Histologic findings in cutaneous lupus erythematosus. The clinical presentations range from a skin rash unaccompanied by extracutaneous. Lupus profundus is a rare manifestation of cutaneous lupus erythematosus, seen in 1% to 3% of patients. Lupus tumidus, photosensitivity, direct immunofluorescence doi. Risk of systemic lupus erythematosus is approximately 5%. Lupus erythematosus le is an autoimmune disorder resulting from an interplay of genetic, environmental, and hormonal elements with a heterogeneous clinical expression extending from a localized cutaneous form to a lifethreatening systemic form. The histopathologic features of scle are almost identical to those of dle.
Tumid lupus erythematosus tle is a subtype of discoid lupus erythematosus dle whose clinical characteristics rarely have been described in the literature. The lesions are typically chronic scaly plaques on the face, scalp and ears. Eosinophils in druginduced cases and urticarial lesions. Lupus erythematosus le is an autoimmune disease with heterogeneous presentations often involving the skin. Im tagging others who have mentioned having lupus, but not specifically this rare type. Cutaneous lupus erythematosus cle can be divided into acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus. Discoid lupus erythematosus dle is the most common form of cutaneous lupus erythematosus. What are some signs and symptoms of tumid lupus erythematosus. Histopathology of a skin biopsy from the trunk showed basal cell layer liquefaction and lichenoid interface changes suggestive of lupus erythematosus.
Initial symptoms of tumid lupus appear in the lower back pain and in the legs. The cutaneous forms of le may be divided into acute, subacute, and chronic skin. Gougerot and bournier first described its hallmark clinical features in 19301 and it has been very rarely reported since. Early diagnosis and treatment of discoid lupus erythematosus. Lupus erythematosus tumidus let is considered a form of chronic cutaneous lupus erythematosus cle. In english, tumid means swelling of parts of the body. John libbey eurotext european journal of dermatology. Some of the common types include chronic cutaneous lupus erythematosus le, which includes localized and generalized discoid lupus erythematosus dle, hypertrophic le a form of dle, tumid le, and lupus panniculitis. Tumid erythematosus lupus is characterized by smooth, nonscarring, pink to violetcolored pimples papules on the skin without any other apparent skin changes, such. Clinically, it lacks typical changes found in discoid lupus and antinuclear antibodies ana levels are elevated in only 10% of the patients. Histopathological and direct immunofluorescence examination established a.
While systemic lupus can affect any area of the body. Tumid lupus erythematosus, or tle, is perhaps the rarest form. Clinical evaluation, histopathologic and direct immunofluorescence analyses of skin. Pathology outlines lupus erythematosus panniculitis. Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. 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. To describe the clinical, histopathologic, and other laboratory findings in tumid lupus erythematosus. May 02, 2016 patients with tumid lupus erythematosus usually do not have other symptoms of systemic lupus erythematosus or other types of cutaneous lupus erythematosus. Fibrinoid material is deposited in the dermis around capillary blood vessels, on collagen and in the interstitium. Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous violaceous.
Cutaneous histopathology of lupus erythematosus a neil crowson cynthia m magro abstract 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 extra. The condition clinically resembles polymorphous light eruption, systemic lupus erythematosus sle, reticulated erythematous mucinosis, or. It is a photosensitive skin disorder, a different subtype of cutaneous lupus erythematosus cle from discoid lupus erythematosus dle or subacute cle scle. 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. Pathology of discoid lupus erythematosus dr sampurna roy md. It most commonly presents in association with classic chronic cutaneous lesions of discoid lupus erythematosus. Tumid lupus erythematosus is a rare but distinctive entity that is characterized by plaques that appear on patients trunks. Criteria for the classification of the most severe form, systemic le, have been established by the american college of rheumatology acr, formerly american rheumatism association 1, 2. Tumid lupus symptoms can be either acute, which is developing dramatically over days and takes weeks to develop. About 25% of patients with chronic cutaneous le also have systemic le discoid le. In the last 20 years, extensive research on clinical and histological aspects of the disease have led to a better characterization of this nosological entity and to differentiate it. Notably, the epidermis was unremarkable with no significant vacuolar changes. Ccle can be further be subdivided into chronic subtypes consisting of tumid lupus erythematosus tle, discoid lupus erythematosus dle, chilblain lupus erythematosus, and lupus.
The diagnosis is usually made by clinical examination. Tumid lupus erythematosus le is a rare variant of lupus erythematosus, which often follows a favorable course. Here is further information on the types of lupus from johns hopkins. Diffuse thinning or hair fragility with visible broken hairs in the absence of other causes such as alopecia areata, drugs, iron deficiency and androgenetic alopecia. It has a prolonged course and can have a considerable effect on quality of life. There are different types of lupus erythematosus, including systemic, discoid, neonatal and tumid. Overall, let is characterized by intense photosensitivity, skin lesions, a. Historically tumid lupus was not identified as a separate entity until very recently. Tumid lupus erythematosus tle is a variant of cutaneous lupus erythematosus. Dec 06, 2009 tumid lupus is a restricted form of lupus in which the only involvement is that of the skin with a superficial skin lesion. It was not until 2003 that alexiadesarmenakas et al. This specific type of lupus, also known as tumid lupus erythematosus le, is indeed a rare variant.
Lupus erythematosus tumidus let, first reported by gougerot and burnier 1 in 1930, is a highly photosensitive skin disorder with distinct clinical and histopathologic characteristics. It may affect virtually any organ or structure of the body, especially the skin, kidneys, joints, heart, gastrointestinal tract, brain, and serous membranes membranous linings of organs, joints, and cavities of the body. Tumid lupus erythematosus statpearls ncbi bookshelf. Cureus tumid lupus erythematosus and systemic lupus. Most patients who present with these skin lesions are young women. A case of a young woman is illustrated, who presented with an asymptomatic. Several patients have reported experiencing muscle spasms, a. Tumid lupus erythematosus tle, also known as lupus erythematosus tumidus, is a highly photosensitive form of cutaneous lupus erythematosus cutaneous.
Given her clinical presentation and histopathologic findings, a diagnosis of lupus. Pdf tumid lupus erythematosus le is a rare variant of lupus. It is classified as either localised, affecting skin above the neck only, or generalised, affecting above and below the neck. Initial symptoms of tumid lupus occur in the lower back pain and in the legs. Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaques, usually on the trunk lupus erythematosus tumidus let was reported by henri gougerot and burnier r. Histopathological and direct immunofluorescence examination established a diagnosis of tumid lupus erythematosus.
Comprehensive clinical, histopathologic, and immunohistochemical. Tumid lupus erythematosus indian journal of dermatology. With let, as with other chronic cutaneous lupus diseases, the prevalence of lupus related autoantibodies and development of systemic lupus erythematosus sle is low. Histopathologic findings in lupus erythematosus tumidus. Ophthalmic literature reports disease manifestation as orbital inflammation. Coexistent systemic lupus erythematosus sle has been reported to be rare, and literature shows only a few case reports. Tumid lupus erythematosus is a rare but distinctive entity which is characterized by plaques occur on patients trunks. Criteria for classification with immunohistochemical analysis. Request pdf histopathologic findings in lupus erythematosus tumidus. Edema, small hemorrhages and a mild infiltrate of inflammatory cells, principally lymphocytes, in upper dermis. Chronic cutaneous le accounts for 80% of presentations with cutaneous le. Tumid lupus symptoms may be either acute, that is developing dramatically over days, or subacute, which takes weeks to develop.
Harris skin and cancer pavilion with asymptomatic red lesions on his chest of nine years duration. In lep the overlying skin appears normal while the deep dermis and subcutaneous fat is involved by the inflammatory process 1, 2. If you would like a highquality copy or to use the image for commercial purposes, contact the owner. Lupus hair in sle is a poorly characterised entity and may be a form of telogen effluvium. Tumid lupus erythematosus tle is a rare variant of cutaneous lupus erythematosus. Histopathology reveals perivascular and periannexal lymphohistiocytic infiltrates in the papillary and reticular dermis and interstitial mucin deposition. Lupus erythematosus tumidus genetic and rare diseases. It is more prevalent in patients with skin of colour, who are at greater risk of postinflammatory hyperpigmentation and hypertrophic scarring. Review on lupus erythematosus tumidus lupus erythematosus tumidus let is a rare subset of chronic cutaneous lupus erythematosus le. The number of skin biopsies performed per patient ranged from 1 to 3.
Tumid lupus erythematosus macrene alexiadesarmenakas dermatology online journal 72. Lupus erythematosus tumidus tumid lupus dermatology. Mar 27, 2019 lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous violaceous. Lupus erythematosus is an inflammatory disorder of the immune system wherein the immune system attacks the bodys own organs and systems. Tumid erythematosus lupus is considered a rare type of chronic cutaneous lupus erythematosus.
Lupus erythematosus profundus is the preferred terminology for lesions of lupus erythematosus panniculitis that have overlying changes of discoid lupus, namely vacuolar interface dermatitis, basement membrane thickening, scale, follicular plugging and both perifollicular and periadnexal inflammation. Cutaneous lupus erythematosus cle can present in three forms. Lupus erythematosus tumidus let is a rarely diagnosed condition that was first described in 1909 by hoffmann. A unique presentation of lupus erythematosus tumidus in an. Tumid lupus erythematosus is a variant of cutaneous lupus erythematosus. One patient developed erythematous scaling papules, patches and plaques on the upper extremities and trunk resembling subacute cutaneous lupus erythematosus. Tumid erythematosus lupus is characterized by smooth, nonscarring, pink to violetcolored pimples papules on the skin without any other apparent. Most experts nowadays agree that let is a distinct subtype of cutaneous lupus erythematosus cle. The most common and most severe form is systemic lupus erythematosus. Tumid lupus is an uncommon variant of lupus erythematosus.
Traditionally, tle has been classified as a subset of chronic cutaneous le. Alopecias which show histological characteristics of le are lespecific, and include discoid le dle, diffuse or patchy hair loss in acute le, subacute cutaneous le, and rarely tumid le. It is a photosensitive skin disorder, a different subtype of cutaneous lupus erythematosus cle from discoid. Lupus erythematosus le is a chronic inflammatory disease that can be clinically. Lupus erythematosus tumidus let is an uncommon and photosensitive inflammatory skin disorder which is characterised by erythematous urticarial plaques. Several patterns of hair loss can occur in lupus erythematosus le. Early recognition and treatment improves the prognosis. Without a high index of suspicion, the diagnosis is. Our purpose was to analyze skin biopsy specimens from 80 patients with this disease and to determine whether it could be differentiated from. Recent advances in our understanding of the pathophysiological basis of le bring hope for novel therapeutic approaches.
It has a self limited spread and does not involve the remaining organs like in the case of full blown sle. Systemic lupus erythematosus is the most common form of the disease. Coexistence of tumid lupus erythematosus with systemic lupus erythematosus and discoid lupus erythematosus. Discoid le is the most common form of chronic cutaneous le. We are compelled to respond to his rhetorical question is tle tumid le a misnomer. Tumid lupus erythematosus tle, also known as lupus erythematosus tumidus, is a highly photosensitive form of cutaneous lupus erythematosus cutaneous le that classically presents with erythematous, edematous plaques picture 1ac. Severe chronic blepharitis and scarring ectropion associated with discoid lupus erythematosus. Thiazide diuretics induce cutaneous lupuslike adverse reaction. Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaques, usually on the trunk. More mucin deposition in reticular dermis than discoid lupus edema, small hemorrhages and a mild infiltrate of inflammatory cells, principally lymphocytes, in upper dermis eosinophils in drug induced cases and urticarial lesions.
Review of 80 patients in a recent study, we demonstrated that lupus erythematosus le tumidus let is a distinct subset. Lupus erythematosus tumidus let was reported by henri gougerot and burnier r. A case of a young woman is illustrated, who presented with an asymptomatic erythematous, solitary plaque over her face. Lupus erythematosus tumidus, systemic lupus erythematosus, lupus nephritis, cutaneous lupus erythematosus case presentation a 22yearold caucasian woman with a oneyear history of systemic lupus erythematosus sle was referred to dermatology clinic for a persistent eruption of a rash on her face and arms. Leukemia cutis mimicking tumid lupus as the presenting. In nonbullous cases, neutrophils are sometimes present in. Lep, also called lupus panniculitis and tumid lupus, is an uncommon variant of lupus erythematosus le characterized by inflammation involving deep dermis and subcutaneous tissue. The condition clinically resembles polymorphous light eruption, systemic lupus erythematosus sle, reticulated erythematous mucinosis, or gyrate erythema. Histopathologically, the lesions resemble classic lupus erythematosus because of their. In a recent study, we demonstrated that lupus erythematosus le tumidus let is a distinct subset of cutaneous le cle, which is clinically characterized by erythematous, urticarialike, nonscarring plaques in sunexposed areas. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. Aug 02, 2017 tumid lupus erythematosus is a rare but distinctive entity which is characterized by plaques occur on patients trunks. Cutaneous lupus erythematosus encompasses at least 10 to 15 different clinical presentations. Lupus erythematosus is a chronic inflammatory autoimmune disease with a wide range of clinical manifestations ranging from an indolent chronic cutaneous form to a severe potentially lifethreatening disease, systemic lupus erythematosus discoid lupus erythematosus is a subphenotype of chronic cutaneous lupus erythematosus, characterised by scaly diskshaped plaques which may be localised.
829 642 989 38 1251 696 88 412 1578 1364 1154 1143 983 640 1222 472 1133 1458 475 156 1403 127 764 1411 1082 1598 1561 879 260 1531 308 1526 1419 651 823 939 1317 693 924 1296 229 365 299 9 587 1299