Erythematosus 1
The clinical expression of skin involvement in patients with lupus erythematosus LE is very common and shows great variation. Therefore, it has been difficult to develop a unifying concept of the cutaneous manifestations of this disease, and much attention has been paid to the issue of classifying LE from the dermatologic perspective in the past. However, the only universally accepted criteria for the classification of LE are those of the American Rheumatism Association ARA , now known as the...
MRL Mouse
Fas-Defect MRL Mice as a Model of Systemic Lupus Erythematosus MRL mice were originally developed by Murphy and Roths at Jackson Laboratories Murphy and Roths 1978 . Two substrains of MRL mice were developed the MRL lpr and the MRL n mouse. These substrains were derived mainly from the LG J strain with contributions from AKR J, C3H Di, and C57BL 6J mouse strains. The spontaneous autosomal recessive mutation Ipr lymphoproliferation was first observed at the 12th generation of inbreeding. The...
Perniosis Chilblains
Perniosis is an inflammatory disease process commonly occurring in patients living in cold, damp geographical regions. It typically consists of erythematous papules involving the fingers, toes, nose, or ears. In general, most cases resolve within a few days and are not related to SLE. However, some lesions persist and demonstrate blister or ulcer formation. French investigators have determined that one third of the patients with chilblain lupus having this disease process for greater than 1...
Discoid Lupus Erythematosus of the Palms and Soles
DLE of the palms and soles is a rare occurrence characterized by sharply demarcated erythemas and adherent scaling the lesions tend to be painful and may become erosive. Atrophy and scarring are the features that allow a distinction from the following Palmoplantarpsoriasis has no signs of atrophy. Note that palmoplantar psoriasis is only rarely an isolated finding. Keratoderma blennorhagicum in patients with Reiter's disease presents with pso-riasiform palmoplantar lesions composed of...
Erythema Multiforme and Lupus Erythematosus Rowells Syndrome
In 1963, Rowell et al. Rowell et al. 1963 described four patients with the clinical picture of chronic DLE associated with erythema multiforme EM . Besides the skin changes compatible with both diseases, the patients had characteristic immunologic findings consisting of the speckled type of ANAs, the anti-SjT type of precipitating antibody to saline extract of human tissues and rheumatoid factor. In some patients, perniotic-like lesions were also observed. Later it turned out that SjT is...
CAMs in Cutaneous Lupus Erythematosus
Different patterns of ICAM-1 expression in the epidermis have been documented in LE vs other cutaneous inflammation and also between subsets of LE. In experimentally UVA- and UVB-induced lesions in patients with LE and PLE, those with SCLE showed ICAM-1 expression throughout the epidermis, those with DLE showed basal ICAM-1 staining, and those with PLE showed focal basal ICAM-1 staining associated with lymphocyte infiltrates Stephansson and Ros 1993 . SCLE, erythema multiforme, and lichen...
Management
Cutaneous NLE is best managed conservatively. Low-potency topical corticosteroid therapy may aid in decreasing inflammation, and sun protection measures are advisable. Residual telangiectasia may be treated with a vascular laser such as a pulsed dye laser. Optimal therapy for cardiac NLE has not been established. Pacemaker implantation and medications traditionally used to treat heart failure are used when indicated. Several other therapeutic interventions have been tried, including...
Erythromelalgia Erythromalgia
Erythromelalgia is a rare disease existing in two forms primary and secondary Cohen 2000 . Primary erythromelalgia is the most common form and can begin at any age. Secondary erythromelalgia has been reported with many disorders, most commonly polycythemia and myelodysplastic disorders. It has also been associated with connective tissue disorders, most commonly SLE. Erythromelalgia is characterized by prominent erythema and increased temperature involving predominantly the feet, but the hands...
of Cutaneous Lupus Erythematosus
The term lupus erythemateaux was used for the first time by Cazenave Cazenave 1851 in 1851 to distinguish the noninfectious forms of lupus from cutaneous tuberculosis lupus vulgaris . Cazenave referred in his original paper to Biett's earlier report on this disease, which was termed erytheme centrifuge, as being a very good description of what nowadays would be called discoid lupus erythematosus DLE . Also, in 1845, Hebra Hebra 1845 precisely described systemic manifestations of LE that...
Lupus Erythematosus Profundus
LEP, historically referred to as Kaposi-Irgang disease or also known as lupus pan-niculitis, is a rare variant of CCLE in which pathologic changes occur primarily in the lower dermis and subcutaneous tissue. In 1883, subcutaneous nodules associated with LE were first described by Kaposi Kaposi 1883 , but the term lupus profundus was coined by Irgang Irgang 1940 in 1940. Subsequently, different authors reported new cases and contributed to define the clinical and histopathologic characteristics...
References Nsq
O'Leary PA 1934 Disseminated lupus erythematosus. Minn Med 17 637-644 Baer RL, Harber LC 1965 Photobiology of lupus erythematosus. Arch Dermatol 92 124-128 Brocq L 1921 Precis-atlas de pratique dermatologique. G Doin, Paris, pp 468-477 Casciola-Rosen LA, Anhalt G, Rosen A 1994 Autoantigens targeted in systemic lupus erythematosus are clustered in two populations of surface structures on apoptotic keratinocytes. J Exp Med 179 1317-1330 Cazenave PLA 1851 Lupus erythemateux erytheme centrifuge...
Lupus Erythematosus Tumidus
In 1909, the term lupus erythematosus tumidus was first used by Hoffmann Hoffmann 1909 at a meeting of the Berlin Dermatological Society, and, in 1930, five further patients were reported by Gougerot and Burnier Gougerot and Burnier 1930, 1931,1932 to describe a disease with erythematous, indurated, nonscarring lesions on the face with minimal or absent surface changes. However, the next case reports of LET were not mentioned until 1965 in the German and French literature Bazex et al. 1965,...
Subacute Cutaneous Lupus Erythematosus
SCLE, a subset with specific clinical and serologic features occurring preferentially in white females, was first discussed as a distinct entity by Gilliam in 1977 Gilliam 1977 , with expanded descriptions in 1979 Sontheimer et al. 1979 , 1981 Gilliam and Sontheimer 1981 , and 1982 Gilliam and Sontheimer 1982 . This subtype shows a significant coincidence with HLA-DR2 or -DR3, and patients with overlapping manifestations of Sjogren's syndrome or high levels of anti-Ro SSA antibodies are more...
Acute Cutaneous Lupus Erythematosus
The typical clinical manifestations of ACLE are characterized by a localized erythema known as the malar rash or butterfly rash on the central portion of the face or by a generalized, more widespread form Fabbri et al. 2003, Sontheimer and Provost 2003 . Localized ACLE may only affect the skin transiently, and the lesions may last for only several days up to a few weeks. Therefore, at the onset of disease, the patients may mistake this rash for sunburn and may seek medical advice only after the...
Bullous Lesions in Lupus Erythematosus
The frequency of bullous lesions associated with LE BLE is low, and it has been reported that less than 5 of patients with SLE and skin changes had chronic vesicu-lobullous lesions Gammon and Briggaman 1993, Yell and Wojnarowska 1997 . Confusion has always existed concerning the nosology and classification of the bullous skin changes that can occur in patients with LE, and there has been no consensus in this area for a long time. In 1997, Sontheimer Sontheimer 1997 divided the various types of...
Discoid Lupus Erythematosus
The most common form of all chronic cutaneous variants is DLE, which can be localized or generalized, both with and without systemic manifestations of LE. Typical DLE lesions may be present at the onset of SLE in about 5 -10 of patients, and approximately 30 of patients may develop DLE lesions, usually of the generalized type, during the course of SLE Cervera et al. 1993,Hymes and Jordon 1989,Tebbe et al. 1997 . The localized form presents with sharply demarcated, erythematokeratotic, atrophic...
List of Contributors
Medicas y Nutricion Salvador Zubiran Bacman, David, M.D. University of D sseldorf Department of Dermatology Moorenstra e 5 40225 D sseldorf Germany Beissert, Stefan, M.D. University of M nster Department of Dermatology Von-Esmarch-Stra e 58 48149 M nster Germany Callen, Jeffrey P., M.D. University of Louisville School of Medicine Department of Dermatology 310 East Broadway, Suite 200 Louisville KY 40202 USA Casciola-Rosen, Livia A., Ph.D. John Hopkins University School of Medicine Departments...