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. Subacute cutaneous lupus erythematosus scle workup. Systemic lupus erythematosus sle is a prototypic auto immune disease resulting from abnormal immune re sponses of immune cells including dendritic cells dcs, macrophages, monocytes, neutrophils, and lymphocytes 1, 2. Early recognition and treatment improves the prognosis. Many studies have explored the pathology of systemic lupus erythematosus sle, an autoimmune rheumatic disorder with a striking female predominance. It has a prolonged course and can have a considerable effect on quality of life. Toxic epidermal necrolysislike subacute cutaneous lupus. Clinical, histologic, and immunofluorescent distinctions. Symptoms vary between people and may be mild to severe. But the clinical differential diagnosis of lupus erythematosus.
Note that this may not provide an exact translation in all languages. Lupus erythematosus is an autoimmune system disease that may take three forms. The subacute cutaneous lupus typically manifests solely on the skin, but in some cases, it may also. The lesions of tle may be indistinguishable from those of subacute cutaneous lupus. 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. Pauciinflammatory cellpoor vacuolar lymphocytic interface dermatitis in a patient with systemic lupus erythematosus. Lupus erythematosus le is a chronic, autoimmune disease that includes a broad spectrum of symptoms. It is important to confirm a cle diagnosis histopathologically by a. We report a patient with typical subacute cutaneous lupus erythematous that progressed with large sheetlike areas of epidermal detachment and nikolsky sign resembling ten. 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. Cutaneous lupus erythematosus cle comprises various forms of lupus, including acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus. Johns hopkins university school of medicine, ba,ltimore.
Lupus erythematosus is an autoimmune disease whereby the. Subacute cutaneous lupus erythematosus scle lies clinically and histologically between the more aggressive form of dle with a cicatricial tendency, and the shortlived nondestructive malar erythema of acute lupus erythematosus ale. Pathology of lupus erythematosus connecting repositories. 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. Lupus erythematosus primary care dermatology society uk. Subacute cutaneous lupus erythematosus scle is a clinically distinct disease that is associated with systemic lupus erythematosus in 50% of patients, and can be associated with sjogrens syndrome and patients with a deficiency in the second and fourth components of complement.
Subacute cutaneous lupus erythematosus observed with. About 15% of patients with cutaneous le have subacute cutaneous le. Scle commonly develops in sunexposed areas, including the upper back, shoulders, extensor arms, neck, and upper torso, while the face is often spared. Scle presents as a small, erythematous, scaly papular eruption in a photosensitive distribution that subsequently leads to a psoriasiform or annular lesion. 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 triggered by. These clinical observations have enhanced our ability to diagnose and treat lupus patients, and inspired basic research that has advanced our understanding of. The main types of lupus are discoid lupus erythematosus dle, subacute cutaneous lupus erythematosus scle and systemic lupus erythematosus. Re cent advances in our understanding of the cutaneous histopathology which correlates with. Original research article role of histopathology in. Lupus erythematosus le comprises an uncommon group of connective tissue disorders. Utilizing criteria proposed by these authors, we examined 77. Chilblain lupus erythematosus is a rare subset of chronic cle that first was described in 1888 1 and is characterized by tender violaceous papules and plaques that typically present in an. Histologic findings in cutaneous lupus erythematosus.
Pdf cutaneous lupus erythematosus issues in diagnosis and. 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. Systemic lupus erythematosus mycosis fungoides bullous pemphigoid cutaneous. Discoid lupus affects only the skin and does not usually involve internal organs. Pdf cutaneous lupus erythematosus le may present in a variety of clinical forms. Lupus erythematosus le is a multisystem disease with a broad range of clinical manifestations ranging from an isolated cutaneous eruption at one end to a fatal systemic illness at the other. Lesions that are histopathologically specific for le. In north america, scle is more common in caucasians than other ethnicities. We classified 27 lupus patients, on the basis of cutaneous exam, as having discoid lupus skin lesions, subacute cutane ous skin lesions, or systemic lupus erythematosus sle without dle or scle lesions. Jn 1984 subacute cutaneous lupus erythematosus and discoid lupus erythematosus. Jerdan js, hood af, moore gw, callan jp 1990 histopathologic comparison of the subsets of lupus erythematosus. Comparative analysis of acute cutaneous lupus erythematosus. This chemotherapeutic agent is approved for the treatment of multiple solid tumors.
Davidbajar dermatology service, department of medicine, fitzsimons army medical center, aurora, colorado, u. Lupus erythematosus le is a chronic inflammatory disease that can be clinically. Histopathology from skin biopsy of the arm demonstrated interface change with extensive epidermal necrosis, dermal. Histologic findings in cutaneous lupus erythematosus springerlink. Cutaneous pathology issn 03036987 the cutaneous pathology of lupus erythematosus. Recent advances in our understanding of the cutaneous histopathology which correlates with the traditional forms of le, along with certain novel le subtypes, are the focus of. The histopathologic features of scle are almost identical to those of dle. Histopathology the examination by he of the skin fragment obtained from the upper right. Article information, pdf download for pathology, immunopathology, and. However, scle is more often diagnosed in middleaged women. Pathology, immunopathology, and immunohistochemistry in.
Neil crowson1 and unaccompanied by extracutaneous stigmata to a rapidly progressive cynthia magro2 lethal multiorgan disease. Maryland lupus erythematosus le is a disease with a spectrum of disease. Lupus erythematosus, also called lupus, an autoimmune disorder that causes chronic inflammation in various parts of the body. Recent advances in our understanding of the pathophysiological basis of le bring hope for novel therapeutic approaches. Subacute cutaneous lupus erythematosus scle is a recently described distinct subset of lupus erythematosus le having characteristic clinical, serologic, and genetic findings. Patients with acute cutaneous lupus erythematosus acle usually have systemic. The aim of this chapter, which is set out as below, is to highlight the key features of these conditions. Acute cutaneous lupus erythematosus, chronic cutaneous lupus. Relatively common autoimmune disease of women, due to antidna antibodies fatigue, fever, arthritis, erythematous, bilateral butterfly malar rash of. Subacute cutaneous lupus erythematosus an overview. Lupus is the latin word for wolf and has been used to name various skin diseases at least since the 10 th century. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli.
Systemic lupus erythematosus sle, also known simply as lupus, is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue in many parts of the body. Subacute cutaneous lupus erythematosus cutaneous lupus erythematosus cle is a potentially disfiguring autoimmune disease that can manifest only in the skin or can be present as part of a diverse range of potentially lifethreatening and debilitating symptoms in patients with lupus erythematosus le. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel. Lupus erythematosus lupus erythematosus le may be seen in one of two wellrecognized forms systemic acute lupus erythematosus sle profound impact it has on many organs discoid chronic lupus erythematosus dle chronic and localised skin lesions involving the bridge of nose and. 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. Chilblain lupus erythematosus presenting with bilateral. It includes systemic lupus erythematosus, a chronic inflammatory disease affecting many systems of the body. She denied having other systemic symptoms or taking other medications. Histopathologic comparison of the subsets of lupus. Druginduced lupus is a form of lupus caused by medications. Onethird of the cases are due to previous drug exposure. In addition, nonimmune cells such as endothelial, epithelial, and renal tubular cells contribute to the devel opment of sle. The infiltrate is confined to the superficial and mid dermis, and is. 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.
Subacute cutaneous lupus erythematosus triggered by radiotherapy. Subacute cutaneous lupus erythematosus induced by gemcitabine in 2 patients with pancreatic cancer matthew a. Cutaneous lupus erythematosus may be subdivided into acute, subacute, or chronic based upon the. 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. Le is included among the so called connective tissue diseases and is divided into one systemic form sle and one cutaneous form cle. Autoimmune disease, subacute cutaneous lupus erythematosus, discoid. A recent study by bangert et al suggests that there are quantitative histologic differences that distinguish discoid lupus erythematosus dle and subacute cutaneous lupus erythematosus scle. Druginduced lupus accounts for up to 10% of lupus erythematosus cases. Imiquimodinduced subacute cutaneous lupus erythematosus. From a dermatologic standpoint, the type of skin involvement can prove to be a good barometer of the pattern of underlying systemic activity.
Subacute and chronic cutaneous lupus erythematosus. Histology of a skin biopsy taken from the left breast showed an acute. Three main types of lupus are recognizeddiscoid, druginduced, and systemic. Authoritative facts about the skin from dermnet new zealand trust. The differential diagnosis includes other annular papulosquamous conditions including granuloma annulare and tinea corporis.
Cutaneous histopathology of lupus erythematosus request pdf. Early diagnosis and treatment of discoid lupus erythematosus. 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. The diagnosis is usually made by clinical examination. Subacute cutaneous lupus erythematosus a twodecades.
Histopathology results were identical to that of the previous 2 patients. I wish to describe the cardinal cutaneous histolpgic changes seen in. Subacute cutaneous lupus erythematosus scle manifested. Antinuclear antibody ana and sjogren syndrome antibodya ssa were the only significant positive serology.
Subacute cutaneous lupus erythematosus scle is a nonscarring, nonatrophyproducing, photosensitive dermatosis. The clinical presentations range from a skin rash unaccompanied by extracutaneous. Gemcitabine is a nucleoside analog that inhibits cell growth by incorporating into dna and prohibiting dna polymerases. Clinically, subacute cutaneous lupus erythematosus scle was suspected. Acute cutaneous lupus erythematosus demonstrating the malar. Cutaneous lupus erythematosus cutaneous le includes three subsets of lespecific skin diseases. Systemic lupus erythematosus sle is a chronic inflammatory disease of unknown cause that affects, either singularly or in combination, the skin, joints, kidneys, nervous system, and membranes lining body cavities and often other organs as well.
Subacute cutaneous lupus erythematosus and discoid lupus. Cutaneous lupus erythematosus cle is a potentially disfiguring autoimmune disease that can manifest only in the skin or can be present as part of a diverse range of potentially lifethreatening and debilitating symptoms in patients with lupus erythematosus le. Gilliam recognized subacute cutaneous lupus erythematosus scle as a lupusspecific eruption that identifies a uni ue subset of lupus erythematosus. Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sun. Common side effects include hematologic toxicity, flulike symptoms, peripheral edema, and gastrointestinal disturbances. This study describes the histopathologic characteristics of scle and. However, there are forms of lupus which chiefly affect the skin, such as discoid lupus erythematosus and subacute lupus erythematosus. Ccle encompasses discoid lupus erythematosus dle, lupus erythematosus tumidus le tumidus, lupus profundus also known as.
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