Cutaneous Manifestations Of Lupus Erythematosus Research Articles

Systemic correlates of cutaneous manifestations of lupus erythematosus.

We aimed to investigate the prevalence of skin disease among patients with systemic lupus erythematosus (SLE) and determine whether LE skin disease had clinical or serologic correlates with SLE. We reviewed records of 335 patients with SLE (seen at Mayo Clinic, Rochester, Minnesota, USA) and abstracted skin manifestations, fulfilled mucocutaneous SLE criteria, and clinical and serologic parameters. Of the 231 patients with skin manifestations, 57 (24.7%) had LE-specific conditions, 102 (44.2%) had LE-nonspecific conditions, and 72 (31.2%) had both. LE skin disease was associated with photosensitivity, anti-Smith antibodies, and anti-U1RNP antibodies (all P < 0.001). Patients without LE skin disease more commonly had elevated C-reactive protein levels (P = 0.01). Patients meeting 2-4 mucocutaneous American College of Rheumatology criteria less commonly had cytopenia (P = 0.004) or anti-double-stranded DNA antibodies (P = 0.004). No significant associations were observed for systemic involvement (renal, hematologic, neurologic, and arthritis) when comparing patients with or without LE skin involvement. LE skin involvement was not significantly associated with internal SLE disease flare, number of medications, or overall survival. LE skin disease commonly occurs in patients with SLE. The presence of LE skin disease had no mitigating impact on the severity of SLE sequelae, disease flares, number of medications, or overall survival.

Direct Immunofluorescence in Cutaneous and Systemic Lupus Erythematosus: A Literature Review

Cutaneous manifestations of lupus erythematosus (LE) have a variety of clinical phenotypes and require proper investigation for diagnosis. Knowledge of the direct immunofluorescence (DIF) technique has improved and played an important role in the diagnosis of cutaneous LE. This review explores and summarizes reported DIF findings of each cutaneous LE variant. Historically, DIF findings of cutaneous LE have revealed deposits of multiple immunoreactants at the dermo-epidermal junction with either linear or granular patterns. Immunoglobulin M is the most common immunoreactant and DIF findings of cutaneous LE variants overlap. Therefore, diagnosis of cutaneous LE requires a combination of monitoring patient history, physical examinations and laboratory studies. This review helps interpret and better understand the application of DIF studies in cutaneous LE.

Lupus panniculitis: a rare bilateral and symmetrical manifestation of cutaneous lupus erythematosus in an adolescent girl.

Lupus panniculitis is included in the chronic cutaneous lupus erythematosus group. An 18-year-old female patient came with the complaint of lumps on her face. When she was 16 years old, the patient started to complain about lumps on her right lower arm. Lumps were observed on her left cheek and right chin during the ongoing treatment. Histopathology results showed lymphocyte infiltration in between lobular adipocyte with fibrotic and fat necrosis in the subcutis. Lupus panniculitis lesions in this patient were found both on her face and on her lower arms, which are not considered common predilection sites of lupus panniculitis. The skin lesion observed in this patient was also bilateral and symmetrical, which was a rare finding.

Cutaneous Manifestations of Lupus Erythematosus

Journal of the Dermatology Nurses’ Association 14(4):p E11, 7/8 2022. | DOI: 10.1097/JDN.0000000000000698

Cutaneous manifestations of lupus erythematosus: a practical clinicopathological review for pathologists.

Accurate diagnosis of connective tissue diseases is often challenging, and relies upon careful correlation between clinical and histopathological features, direct immunofluorescence studies and laboratory work-up. Lupus erythematosus (LE) is a prototype of connective tissue disease with a variety of cutaneous and systemic manifestations. Microscopically, cutaneous LE is classically characterised by an interface dermatitis although other histopathological patterns also exist, depending upon the clinical presentation, location and chronicity of the skin lesions. In this article, we review the clinical, serological, histopathological and direct immunofluorescence findings in LE-specific and LE non-specific skin lesions, with an emphasis upon lesser-known variants, newly described features and helpful ancillary studies. This review will guide general pathologists and dermatopathologists in accurately diagnosing and subclassifying cutaneous LE.

Cutaneous Manifestations of "Lupus": Systemic Lupus Erythematosus and Beyond.

Lupus, Latin for “wolf,” is a term used to describe many dermatologic conditions, some of which are related to underlying systemic lupus erythematosus, while others are distinct disease processes. Cutaneous lupus erythematosus includes a wide array of visible skin manifestations and can progress to systemic lupus erythematosus in some cases. Cutaneous lupus can be subdivided into three main categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Physical exam, laboratory studies, and histopathology enable differentiation of cutaneous lupus subtypes. This differentiation is paramount as the subtype of cutaneous lupus informs upon treatment, disease monitoring, and prognostication. This review outlines the different cutaneous manifestations of lupus erythematosus and provides an update on both topical and systemic treatment options for these patients. Other conditions that utilize the term “lupus” but are not cutaneous lupus erythematosus are also discussed.

Lupus Erythematosus: Cutaneous Manifestations and Treatment

Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic manifestations. Specific CLE - defined by the presence of an interface dermatitis on histopathological evaluation - is divided into several sub-types, namely acute CLE (ACLE), subacute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), as well as other rarer forms such as LE profundus (LEP). Nonspecific skin findings, such as livedo reticularis or purpura are more frequently seen in patients with systemic disease. Diagnosis requires classification of the subtype, through a combination of physical examination, laboratory studies, histology and sometimes direct immunofluorescence, at the same time ensuring to exclude systemic disease. Regarding the treatment of CLE, antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for recalcitrant disease. Patient education on proper sun protection and avoidance of triggers is crucial. This paper reviews the clinical manifestations of CLE, as well as the treatment.

Systemic Lupus Erythematosus

The cutaneous manifestations of lupus erythematosus (LE) include LE-specific and LE-nonspecific skin lesions. LE-specific skin lesions are divided into chronic, subacute, and acute types. The representatives of the chronic and acute types are discoid lupus erythematosus (DLE) and butterfly rash, respectively. Based on the systemic manifestations, we can classify LE into cutaneous-limited LE and systemic LE (SLE). Chronic LE eruptions tend to be seen in cutaneous-limited LE, and acute LE eruptions mainly appear in SLE. Some skin lesions are related to the neurological manifestations of SLE.

Obliterative Bronchiolitis as a Systemic Manifestation of Cutaneous Lupus Erythematosus.

Deshwal, Himanshu MD; Valeria Arrossi, A. MD; Parambil, Joseph G. MD Author Information

Obliterative Bronchiolitis as a Systemic Manifestation of Cutaneous Lupus Erythematosus

Deshwal, Himanshu MD; Valeria Arrossi, A. MD; Parambil, Joseph G. MD Author Information

Cutaneous Lupus Erythematosus in Children: Experience from a Tertiary Care Pediatric Dermatology Clinic.

The manifestations of cutaneous lupus erythematosus (CLE) and their relevance to systemic disease are well characterized in adults, but data are limited in children. The objective of the current study was to examine the spectrum of CLE and its relationship to systemic disease in children from a tertiary care pediatric dermatology clinic. An analysis of 26 children with CLE registered consecutively over 14 years was performed. Ninety-six percent of the patients were of Arab ethnicity. They included seven (27%) cases with neonatal lupus erythematosus (LE) (71% females and 29% males). Of the other 19 children with CLE, 95% were female. The mean and median age at diagnosis was 11 years. Eighty-nine percent of the patients fulfilled the criteria for systemic LE. All patients had LE-specific lesions and 83% had LE-nonspecific manifestations. Atypical initial presentations were recorded in 28% of the patients, and 22% of the patients had the rare LE variants. Of the LE-specific manifestations, acute CLE was seen in 83%, subacute in 44%, and chronic in 22%. Autoimmune associations were recorded in 44% and a positive family history of autoimmune diseases in 61%. This study highlights a striking female predominance, higher risk of systemic disease in children presenting with CLE, higher prevalence of atypical presentation and rare CLE variants, and underrepresentation of discoid LE in children and signifies the need for more surveys to delineate the spectrum of pediatric CLE in different parts of the world.

Genome-wide transcriptional profiling data from skin of chronic cutaneous lupus erythematosus (CCLE) patients.

Cutaneous features manifest as a wide range of clinically significant, and in many cases disfiguring and debilitating components of lupus erythematosus (LE). While the definitive etiology is in question, multifactorial and polygenic causes are likely to be involved in the production of the characteristic anti-nuclear autoantibody titers and immune cell infiltrates observed in chronic cutaneous LE (CCLE) [1–3]. There is significant overlap of patients with systemic and cutaneous manifestations of LE, which suggests shared pathways and genetic background between the two. We have employed genome-wide microarray technology along with pathway-based analyses to investigate transcriptional differences between lesional and non-lesional skin from CCLE patients to address existing gaps in knowledge regarding disease mechanisms in lupus [4].

Lupus erythematosus. Part II: Clinical picture, diagnosis and treatment

Lupus erythematosus (LE) is an important dermatologic autoimmune disease. 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 response to immunosuppressive agents which target B cells and T cells and their cytokines. A recently published article in this Journal summarized the most important knowledge about epidemiology, genetics, and immunology of LE. Here, the different clinical manifestations, diagnostic procedures and current therapeutic approaches will be described. Special emphasis is placed on different cutaneous manifestations of LE. In regard to treatment, the classic treatment approaches such as corticosteroids, methotrexate, chloroquine and hydroxychloroquine will be described. Lastly, new therapeutic approaches with specific monoclonal antibodies which are currently used in systemic LE, such as belimumab (Benlysta®), will be addressed. The most recent developments in this area could have implications even for purely cutaneous forms of LE.

Lupus Profundus (Panniculitis): A Potential Mimic of Subcutaneous Panniculitis-like T-Cell Lymphoma

Lupus profundus is a rare manifestation of cutaneous lupus erythematosus, seen in 1% to 3% of patients. It most commonly presents in association with classic chronic cutaneous lesions of discoid lupus erythematosus; however, such lesions, as well as a clinical history of lupus erythematosus, may be lacking. The differential diagnosis for lymphocytic lobular panniculitides is broad; however, the consideration of subcutaneous panniculitis-like T-cell lymphoma is most critical. Recently, there have been several reports emphasizing the overlapping histomorphologic features between lupus profundus and subcutaneous panniculitis-like T-cell lymphoma. Although this is controversial, some authors suggest that patients with lupus profundus are at risk for the development of abnormal, clonal T-cell proliferations and/or overt subcutaneous panniculitis-like T-cell lymphoma. In cases of atypical lymphocytic lobular panniculitis that fail to meet diagnostic criteria for subcutaneous panniculitis-like T-cell lymphoma, patients should be clinically followed indefinitely, as future subcutaneous lymphoma cannot be excluded.

Spring and summer eruption of the elbows: A peculiar localized variant of polymorphous light eruption

Polymorphous light eruption (PLE) is the most common form of idiopathic photodermatosis. Several morphologic variants of PLE have been described, including a localized form of PLE primarily affecting the helices of the ears. To our knowledge, the presence of lesions on the elbows as the unique manifestation of PLE has not yet been reported. We have studied 9 patients presenting with a recurrent eruption on the elbows, with clinical and histopathologic features indistinguishable from PLE, occurring during springtime. Recently, a peculiar eruption of the elbows, with similar clinical features to our patients, has been proposed as a manifestation of cutaneous lupus erythematosus. We sought to describe the clinical, histopathological, and immunohistochemical features of this peculiar eruption of the elbows. Nine patients presenting a recurrent spring eruption on the elbows, collected from April 1989 to June 2012, were retrospectively analyzed. We studied their clinical and histopathological features, and the immunophenotype of the infiltrate. Five patients were men and 4 were women. The mean age was 44.7 years. The lesions consisted of pruriginous, erythematous-edematous papules and plaques, located on both elbows. The eruption appeared during the spring or early summer and recurred seasonally. No associated symptoms were present and the eruption regressed spontaneously or with topical corticosteroids after 7 to 15 days. Histopathologically, the lesions showed typical features of PLE, with variable degree of edema in the papillary dermis, and a papillary and reticular dermal perivascular infiltrate mostly composed of small lymphocytes. Immunohistochemical studies demonstrated strong immunoreactivity for CD2, CD4, and CD8, revealing the infiltrate was composed predominantly of T lymphocytes, with a predominance of T-helper over T-cytotoxic lymphocytes. Immunostaining for CD123 was negative, highlighting the absence of plasmacytoid dendritic cells. Other T- and B-cell markers, including CD30, PD-1, CXCL13, FoxP3, CD79a, and CD56 were also negative. Retrospective case series design is a limitation. Phototests were not performed. Results of antinuclear antibodies were only available in 1 patient. We believe this recurrent eruption of the elbows represents a distinctive and localized variant of PLE rather than a peculiar manifestation of cutaneous lupus erythematosus and suggest the term "spring and summer eruption of the elbows" for this peculiar condition. The mechanism of this localization on the elbows, with sparing of other photoexposed areas, remains unknown.

Pulsed dye laser as an excellent choice of treatment for lupus tumidus: a prospective study

Clinical manifestations of cutaneous lupus erythematosus (CLE) vary widely, and different subtypes of this autoimmune disease exist. Tumidus subtype (LT) has been recently separated from the chronic subtypes and is now considered an independent entity due to its particular clinical and histological features. Different treatments are usually prescribed for CLE. Our group has experience of CLE effectively treated with pulsed dye laser (PDL). It was our impression that better responses were achieved in the LT subtype, but no controlled prospective studies with PDL have been specifically performed. A prospective study was performed on 10 patients with histologically confirmed LT treated with PDL. All patients were treated with 595 nm PDL using the 10 mm spot size at 0.5 ms pulse width and a fluence of 8 J/cm(2). Biopsies were taken before and 4 weeks after treatment and were stained with haematoxylin-eosin. Evaluation after PDL treatment showed clinical improvement in all of them without side-effects and reduction of the dermal lymphocytic infiltrate in 9/10 of the patients. Epidermal changes were absent in all patients. Mucin deposition persisted only in one patient. However, 50% of the patients developed new lesions nearby or distant to the treated zones. PDL therapy is an effective and fast treatment option for acute flares of LT; however, it does not prevent recurrences. A histological improvement has been confirmed in this study. Purpura seems to be necessary to achieve a good response.

Cytokines in cutaneous lupus erythematosus

Clinical manifestations of cutaneous lupus erythematosus are diverse and different subtypes of the disease have been defined. It is characteristic for lupus skin inflammation that a significant activation of innate interferon pathways is observable. Endogenous molecules indicative for tissue damage (damage-associated molecular patterns [DAMPs]) show cytokine-like properties once released from intracellular compartments and these DAMPs have been shown to influence the course of inflammatory responses. Cytokines including TNF-α and IL-1 family members significantly contribute to the chronic inflammatory phenotype and, in combination with DAMPs, impact on the activation of innate interferons. A better understanding of the deregulated cytokine network in cutaneous lupus erythematosus patients with distinct disease phenotypes and genetic backgrounds is of high importance and a prerequisite for recommending and developing more individualized therapeutic strategies.

Moulage: "Lupus erythematosus".

The first description of lupus erythematosus was given by Pierre Louis Alphee Cazenave, who was a coworker of Laurent-Theodore Biett, in 1851. He used the French term “lupus erythemateux.” In 1856 Ferdinand Hebra translated this work into German and coined the term lupus erythematosus, which is the Latin translation, and in 1863 Isidor Neumann propagated the Greek term “lupus erythematodes.” In 1869 Moritz Kaposi published a comprehensive description of the cutaneous manifestations of lupus erythematosus. [1] The Federal Pathologic-Anatomical Museum in Vienna houses 36 moulages with the diagnosis of lupus erythematosus, one of which is shown on the cover of the first issue of Dermatology: Practical and Conceptual (Figure 1). The oldest one dates back to 1894. Most moulages originate from patients of Moritz Kaposi and Gustav Riehl, who succeeded his teacher Kaposi as the professor of Dermatologie und Syphilis at the University of Vienna in 1902. Figure 1. Moulage. “Lupus erythematosus”. Federal Pathologic-Anatomical Museum. Vienna, Austria, 1894. Moulages are wax preparations (molds from real patients) made for teaching purposes. All moulages at the Federal Pathologic-Anatomical Museum in Vienna were fabricated by the manufacturer Karl Henning and his son Theodor.

Update on the use of topical calcineurin inhibitors in cutaneous lupus erythematosus

Cutaneous manifestations of lupus erythematosus (CLE) are manifold, presenting with unspecific skin manifestations or well-defined clinical dermatological entities. Their relation to each other as well as to systemic lupus erythematosus is variable, yet diagnostically and therapeutically challenging. Therapeutic decisions have to be based on the activity and distribution as well as the type of skin lesions and the extent of systemic disease. Limited skin manifestations may be amply tackled by topical therapy, so far, mainly relying on corticosteroids. In many cases, however, internal treatment has to be combined by using antimalarials, in addition to strict UV-protection. The advent of topical calcineurin inhibitors has contributed substantially to the armamentarium of external treatment options. By specifically interfering with intracytoplasmic signal transduction to activate the nuclear factor of activated T-cells (NF-AT), they are able to modulate various inflammatory mechanisms. The two available compounds, pimecrolimus and tacrolimus, do not induce the skin atrophy characteristic of corticosteroids. They have been studied in a number of case reports, but only in a few randomized, comparative studies. Both are well-tolerated, but differentially effective in the various subsets of CLE. Further studies are needed to directly compare the two compounds to each other, as well as to topical corticosteroids, before final recommendations can be made.

Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates

The skin is the second most frequently affected organ system in lupus erythematosus. Although only very rarely life threatening--an example is lupus erythematosus-associated toxic epidermal necrolysis--skin disease contributes disproportionally to disease burden in terms of personal and psychosocial wellbeing, vocational disability, and hence in medical and social costs. Since several manifestations are closely associated with the presence and activity of systemic lupus erythematosus, prompt and accurate diagnosis of cutaneous lupus erythematosus is essential. This review aims to cover common, rare, and atypical manifestations of lupus erythematosus-associated skin disease with a detailed discussion of histopathological correlates. Cutaneous lupus erythematosus covers a wide morphological spectrum well beyond acute, subacute and chronic cutaneous lupus erythematosus, which are commonly classified as lupus-specific skin disease. Other uncommon or less well-known manifestations include lupus erythematosus tumidus, lupus profundus, chilblain lupus, mucosal lupus erythematosus, and bullous lupus erythematosus. Vascular manifestations include leukocytoclastic and urticarial vasculitis, livedoid vasculopathy and livedo reticularis/ racemosa. Finally, we discuss rare presentations such as lupus erythematosus-related erythema exsudativum multiforme (Rowell syndrome), Kikuchi-Fujimoto disease, extravascular necrotizing palisaded granulomatous dermatitis (Winkelmann granuloma), and neutrophilic urticarial dermatosis.