficiency. Subacute cutaneous lupus erythematosus induced or exacerbated by terbinafine. Acute cutaneous lupus erythematosus acle lesions can be localized or generalized. This study describes the histopathologic characteristics of scle and determines whether it could be differentiated from. Lupus, technically known as systemic lupus erythematosus sle, is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue in many parts of the body. The hallmark feature of acle is localized to the malar region butterfly rash and is characterized by confluent, macular or papular erythema lasting days to weeks that occurs symmetrically on the cheeks and bridge of the nose, sparing the nasolabial folds figure 801. Acle may be localized most often as a malar or butterfly rash or generalized. Davidbajar gilliam recognized subacute cutaneous lupus erythematosus scle as a lupus specific eruption that identifies a unique subset of lupus erythematosus. Case 1 an 88yearold active, independent woman with hypothyroidism, hypertension, gastroesophageal re. Subacute cutaneous lupus erythematosus scle is a welldefined subtype of lupus erythematosus, characterised by photosensitivity, annular andor psoriasiform lesions, variable systemic involvement and presence of circulating ssaantiro antibodies. Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Common side effects include hematologic toxicity, flulike symptoms, peripheral edema, and gastrointestinal disturbances.
Davidbajar gilliam recognized subacute cutaneous lupus erythematosus scle as a lupusspecific eruption that identifies a unique subset of lupus erythematosus. Subacute cutaneous lupus erythematosus scle clinical features table 2 patients with subacute cutaneous le scle manifest a photodistributed, nonscarring papulosquamous andor annular, polycyclic eruption which may occur in isolation or be accompanied by usually mild extra cutaneous manifestations. We report a patient with typical subacute cutaneous lupus erythematous that progressed with large sheetlike areas of epidermal detachment and nikolsky sign resembling ten. Lupus is the latin word for wolf and has been used to name various skin diseases at least since the 10 th century. In the years of published reports of scle patients following the initial study by sontheimer et al subacute cutaneous lupus erythematosus. A histopathologic study of cutaneous lesions of lupus. This study describes the histopathologic characteristics of scle and. Anastrozoleinduced subacute cutaneous lupus erythematosus.
Pattern of cutaneous immunoglobulin g deposition in subacute cutaneous lupus erythematosus is reproduced by infusing purified antirossa antibodies into human skin grafted mice. Subacute cutaneous lupus erythematosus with a possible. Cutaneous lupus erythematosus cle is the second most common presenting symptom of autoimmune lupus erythematosus le. The subacute cutaneous lupus typically manifests solely on the skin, but in some cases, it may also involve the joints. Imiquimodinduced subacute cutaneous lupus erythematosus. Acles histopathology demonstrates an interface dermatitis, with mild focal vacuolar alteration of the basal layer. We have characterized the clinical and laboratory features of 27 patients who had in common a recurring, superficial, nonscarring type of cutaneous lupus erythematosus le that occurred in a characteristic distribution subacute cutaneous lupus erythematosus scle. Three recognized subtypes of cutaneous le are acute cutaneous le acle, subacute cutaneous le scle, and chronic cutaneous le ccle. Systemic lupus erythematosus sle is a multiorgan autoimmune disease of unknown etiology that can have many clinical manifestations table 1.
Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. Histopathology of cutaneous lupus erythematosus sciencedirect. Pathology, immunopathology, and immunohistochemistry in. From a dermatologic standpoint, the type of skin involvement can prove to be a good barometer of the pattern of underlying systemic activity. Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous violaceous.
Cutaneous lupus erythematosus le may present in a variety of clinical forms. Fibrinoid necrosis at dermoepidermal junction with liquefactive degeneration and atrophy of epidermis. Request pdf cutaneous histopathology of lupus erythematosus lupus erythematosus le is an autoimmune connective tissue disease whose precise aetiological basis is at present uncertain, in. Subacute cutaneous lupus erythematosus lupus erythematosus. Authoritative facts about the skin from dermnet new zealand. Subacute cutaneous lupus erythematosus observed with. Pdf cutaneous lupus erythematosus issues in diagnosis. Davidbajar dermatology service, department of medicine, fitzsimons army medical center, aurora, colorado, u. May 10, 2017 from a dermatologic standpoint, the type of skin involvement can prove to be a good barometer of the pattern of underlying systemic activity.
Three recognized subtypes of cutaneous le are acute cutaneous le. Le is included among the so called connective tissue diseases and is divided into one systemic form sle and one cutaneous form cle. Subacute cutaneous lupus erythematosus scle is a recently described distinct subset of lupus erythematosus le having characteristic clinical, serologic, and genetic findings. Lupus erythematosus le is an autoimmune connective tissue disease whose precise aetiological basis is at present uncertain, in which the clinical presentations range from a skin rash unaccompanied by extracutaneous stigmata to one comprising progressive multisystem disease. Subacute cutaneous lupus erythematosus scle workup. The skin is involved in up to 85% of systemic lupus erythematosus sle cases and may be the only organ involved in cutaneous lupus erythematosus cle. Az of skin cutaneous lupus erythematosus back to az search. Tumid lupus erythematosus tle, also known as lupus erythematosus tumidus, is a highly photosensitive form of cutaneous lupus erythematosus cutaneous. The term subacute cutaneous lupus erythematosus was coined in 1977 by gilliam. Dle most commonly occurs on the head and is characterized by welldefined inflammatory plaques that evolve into atrophic, disfiguring scars. Druginduced subacute cutaneous lupus erythematosus discle has been associated with numerous drugs, but there are limited reports of its association with aromatase inhibitor anastrozole.
Subacute cutaneous lupus erythematosus is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as polycyclic annular lesions or plaques similar to those of plaque psoriasis. The acr criteria, which include four cutaneous signs, may lead to overdiagnosis of sle in patients with predominantly cutaneous disease. More mucin deposition in reticular dermis than discoid lupus. Subacute cutaneous lupus erythematosus an overview.
The diagnosis is usually made by clinical examination. Early recognition and treatment improves the prognosis. Toxic epidermal necrolysislike subacute cutaneous lupus. Lupus erythematosus le comprises an uncommon group of connective tissue disorders. Linear chronic discoid lupus erythematosus following the. Subacute cutaneous lupus erythematosus scle is a subtype of cutaneous lupus erythematosus cle with distinct clinical and immunologic features.
Subacute cutaneous lupus erythematosus scle as a distinct subset of lupus erythematosus le was first suggested by gilliam in 1977. Acute cutaneous lupus erythematosus, chronic cutaneous lupus. Article information, pdf download for pathology, immunopathology, and. The recognition and management of discle can be challenging, as the condition may be triggered by different classes of drugs after variable lengths of time. Affected patients are almost always caucasian and cutaneous manifestations often. Cutaneous histopathology of lupus erythematosus request pdf. Druginduced lupus accounts for up to 10% of lupus erythematosus cases. Chronic cutaneous lupus erythematosus chronic cutaneous lupus erythematosus ccle is more common in women. Subacute cutaneous lupus erythematosus scle clinical features table 2 patients with subacute cutaneous le scle manifest a photodistributed, non. Recent advances in our understanding of the pathophysiological basis of le bring hope for novel therapeutic approaches.
Hypertrophic chronic cutaneous lupus erythematosus. Cutaneous lupus erythematosus cle is lupus affecting the skin in this autoimmune disease, the bodys immune system attacks healthy skin. Subacute cutaneous lupus erythematosus and discoid lupus. The main types of lupus are discoid lupus erythematosus dle, subacute cutaneous lupus erythematosus scle and systemic lupus erythematosus. Gilliam recognized subacute cutaneous lupus erythematosus scle as a lupusspecific eruption that identifies a uni ue subset of lupus erythematosus. Linear chronic discoid lupus erythematosus is a rare manifestation of cutaneous lupus in which erythematous, atrophic, dyschromic lesions are located along the lines of blaschko. Subacute cutaneous lupus erythematosus exacerbated or. Gemcitabine is a nucleoside analog that inhibits cell growth by incorporating into dna and prohibiting dna polymerases. The prototypical example of acute cutaneous lupus is the malar rash. Both the idiopathic and druginduced forms of scle are analogous in their clinical, serological and.
Lupus erythematosus le is a common autoimmune disease that manifests in systemic and cutaneous forms. Druginduced scle and drug induced chronic cutaneous lupus. Subacute cutaneous lupus erythematosus in older man. Discoid lupus erythematosus dle and subacute cutaneous lupus erythematosus scle are variants of cutaneous lupus that may occur independently or as manifestations of systemic lupus erythematosus. Subacute cutaneous lupus erythematosus induced by gemcitabine. Cutaneous lupus or skin lupus systemic lupus lupus. Cutaneous manifestations of systemic lupus erythematosus. It can be associated with sjogren syndrome and rheumatoid arthritis ra as well as several classes of medications. First case of paraneoplastic subacute cutaneous lupus erythematosus associated with colon cancer. The most common forms are subacute cutaneous lupus erythematosus scle and discoid lupus erythematosus dle. Gilliam recognized subacute cutaneous lupus erythematosus scle as a lupus specific eruption that identifies a uni ue subset of lupus erythematosus. Cutaneous lupus erythematosus clinical and laboratory. In many respects, it may be regarded as a model autoimmune disease due to its spectrum of autoimmune antibodies and involvement of different organ systems, as well as respon.
Lupus erythematosus le is a chronic, autoimmune disease that includes a broad spectrum of symptoms. An increasing number of drugs have been linked to druginduced subacute cutaneous lupus erythematosus discle. Lastly, chronic cutaneous lupus can be further subdivided into a number of cutaneous findings including discoid, tumidus, profundus and chilblains. Figure 1 acute cutaneous lupus erythematosus a, subacute. Lupus erythematosus le is a chronic inflammatory disease that can be clinically. Pdf cutaneous lupus erythematosus le may present in a variety of clinical forms. Subacute cutaneous lupus erythematosus is a widespread, photosensitive nonscarring form of cutaneous lupus often associated with antiro antibodies. Lupus erythematosusspecific skin diseases are classified into three categories, 1 acute cutaneous lupus erythematosus acle, 2 subacute cutaneous lupus erythematosus scle, and 3 chronic cutaneous lupus erythematosus ccle. Although subacute cutaneous lupus erythematosus scle has been reported in association with multiple systemic medications, scle in patients treated with topical agents has not been. Druginduced subacute cutaneous lupus erythematosus. Histopathologic comparison of the subsets of lupus. Pdf cutaneous lupus erythematosus issues in diagnosis and. Early diagnosis and treatment of discoid lupus erythematosus.
Here we present a patient with newonset scle and systemic lupus erythematosus sle after treatment with abatacept. Imiquimodinduced subacute cutaneous lupus erythematosuslike. Maryland lupus erythematosus le is a disease with a spectrum of disease. Subacute cutaneous lupus erythematosus and discoid lupus erythematosus.
Histologic findings in cutaneous lupus erythematosus. It can occasionally be induced by medications, as first reported for hydrochlorothiazide in 1985 2 and subsequently reported. Specific vesiculobullous skin lesions in lupus erythematosus le are rare and must be differentiated from toxic epidermal necrolysis ten, tenlike dermatoses and other vesiculobullous conditions. Apr 14, 2020 subacute cutaneous lupus erythematosus scle is a nonscarring, nonatrophyproducing, photosensitive dermatosis. Subacute cutaneous lupus erythematosus subacute cutaneous lupus erythematosus scle is a distinct subset of le that identifies patients with clinically erythematous, nonscarring lesions, pronounced light sensitivity, musculoskeletal complaints, characteristic serological abnormalities and a variety of relatively mild systemic symptoms. Wahrenherlenius from the department of medicine, rheumatology unit, karolinska institutet, karolinska university hospital in solna, stockholm, sweden. Sep, 2012 cutaneous lupus erythematosus le may present in a variety of clinical forms. Cutaneous lupus erythematosus comprises a range of dermatologic manifestations, including acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. The cutaneous pathology of lupus erythematosus wiley online.
Subacute cutaneous lupus erythematosus a twodecades. Subacute cutaneous lupus erythematosus a twodecades perspective. Another classification form, proposed by gilliam, separates the lesions according to their histopathology. Subacute cutaneous lupus erythematosus scle is a nonscarring, nonatrophyproducing, photosensitive dermatosis. Clinical picture, diagnosis and treatment summary lupus erythematosus le is an important dermatologic autoimmune disease.
Subacute cutaneous lupus erythematosus scle is a distinct clinical variant of lupus erythematosus that typically presents as annular, often scaly, erythematous plaques in photodistributed areas. Subacute cutaneous lupus erythematosus scle, first described in 1979, 1 is a distinct subset of cutaneous lupus erythematosus characterized by nonscarring, photodistributed, annular or papulosquamous plaques with minimal or no associated systemic findings. Subacute cutaneous lupus erythematosus scle has a rather sudden onset with annular or psoriasiform plaques erupting on the upper trunk, arms, andor dorsa of hands, usually after exposure to sunlight. This chemotherapeutic agent is approved for the treatment of multiple solid tumors. This paper describes our personal experience in the management of cle over. These patients were noted to have prominent photoaggravated skin disease. Systemic lupus erythematosus sle is an autoimmune disorder affecting not just one but many organs and systems in the body like the heart, joints, skin, lungs, blood vessels, liver, kidneys and the nervous system.
Druginduced scle is the most common form of druginduced lupus erythematosus, and these conditions are thought to be independent of their idiopathic lupus syndrome counterparts. Winkelmann rk 1981 the histopathology of lupus erythematosus. Aug 21, 2012 cutaneous lupus erythematosus cle includes a variety of lupus erythematosus lespecific skin lesions that are subdivided into three categories chronic cle ccle, subacute cle scle and acute cle acle based on clinical morphology, average duration of skin lesions and routine histopathologic examination. More mucin deposition in reticular dermis than discoid lupus edema, small hemorrhages and a mild infiltrate of inflammatory cells, principally.
It has a prolonged course and can have a considerable effect on quality of life. Subacute cutaneous lupus erythematosus occurs in genetically predisposed individuals, most often in patients with the antiro ssa autoantibody, a positive antinuclear antibody ana reaction and human leukocyte antigens b8, dr3, drw52 and dq1. Discoid lupus erythematosus is a chronic dermatological disease that can lead to scarring, hair loss, and hyperpigmentation changes in skin if it is not treated early and promptly. Subacute cutaneous lupus erythematosus scle is a distinct entity with specific clinical and serologic features that was first described by gilliam in 1977 gilliam, 1977, with expanded discussion in 1979 sontheimer et al. There is a very small chance that these cutaneous forms of lupus may develop into systemic lupus erythematosus sle with internal organ involvement. Subacute cutaneous lupus scle usually involves rashes on sunexposed areas. The aim of this chapter, which is set out as below, is to highlight the key features of these conditions. Lupus erythematosus is an autoimmune system disease that may take three forms. Multisystem involvement as a component of systemic le sle is common, with prominent. Relatively common autoimmune disease of women, due to antidna antibodies fatigue, fever, arthritis, erythematous, bilateral butterfly malar rash of the face, renal disease, lymphadenopathy, and serositis. Most patients with scle are sensitive to sunlight and have prominent cutaneous and musculoskeletal complaints but. Symptoms vary between people and may be mild to severe.
In between the 2 ends of the spectrum are disorders like subacute cutaneous lupus. Multisystem involvement as a component of systemic le sle is common, with. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is. We report the case of a patient undergoing treatment with anastrozole for breast cancer who presented with clinical, serological, and histological evidence consistent with discle.
Subacute cutaneous lupus erythematosus and systemic lupus. Lesions precede the onset of systemic symptoms in 25 percent of patients, many of whom present to dermatologists for their initial evaluation. Imiquimod is a topical immunomodulator used to treat genital warts and cutaneous malignancies that exerts its effects via induction of proinflammatory cytokines through activation of tolllike receptor tlr 7. Cutaneous lesions take the form slightly indurated patches with minimal scale. Discoid lupus type lesions in 33% cutaneous lesions take the form slightly indurated patches with minimal scale. Scle commonly develops in sunexposed areas, including the upper back, shoulders, extensor arms, neck, and upper torso, while the face is often spared. Re cent advances in our understanding of the cutaneous histopathology which correlates with.
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