DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

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Sunday, August 15, 2010

Answer of Dermatopathology Case 55

Discoid Lupus Erythematosus

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Histologic features of cutaneous lupus erythematosus.Autoimmun Rev.2009 May;8(6):467-73. Epub 2009 Jan 20

Histologic examination of lesions plays a key role in the diagnostics of cutaneous lupus erythematosus (LE). LE has a broad spectrum of histopathological signs, which are related to the stages of the lesions. In addition to the main subtypes of LE, we report on special manifestations like Rowell's-syndrome and Chilblain LE, and give an account of Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis), which may be associated with systemic LE. Furthermore the most considerable histopathologic differential diagnoses are discussed.

The different faces of cutaneous lupus erythematosus. G Ital Dermatol Venereol.2009 Apr;144(2):135-47.

Lupus erythematosus is a chronic and inflammatory multiorgan disease with variable clinical appearance and variable course. Most patients with systemic lupus erythematosus show cutaneous manifestations and conversely, all forms of cutaneous LE may change into a systemic involvement. Specific lesions of cutaneous LE are classified in different subtypes of acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), chronic cutaneous lupus erythematosus (CDLE) and intermittent cutaneous lupus erythematosus (ICLE) according to clinical, histological and immunoserological parameters. Regular laboratory tests are important to monitor the activity and course of the disease or side effects of the therapy. In case of clinical or laboratory dysfunctions of internal organs, additional technical investigations are necessary. Histology is needed to support clinical diagnosis. A large number of drugs are able to induce SCLE, e.g. hydrochlorothiazide, terbinafine, or angiotensin-converting enzyme inhibitors. Drug-induced SCLE can be differentiated by possible complementary immunoserological parameters. Neonatal lupus can be induced by transplacental transmission of maternal anti-Ro(SS-A) and anti-La(SS-B)-antibodies. Children with neonatal lupus might suffer from congenital atrioventricular block. Their mothers may suffer from active LE, but can be clinically healthy as well. As a consequence, pregnancies at risk should be monitored in short intervals by serial echocardiographic interventions. Protection against UV light is recommended for all types of CLE. There are some topical and many systemic treatment options e.g. topical and systemic glucocorticosteroids, antimalarial drugs, dapsone, azathioprine, or mycophenolate mofetil with different response to skin or organ involvement.

Cutaneous lupus erythematosus: issues in diagnosis and treatment.Am J Clin Dermatol. 2009;10(6):365-81. doi: 10.2165/11310780-000000000-00000.

Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. Three recognized subtypes of cutaneous LE are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). ACLE may be localized (most often as a malar or 'butterfly' rash) or generalized. Multisystem involvement as a component of systemic LE (SLE) is common, with prominent musculoskeletal symptoms. SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-Ro antibodies and may be induced by a variety of medications. Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic scarring and pigmentary change. Less common forms of CCLE include hyperkeratotic LE, lupus tumidus, lupus profundus, and chilblain lupus. Common cutaneous disease associated with, but not specific for, LE includes vasculitis, livedo reticularis, alopecia, digital manifestations such as periungual telangiectasia and Raynaud phenomenon, photosensitivity, and bullous lesions. The clinical presentation of each of these forms, their diagnosis, and the inter-relationships between cutaneous LE and SLE are discussed. Common systemic findings in SLE are reviewed, as are diagnostic strategies, including histopathology, immunopathology, serology, and other laboratory findings. Treatments for cutaneous LE initially include preventive (e.g. photoprotective) strategies and topical therapies (corticosteroids and topical calcineurin inhibitors). For skin disease not controlled with these interventions, oral antimalarial agents (most commonly hydroxychloroquine) are often beneficial. Additional systemic therapies may be subdivided into conventional treatments (including corticosteroids, methotrexate, thalidomide, retinoids, dapsone, and azathioprine) and newer immunomodulatory therapies (including efalizumab, anti-tumor necrosis factor agents, intravenous immunoglobulin, and rituximab). We review evidence for the use of these medications in the treatment of cutaneous LE.

Classification of lupus erythematosus based upon cutaneous manifestations. Dermatological, systemic and laboratory findings in 191 patients.Dermatology. 1995;190(4):277-83.

BACKGROUND: Lupus erythematosus (LE) is a multi-organ-system disease, the characteristics of which are reflected in the 1982 American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE). From a dermatological point of view, only the presence of LE-specific histopathology is necessary and sufficient for the diagnosis of LE. The association between the type of LE-specific skin lesion and the severity of extracutaneous manifestations of LE has not yet been investigated systematically.
OBJECTIVE: The aim of this study was to evaluate, according to the type of LE skin lesions, the prevalence of the 1982 criteria for SLE.
METHODS: We selected 191 patients whose skin lesions were histologically diagnosed as LE specific. Patients were classified on the basis of skin disease, and their clinical and laboratory data were analyzed.
RESULTS: Of 191 patients, 130 (68%) exhibited only one type of LE-specific skin lesion (monomorphic), 55 (29%) had two types (bimorphic) and the remaining 6 (3%) displayed three types (trimorphic). Nineteen of 22 (86%) patients who presented discoid lupus skin lesions above the neck without other eruptions were classified in the cutaneous-limited LE spectrum. Of 116 patients with acute lupus skin lesions (malar rash), 83 (72%) clearly fulfilled the 1982 ARA criteria for SLE. In skin lesions of LE profundus, chilblain lupus, subacute lupus (annular-polycyclic erythema and the papulosquamous variant), there were no significant correlations between the type of eruption and the severity of extracutaneous manifestations.
CONCLUSION: Patients with acute lupus skin lesions could usually be classified as suffering from SLE, whereas monomorphic patients with localized discoid lesions rarely exhibited extracutaneous manifestations. This tendency was less distinct in bimorphic patients. Almost all patients with subacute skin lesions were bimorphic or trimorphic, which might be due to genetic or racial differences between Japanese and other populations.

Markers in cutaneous lupus erythematosus indicating systemic involvement. A multicenter study on 296 patients.Acta Derm Venereol.1997 Jul;77(4):305-8.

Lupus erythematosus (LE) is an autoimmune disorder, involving the skin and/or other internal organs. As cutaneous variants, chronic discoid LE (CDLE) and subacute cutaneous LE (SCLE) usually have a better prognosis, however, involvement of internal organs with transition into systemic disease may occur. The aim of this study was to assess the significance of some clinical and laboratory criteria that could serve as markers for early recognition of systemic involvement in cutaneous LE. Three hundred and seventy-nine patients with LE, seen in five cooperating Departments of Dermatology during the years 1989-1994, were documented by electronic data processing according to a common protocol. Two hundred and forty-five of these patients had cutaneous LE (CDLE or SCLE), and 51 had systemic LE (SLE) and were included in this study. Forty-nine patients with either CDLE/SCLE or SLE were not evaluated because of incomplete documentation; also, 34 patients suffered from other LE subsets and were likewise excluded from the evaluation. Multivariate statistical analysis was used to assess the value of seven selected variables for distinguishing between the CDLE/SCLE and SLE groups: ESR, titers of antinuclear antibodies, anti-dsDNA-antibodies, photosensitivity, presence of arthralgias, recurrent headaches and signs of nephropathy. Univariate and multivariate analysis of the obtained data showed that signs of nephropathy (proteinuria, hematuria) was the variable with the highest statistical relevance for distinguishing between patients with cutaneous (CDLE/SCLE) and with systemic LE (SLE) in all statistical models tested, followed by the presence of arthralgias and of high ANA titers (> or =1:320). In contrast, low ANA titers as well as anti-dsDNA antibodies showed little or no statistical relevance as a criterion for distinction. It seems, therefore, that cutaneous LE patients showing signs of nephropathy, presence of arthralgias and elevated ANA titers (> or =1:320) should be carefully monitored, because they may be at risk of developing systemic LE involvement.