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INTRODUCTION — Cutaneous lupus erythematosus (cutaneous LE) includes three subsets of LE-specific skin diseases: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE) (table 1). CCLE encompasses discoid lupus erythematosus (DLE), lupus erythematosus tumidus (LE tumidus), lupus profundus (also known as lupus panniculitis), chilblain lupus erythematosus (chilblain LE), and lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome).
Cutaneous LE can occur as a manifestation of systemic lupus erythematosus (SLE) or independent of SLE. The varying strengths of association between SLE and the individual subtypes of cutaneous LE are best illustrated in a graphic (figure 1). Patients with SLE may also develop a variety of LE-nonspecific skin diseases, cutaneous disorders that lack histologic features of LE, but occur with increased frequency in patients with SLE.
An overview of the various clinical manifestations of cutaneous LE and LE-nonspecific skin diseases is provided here. In-depth discussions of SLE and the treatment of DLE and SCLE are provided separately. (See "Overview of the clinical manifestations of systemic lupus erythematosus in adults" and "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults" and "Initial management of discoid lupus and subacute cutaneous lupus" and "Management of refractory discoid lupus and subacute cutaneous lupus".)
CLASSIFICATION — The modified Gilliam grouping system for cutaneous manifestations of LE provides a helpful organizational framework for the related but distinct clinical entities that comprise LE-specific and LE-nonspecific skin diseases. The three subcategories under LE-specific skin disease and their major clinical variants include:
●Acute cutaneous lupus erythematosus (ACLE)
•Localized ACLE (ie, malar rash, butterfly rash)
•Toxic epidermal necrolysis-like ACLE
●Subacute cutaneous lupus erythematosus (SCLE)
•Less common variants: erythrodermic, poikilodermatous, erythema multiforme-like (Rowell syndrome), and vesiculobullous annular SCLE
●Chronic cutaneous lupus erythematosus (CCLE)
•Discoid lupus erythematosus (DLE)
•Lupus erythematosus tumidus (LE tumidus)
•Lupus profundus (also known as lupus panniculitis)
•Chilblain lupus erythematosus (chilblain LE)
•Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome)
The key characteristic that unites the LE-specific skin diseases is histopathology. Common shared histopathologic features include hyperkeratosis; epidermal atrophy; vacuolar interface dermatitis (liquefactive degeneration of the basal layer of the epidermis); a superficial, perivascular, and perifollicular mononuclear cell inflammatory infiltrate; thickening of the basement membrane; and pigment incontinence . All of these features are not present in all variants. In particular, interface dermatitis is a consistent histopathologic feature of ACLE, SCLE, and discoid lupus erythematosus (the most common form of CCLE) but is not a typical feature of LE tumidus or lupus profundus. Interface dermatitis may also be seen in non-LE disorders, such as dermatomyositis.
In addition, most LE-specific skin diseases can occur in association with SLE, with the exception of LE tumidus, for which associated SLE is rare (figure 1). LE-specific diseases may also occur in conjunction with other LE-specific skin diseases and have a similar approach to treatment. (See 'Management' below.)
The designations "acute," "chronic," and "subacute" do not necessarily or strictly reflect the duration of activity of the skin disease. The "acute" in ACLE reflects the often transient and recurrent course of ACLE and the tendency for exacerbations of ACLE to occur during acute flares of SLE; in addition, the terminology was originally coined in reference to a lack of residual long-term skin damage, dyspigmentation, or scarring. The "chronic" in CCLE reflects both the often prolonged course of CCLE and the resulting chronic skin changes of dyspigmentation and scarring that occur in DLE. SCLE may lead to longstanding skin dyspigmentation but typically does not cause scarring.
Although ACLE, SCLE, and the variants of CCLE are described as distinct entities, patients may develop more than one form of cutaneous LE. Many patients, up to 30 percent in some reports, may have overlap between subsets of cutaneous LE, particularly SCLE and DLE .
ASSOCIATION WITH SLE — Cutaneous disease is common in systemic lupus erythematosus (SLE); approximately 80 percent of patients develop skin disease at some point in their disease course. However, cutaneous LE frequently exists independently of SLE and may be two to three times more prevalent than SLE [3-6].
The association with SLE varies among the subtypes of cutaneous LE and mainly has been estimated from cross-sectional studies and retrospective studies [6-8]. Studies evaluating cross-sectional percent prevalence of underlying SLE in patients with cutaneous LE have suggested the following levels of association with SLE (these data do not reflect incident cohort data):
●ACLE – >90 percent 
●SCLE – 48 to 50 percent 
●Localized DLE – 5 to 10 percent 
●Generalized DLE – 15 to 28 percent 
●Lupus profundus/panniculitis – 5 to 10 percent 
●Lupus erythematosus tumidus – Rarely associated with SLE 
It is important to again note that these data represent the cross-sectional co-prevalence between cutaneous LE and SLE, rather than a prospective incidence. A graphical representation of the associations between the subtypes of cutaneous LE and SLE is provided (figure 1).
LUPUS ERYTHEMATOSUS-SPECIFIC SKIN DISEASE — Acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE) comprise LE-specific skin disease.
Acute cutaneous lupus erythematosus — ACLE is a manifestation of systemic lupus erythematosus (SLE) that may present as a characteristic localized facial eruption, less commonly as a generalized eruption, and rarely as a toxic epidermal necrolysis (TEN)-like presentation . Localized ACLE appears in approximately one-half of patients with SLE. Almost all patients with ACLE have SLE.
●Clinical manifestations – The facial eruption of localized ACLE (also known as "malar rash" or "butterfly rash") is characterized by erythema in a malar distribution (cheeks and bridge of the nose) (picture 1A-B). The nasolabial folds are spared.
Localized ACLE may precede other symptoms of SLE by months or even years or may be accompanied by other symptoms and signs of acute SLE. The involved skin feels warm and appears slightly edematous. The erythema may last for hours, days, or weeks and often recurs, particularly with sun exposure. In darker skin types, postinflammatory hyperpigmentation or hypopigmentation may persist even after the acute inflammatory stage has resolved.
Generalized ACLE presents as an erythematous maculopapular (morbilliform) eruption involving primarily sun-exposed skin. The extensor surfaces of the arms and hands are common sites. Notably, the skin overlying the knuckles often is spared, a feature that contrasts with dermatomyositis. Occasionally, the inflammatory infiltrate is severe enough to produce vesicles or bullae. Severe cases can resemble TEN-like ACLE [14-18]. (See "Clinical manifestations of dermatomyositis and polymyositis in adults" and "Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis".)
●Histopathology – The classic histologic findings of localized and generalized ACLE are consistent with interface dermatitis and include apoptotic keratinocytes, vacuolization of the basal cell layer of the epidermis, a lymphohistiocytic infiltrate in the superficial dermis, and dermal mucin deposition . The findings can be subtle.
●Differential diagnosis – Localized ACLE should be distinguished from rosacea. Rosacea can present with malar erythema and may be exacerbated by sun exposure (picture 2). Features that favor a diagnosis of rosacea include involvement of the nasolabial fold, papules or pustules (papulopustular variant), and exacerbation of erythema with typical rosacea triggers (spicy foods, caffeine, heat, etc). Although rarely necessary, a skin biopsy can distinguish ACLE from rosacea . (See 'Diagnosis' below and "Rosacea: Pathogenesis, clinical features, and diagnosis".)
Other causes of facial erythema that may be confused with ACLE include sunburn, seborrheic dermatitis, contact dermatitis, erysipelas, flushing (idiopathic or associated with carcinoid syndrome, pheochromocytoma, or mastocytosis), and dermatomyositis. A skin biopsy can aid in distinguishing most of these disorders from ACLE. An exception is dermatomyositis, which exhibits similar pathologic findings. A helpful clinical feature of facial eruptions in dermatomyositis is the tendency to involve the nasolabial folds; in ACLE, the nasolabial folds are spared (picture 3). (See "Approach to the patient with facial erythema" and "Rosacea: Pathogenesis, clinical features, and diagnosis" and "Clinical manifestations of dermatomyositis and polymyositis in adults".)
Subacute cutaneous lupus erythematosus — SCLE frequently is associated with SLE [21,22]. Approximately 50 percent of affected patients meet the 1997 American College of Rheumatology (ACR) classification criteria for SLE, but subsequent studies have revealed that approximately 10 to 15 percent of patients presenting with SCLE go on to develop severe clinical manifestations of SLE (eg, serious central nervous system or renal disease) (table 2) . SCLE can also occur as a result of drug exposure. (See "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults" and 'Drug-induced SCLE' below.)
The ACR classification criteria are known to have inherent limitations, which include classifying SLE too readily in patients with SCLE. Based upon SCLE features alone, at least 3 or 4 of 11 criteria are often met (ie, SCLE patients may have photosensitivity, positive antinuclear antibodies [SSA/Ro+], mild arthralgias, and/or oral ulcers, which could suggest the diagnosis of SLE). However, these patients may have no other systemic end-organ involvement or other SLE features. In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) proposed revised classification criteria that addressed some of these limitations regarding the cutaneous disease manifestations (table 2). It should be noted that the ACR and SLICC criteria were developed for study purposes and have limited use in clinical practice. (See "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults", section on 'Classification criteria'.)
There is a strong association between SCLE, human leukocyte antigen (HLA)-DR3, antibodies to Ro/SSA, and polymorphisms in the tumor necrosis factor (TNF)-alpha promoter gene [23,24]. More than 80 percent of patients with SCLE are positive for anti-Ro/SSA antibodies [25,26]. SCLE has also been associated with homozygous deficiencies of the second component of complement [27,28]. (See "The anti-Ro/SSA and anti-La/SSB antigen-antibody systems".)
●Clinical manifestations – SCLE begins as small, erythematous, slightly scaly papules that evolve into either psoriasiform plaques (papulosquamous SCLE) or annular plaques (annular SCLE) (picture 4A-B). The latter often coalesce to form polycyclic or figurative patterns. The plaques are typically erythematous with variable amounts of overlying scale. The most common sites of involvement are somewhat photodistributed and include the shoulders, forearms, neck, and upper torso. Despite a photoaggravated nature of the condition, the face is often spared . Dyspigmentation at sites of resolved SCLE is common and may resemble vitiligo. Scarring usually does not occur. Less common variants include vesiculobullous annular, poikilodermatous, erythrodermic, and erythema multiforme-like (Rowell syndrome) SCLE. Drug-induced SCLE is reviewed separately. (See 'Drug-induced SCLE' below.)
Most patients with SCLE exhibit photosensitivity, with exacerbations of disease stimulated by sun exposure. A case-control study of 76 patients with SCLE (including 48 who also fulfilled ACR criteria for SLE) and 24 patients with SLE without SCLE found photosensitivity nearly twice as prevalent in patients with SCLE than in patients with SLE without SCLE (86 versus 46 percent, respectively) . Associated arthralgias and oral ulcers are reported; however, serious features such as cytopenias and serositis occur much less frequently.
●Histopathology – Compared with discoid lupus erythematosus (DLE), the histopathology of SCLE shows less follicular plugging and hyperkeratosis, and the perivascular and appendageal lymphocytic infiltrates tend to be more superficial. There is vacuolization of the basement membrane and mucin deposition in the dermis. Basement membrane thickening is generally absent or minimal (picture 5A-B) [22,30]. (See 'Discoid lupus erythematosus' below.)
●Differential diagnosis – The differential diagnosis of SCLE includes other disorders that may exhibit erythematous papules or plaques such as psoriasis, tinea corporis, nummular eczema, dermatomyositis, cutaneous T cell lymphoma, and drug eruptions . If the diagnosis is uncertain, a biopsy can distinguish SCLE. (See 'Diagnosis' below.)
Drug-induced SCLE — Many classes of drugs have been implicated in SCLE, including antihypertensive drugs, lipid-lowering agents, proton pump inhibitors, antifungal agents, TNF-alpha inhibitors, and others (table 3) [10,32-35]. A case-control study that included 234 patients with incident SCLE found that more than one-third of cases appeared related to a drug exposure . (See "Drug-induced lupus", section on 'Drug-induced cutaneous lupus'.)
Drug-induced SCLE may be indistinguishable from idiopathic SCLE clinically, histopathologically, and immunologically. However, a retrospective study of 11 patients with drug-induced SCLE and 79 patients with idiopathic SCLE suggests that drug-induced SCLE tends to be more severe or widespread, is more likely to have a bullous or vasculitic component, exhibits a higher predilection for the face (eg, malar rash), and affects older individuals . Tissue eosinophilia does not help to distinguish idiopathic from drug-induced SCLE .
Drug withdrawal often leads to improvement in drug-induced SCLE. In the retrospective study of 11 patients with drug-induced SCLE and 79 patients with idiopathic SCLE, all cases of drug-induced disease resolved after drug withdrawal. The mean time to resolution was seven weeks and Ro/SSA antibodies eventually disappeared in 8 of the 10 patients who previously tested positive for these antibodies . Similarly, most patients in a retrospective study of 15 patients with drug-induced SCLE experienced improvement in the clinical manifestations of SCLE within eight weeks of drug withdrawal and a decrease in anti-Ro/SSA antibodies within eight months following drug withdrawal .
Neonatal lupus — Neonatal lupus shares a variety of features with classic SCLE; therefore, some clinicians consider the cutaneous manifestations of this entity a subtype of SCLE. Similarities with classic SCLE include clinical features (arcuate erythematous plaques that resolve without scarring (picture 6)), histologic findings (interface dermatitis), and an association with anti-Ro/SSA antibodies. (See 'Neonatal lupus' below and "Neonatal lupus".)
Chronic cutaneous lupus erythematosus — CCLE includes:
●Discoid lupus erythematosus (DLE)
●Lupus erythematosus tumidus (LE tumidus)
●Lupus profundus (lupus panniculitis)
●Chilblain lupus erythematosus (chilblain LE)
●Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome)
The most common type of CCLE is DLE, accounting for 73 to 85 percent of CCLE [7,37].
Discoid lupus erythematosus — It is estimated that 15 to 30 percent of patients with SLE develop DLE [1,38,39]. Patients with localized or generalized DLE are estimated to have cross-sectional prevalences of concurrent SLE between 5 and 28 percent. (See 'Association with SLE' above.)
The presence of DLE lesions among patients with SLE may modify the risk of specific SLE features. Compared with SLE patients without DLE, those with DLE have increased risk for photosensitivity and leukopenia but decreased risk for serositis and arthritis. There is no obvious change in risk of nephritis despite variable reports of a "renal-protective effect" of the presence of discoid lesions among SLE patients [40,41].
Data on the risk for progression of DLE to SLE are limited to retrospective cohort studies, studies lacking power to detect statistical significance of potential markers of progression, and studies that do not address DLE specifically as a CLE subset. In these studies, progression to SLE has occurred in 0 to 28 percent of patients initially presenting with DLE [7,39,42]. Progression to DLE often is delayed; in a Swedish-based population cohort study, 10 percent of patients with DLE who subsequently developed SLE did so within the first year and 17 percent developed SLE within the first three years . Two retrospective studies have suggested SLE develops within five years in 50 percent of the DLE patients who indeed go on to develop SLE . Risk factors for progression include an increasing number of clinical and serologic features of SLE: more widespread DLE lesions, arthralgias and arthritis, high antinuclear antibody (ANA) titers, leukopenia, and high erythrocyte sedimentation rates [10,43,44].
It is worth noting that patients with DLE and other mucocutaneous manifestations of LE may meet ACR classification criteria for SLE without having other end-organ disease . Revised classification criteria (the SLICC criteria) have been proposed to address some limitations of the ACR criteria. (See "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults", section on 'Classification criteria'.)
●Clinical manifestations – The classic findings of DLE are discrete, erythematous, somewhat indurated plaques covered by a well-formed adherent scale that extends into dilated hair follicles (follicular plugging). The plaques tend to expand slowly with active inflammation at the periphery and then heal, leaving depressed central scars, atrophy, telangiectasias, and hyperpigmentation and/or hypopigmentation . DLE most often involves the face, neck, and scalp but may also occur on the ears (particularly conchal bowls) and, less frequently, on the upper torso (picture 7A-D). Localized DLE is limited to sites above the neck. Generalized DLE refers to DLE occurring both above and below the neck.
Hypertrophic DLE is an uncommon clinical variant of DLE characterized by the development of hyperkeratotic, verrucous plaques .
●Histopathology – Pathologic examination of DLE typically reveals hyperkeratosis, follicular plugging, basal layer vacuolar changes, and a mononuclear cell infiltrate (predominantly T cells) near the dermal-epidermal junction, dermal blood vessels, and appendages (picture 8) . The basement membrane is usually thickened, a change that is best appreciated with a periodic acid-Schiff (PAS) stain, and there is dermal mucinosis. Although immunofluorescence microscopy may be positive in cutaneous LE, its use is limited by false-positive results on sun-exposed skin and by negative results in longstanding chronic LE lesions. (See 'Direct immunofluorescence (lupus band test)' below.)
●Differential diagnosis – The cutaneous differential diagnosis of DLE includes:
•Granuloma faciale (picture 9A-B)
•Lupus vulgaris (cutaneous tuberculosis)
•Lymphoproliferative disorders of skin (benign or malignant)
•Cutaneous leishmaniasis (eg, lupoid leishmaniasis, leishmaniasis recidivans)
Verrucous/hypertrophic discoid lupus may be confused with hypertrophic lichen planus, keratoacanthoma, squamous cell cancer, and prurigo nodularis. (See 'Diagnosis' below.)
Less common subtypes — LE tumidus, lupus profundus (lupus panniculitis), chilblain LE, and lupus erythematosus-lichen planus (LE-LP) overlap syndrome are additional manifestations of CCLE.
Lupus erythematosus tumidus — Although often categorized as a form of LE-specific skin disease, the rarity of association with concurrent SLE and the lack of interface dermatitis on pathology have raised debate about where LE tumidus falls in the classification of cutaneous lupus disorders. In a series of 40 patients with LE tumidus, only 10 percent were ANA-positive . An increased incidence among smokers has been noted .
●Clinical manifestations – LE tumidus is characterized by photodistributed, chronic, pink to violaceous, urticarial or edematous plaques or nodules [47,49,50]. Annular plaques may occur (picture 10A-B). Scale and scarring are absent.
●Histopathology – There is a moderate to dense, superficial and deep, perivascular lymphocytic infiltrate, consisting of predominately CD3+/CD4+ lymphocytes. In addition, there is mucin deposition in the papillary and reticular dermis. Interface changes at the dermal-epidermal junction are absent in most cases. A minority of patients exhibit focal interface changes .
●Differential diagnosis – The differential diagnosis of LE tumidus includes causes of erythematous to violaceous plaques with absent surface change, such as benign lymphocytic infiltration of skin (Jessner's disease), polymorphous light eruption, pseudolymphoma, B cell lymphoma, plaque mucinosis, and solar urticaria. (See 'Diagnosis' below.)
Lupus profundus (lupus panniculitis) — Lupus profundus (also known as lupus panniculitis) is an uncommon form of CCLE. Coexistent DLE occurs in at least one-third of patients with lupus profundus; SLE is present in approximately 10 percent of patients [11,51].
●Clinical manifestations – Lupus profundus presents as indurated plaques or nodules with or without overlying cutaneous changes . The plaques or nodules may appear on the scalp, face, upper arms, chest (particularly breasts), lower back, flank, upper thighs, or buttocks and are often tender or painful. Infrequently, patients develop ulceration or calcifications at sites of involvement. Upon resolution, lupus profundus may leave depressed areas of lipoatrophy (picture 11).
●Histopathology – Histopathologic examination reveals perivascular infiltrates of mononuclear cells plus panniculitis, manifested as hyaline fat necrosis with mononuclear cell infiltration and lymphocytic vasculitis (picture 12A-B). The presence of immune deposits in the dermal-epidermal junction on direct immunofluorescence offers support for the diagnosis . (See 'Diagnosis' below.)
●Differential diagnosis – Nodules of lupus profundus on the breast may raise concern for a breast malignancy. The possibility of subcutaneous panniculitis-like T cell lymphoma, which often manifests with subcutaneous nodules or plaques on the trunk or extremities, also should be considered . A biopsy will distinguish lupus profundus from these entities. (See 'Diagnosis' below.)
Chilblain lupus erythematosus — Chilblain LE is diagnosed in patients with clinical findings of pernio (also known as chilblains) in conjunction with clinical or laboratory features of cutaneous or systemic LE. Approximately 25 percent of patients who present with pernio meet classification criteria for SLE, and additional patients (5 to 6 percent in one study) may fulfill SLE criteria subsequently . (See "Pernio (chilblains)".)
Similar to idiopathic pernio, chilblain LE presents with tender, bright red to reddish-blue papules, nodules, or plaques on the toes, fingers, nose, or ears precipitated by cold exposure (picture 13) . Nodules on acral areas may ulcerate. Chilblain LE is reviewed separately. (See "Pernio (chilblains)", section on 'Chilblain lupus erythematosus'.)
Chilblain LE is distinct from lupus pernio, a subtype of sarcoidosis. (See "Cutaneous manifestations of sarcoidosis", section on 'Lupus pernio'.)
Lupus erythematosus-lichen planus overlap syndrome — LE-LP overlap syndrome is a rare, chronic disorder that has clinical, histopathologic, and immunofluorescence findings of both LE and lichen planus [57,58].
●Clinical manifestations – The cutaneous findings of LE-LP overlap syndrome are usually persistent atrophic blue-red to violaceous plaques or patches. The most common sites for involvement are the acral portions of the extremities, particularly the palms and soles. The nails are commonly involved, and there may be anonychia (absence of the nail). Photosensitivity and pruritus are generally absent.
●Histopathology – Histopathologic examination may reveal features of lichen planus (hyperkeratosis, hypergranulosis, irregular acanthosis, pigment incontinence) and LE. Similarly, direct immunofluorescence (DIF) microscopy may reveal features of lichen planus (cytoid bodies staining for immunoglobulin M [IgM] and fibrin in a fibrillar pattern) and LE (immunoglobulin and complement deposition in a linear granular pattern along the dermal-epidermal junction) . (See "Lichen planus".)
Diagnosis — In general, cutaneous LE is largely a clinical diagnosis supported by contextual clinical features (such as the presence of known underlying SLE). Confirmatory histopathologic examination is indicated when diagnostic uncertainty remains (eg, atypical clinical presentation or clinical features that overlap with other cutaneous diseases). For example, localized ACLE often can be diagnosed by recognition of erythema in the classic malar distribution in a patient with known SLE, and clinical recognition of an eruption with the classic morphology and distribution of DLE is acceptable for the diagnosis of DLE. In contrast, the skin lesions of lupus profundus are often biopsied because of the nonspecific appearance of cutaneous nodules, and tumid lupus erythematosus is often biopsied because of the nonspecific clinical features and absence of an association with SLE. The overlap in the appearance of SCLE with other papulosquamous or annular skin disorders often warrants a biopsy; however, in a patient who also exhibits photosensitivity and positive SSA/Ro antibodies, diagnosis without a biopsy is reasonable.
Performance of direct immunofluorescence is of variable utility if the diagnosis remains uncertain after clinical and histologic evaluation. (See 'Direct immunofluorescence (lupus band test)' below.)
Other than the strong association between Ro/SSA antibodies for SCLE, there are no antibodies that are routinely predictive of variants of CLE. (See 'Subacute cutaneous lupus erythematosus' above.)
Given the association of cutaneous LE with SLE, patients with cutaneous LE should be evaluated for SLE. While no clear guidelines exist for SLE screening or monitoring of the patient first presenting with cutaneous LE, obtaining a clinical history with a particular focus on a rheumatologic review of systems is recommended. A physical examination and select laboratory studies also should be performed. The evaluation for SLE is reviewed in detail separately. (See "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults" and "Systemic lupus erythematosus (SLE) in children: Clinical manifestations and diagnosis".)
If systemic features of SLE or laboratory abnormalities are present, referral to a rheumatologist is appropriate for further evaluation and comanagement.
Direct immunofluorescence (lupus band test) — Direct immunofluorescence (DIF) evaluation of lesional or nonlesional skin for deposition of a continuous band of immunoreactants along the dermal-epidermal junction as part of the evaluation for cutaneous lupus or SLE is historically referred to as the "lupus band test." Use of the terms "lesional lupus band test" and "nonlesional lupus band test" can help to clarify the source of the tissue used for the test. Overall, DIF is of unclear value for the diagnosis of cutaneous LE. Therefore, we do not routinely perform DIF.
Occasionally, demonstration of a continuous band of immunoreactants at the dermal-epidermal junction in tissue taken from a site of cutaneous LE (ie, lesional lupus band test) is used to support a diagnosis of cutaneous LE when histologic findings are nondiagnostic (picture 14) [60,61]. Although these findings are detected in lesional skin from most patients with ACLE, the finding is not pathognomonic [62,63]. In particular, biopsies from sun-exposed skin from individuals without cutaneous LE or SLE have demonstrated similar features, as shown in a prospective study of 50 healthy young adults that found deposition of immunoglobulins at the dermal-epidermal junction in 10 of 50 biopsy specimens (20 percent) taken from sun-exposed skin . A subsequent study that did not find immunoreactant deposition in sun-exposed skin from 41 healthy adults may have yielded different results because of differences in protocol or other factors .
Management — The approach to the treatment of LE-specific skin disease is influenced by the subtype of disease and the presence of underlying SLE. In all cases, photoprotection and use of appropriate broad-spectrum sunscreens are recommended, given the known photoexacerbation of cutaneous LE. (See "Selection of sunscreen and sun-protective measures" and "Initial management of discoid lupus and subacute cutaneous lupus", section on 'Photoprotection'.)
Topical and intralesional corticosteroids, oral glucocorticoids, oral antimalarial drugs, and glucocorticoid-sparing immunomodulatory agents have all been utilized depending on extent of disease and response to first- and second-line therapies. The general approach to management of cutaneous LE is as follows:
First-line therapy typically involves:
●Use of topical or intralesional corticosteroids, topical calcineurin inhibitors, and/or systemic glucocorticoids depending on the extent of involvement and subset of disease
Second-line therapy typically involves glucocorticoid-sparing and immunomodulatory therapy and should take into account underlying SLE end-organ manifestations, if present:
●Methotrexate (oral or subcutaneous): additional benefits may include treatment of SLE with inflammatory arthritis component
●Mycophenolate mofetil: may be of dual-benefit to patients with underlying lupus nephritis
Other second- or third-line agents may include:
The treatment of DLE and SCLE is discussed in detail separately. (See "Initial management of discoid lupus and subacute cutaneous lupus", section on 'Photoprotection' and "Management of refractory discoid lupus and subacute cutaneous lupus".)
LUPUS NONSPECIFIC SKIN DISEASE — Cutaneous disorders that occur with increased frequency among patients with systemic lupus erythematosus (SLE), but are not specific to SLE and lack histopathologic features of cutaneous LE comprise LE-nonspecific skin disease (table 4). Of note, many of these lupus nonspecific skin diseases are reported to occur with flares of underlying SLE.
Vascular abnormalities — Examples of cutaneous manifestations of vascular involvement in SLE include periungual erythema, livedo reticularis, telangiectasia, Raynaud phenomenon, and vasculitis. Cutaneous vascular abnormalities occur in approximately 50 percent of patients with SLE .
●Periungual erythema – Periungual erythema is due to dilated tortuous loops of capillaries and a prominent subcapillary venous plexus along the base of the nail. Similar findings have been noted along the edges of the upper eyelid.
●Livedo reticularis – Livedo reticularis refers to a reddish-cyanotic, reticular pattern on the skin of the arms, legs, and torso, particularly with cold exposure (picture 15A-B). In SLE, livedo reticularis is induced by vasospasm of the dermal ascending arterioles . Vasospasm in these cutaneous vessels results in decreased blood supply to the superficial horizontal vascular plexus, with a secondary increase in circulation to the remaining patent vessels. Pathologic examination of involved blood vessels reveals thickening of the walls of the dermal vessels with subsequent narrowing of the lumens and, in some cases, intravascular thrombi.
●Raynaud phenomenon – Raynaud phenomenon in SLE is a vasospastic process that occurs in approximately 15 to 30 percent of patients . It is characterized by blanching of the nail beds, fingers, and toes (and occasionally ears, nose, tongue, and nipples) with accompanying pain. Involvement of the thumb or severe disease that leads to distal digital ulceration should raise suspicion for features of overlap with systemic sclerosis. (See "Clinical manifestations and diagnosis of the Raynaud phenomenon".)
●Vasculitis – Vasculitis develops in approximately 11 to 20 percent of patients with SLE [67,68,70]. The most common form, occurring in 10 to 15 percent of cases, is urticarial vasculitis. In contrast to urticaria, lesions of urticarial vasculitis may persist for more than 24 hours and frequently evolve into painful petechiae or purpura that may heal with hyperpigmentation. (See "Urticarial vasculitis".)
Vasculitis may also affect small arteries, possibly resulting in microinfarcts of the tips of the fingers, the toes, the cuticles of the nail folds (splinter hemorrhages), and the extensor surface of the forearm and shin (picture 16). The palms of the hand, soles of the feet, and area around the ankle are less commonly involved . Ankle involvement may develop into painful punched-out ulcers and may heal slowly. Periarteritis nodosa-like lesions may occur.
Nonscarring alopecia — Nonscarring (reversible) hair loss in patients with SLE may reflect telogen effluvium or "lupus hair." Nonscarring hair loss usually responds well to treatment of SLE.
Telogen effluvium is characterized by a shift in follicular cycling that leads to premature shedding of hair. Telogen effluvium can occur in the setting of serious illness or other significant physiologic stressors. (See "Telogen effluvium".)
"Lupus hair" is generally seen during exacerbations of SLE. It is characterized by thin, unruly hair that easily fractures . Lupus hair usually occurs along the frontal hairline.
Other — Nail abnormalities, particularly pitting, ridging, and onycholysis, have been noted in 25 percent of patients with SLE . Approximately 20 percent of patients have redness of the lunula, a finding nearly always associated with periungual erythema .
Papulonodular mucinosis is another LE-nonspecific skin disorder. This disorder typically presents as asymptomatic, skin-colored papules or nodules on the trunk or proximal extremities and abundant mucin in the papillary dermis and mid-dermis .
Multiple disseminated eruptive dermatofibromas have been reported in SLE, especially in immunosuppressed patients .
Additional disorders included among LE-nonspecific skin disorders include :
●Nonspecific bullous eruptions (resulting from damage to the basal layer of the epidermis)
●Palisaded neutrophilic and granulomatous dermatitis
OTHER MANIFESTATIONS OF LUPUS
Mucosal manifestations — Mucous membrane involvement can occur in the setting of cutaneous LE or systemic lupus erythematosus (SLE). Mucosal involvement occurs in 12 to 45 percent of patients with SLE [77-79].
Oral involvement may manifest as white plaques, areas of erythema, or punched-out erosions or ulcers with surrounding erythema on the soft or hard palate or buccal mucosa (picture 17). The oral ulcers are usually painless. Oral ulcers may be the first sign of SLE. There is no apparent association between the presence of oral ulcers and systemic activity.
The oral manifestations of LE may demonstrate the typical histopathology of discoid lupus erythematosus (DLE), including hyperkeratosis, atrophy of rete processes, and superficial and deep inflammatory infiltrates; edema in the lamina propria, continuous or patchy periodic acid-Schiff (PAS)-positive deposits in the basement membrane zone, deposition of intercellular mucin, and deposits of immunoglobulin and complement at the dermal-epidermal junction are also seen [79,80]. Oral LE should be distinguished from lichen planus, candidiasis, aphthous stomatitis, intraoral herpes, Behçet's syndrome, bite marks, leukoplakia, and malignancy.
Nasal ulcers occur in some patients with SLE . They are usually in the lower nasal septum and tend to be bilateral. The appearance of nasal ulcers tends to parallel other features of active SLE. Nasal perforation, possibly secondary to vasculitis, is infrequent, occurring in 4.6 percent of 885 patients with SLE who were prospectively followed . Involvement of the mucosa of the upper airway may also occur and may cause hoarseness .
Oral mucous membrane lesions may respond to topical corticosteroids, tacrolimus 0.1% ointment, intralesional corticosteroids, and systemic antimalarial drugs. The response to topical corticosteroids (usually Orabase mixed with either triamcinolone 0.1% or clobetasol 0.05%) takes a few days to weeks, while the response to hydroxychloroquine takes weeks to months. If the oral LE is refractory to these interventions and is causing significant symptoms, more aggressive systemic therapies used for cutaneous LE may be tried. (See "Management of refractory discoid lupus and subacute cutaneous lupus".)
Bullous cutaneous lupus erythematosus — Bullous cutaneous lupus erythematosus (bullous CLE) is a rare and distinct complication of SLE characterized by the development of autoantibodies against type VII collagen and subepidermal blistering [83,84]. Affected patients develop a vesicular or bullous eruption that may affect any body site, including oral mucosa (picture 18A). There is a predilection for the upper trunk, upper extremities, and neck. Bullae may arise on normal or erythematous skin. Pruritus is usually absent. Dyspigmentation may occur in sites of resolved bullae. Scarring usually does not occur.
Typical biopsy findings in bullous CLE include subepidermal blistering and a neutrophil-predominant infiltrate in the upper dermis and dermal edema (picture 18A-B). Vasculitis may be present. Direct immunofluorescence studies demonstrate deposition of immunoglobulin (Ig)G, IgA, IgM, and/or complement at the basement membrane zone .
Oral dapsone is the mainstay of treatment. Responses to dapsone are often rapid.
Neonatal lupus — Neonatal lupus is a rare syndrome that is associated with maternal antibodies to Ro/SSA, to La/SSB, and, much less frequently, to U1RNP. Infants develop eruptions characterized by erythematous arcuate patches or plaques with raised active margins shortly after birth (picture 6). Congenital heart block is the most concerning complication of neonatal lupus. Following the birth of an infant with neonatal lupus, the risk for congenital heart block is increased with subsequent pregnancies . Of note, treatment with hydroxychloroquine may decrease the risk of neonatal lupus (congenital heart block) in at-risk pregnancies . Neonatal lupus erythematosus is reviewed separately. (See "Neonatal lupus".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●Basics topic (see "Patient education: Discoid lupus (The Basics)")
SUMMARY AND RECOMMENDATIONS
●Cutaneous lupus erythematosus (cutaneous LE) may occur as an independent disorder or in association with systemic lupus erythematosus (SLE). Cutaneous LE includes three subsets of LE-specific skin disease: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE). A variety of non-LE cutaneous disorders may also occur in patients with SLE (LE-nonspecific skin diseases). (See 'Classification' above.)
●The subtypes of LE-specific skin disease have varying strengths of association with SLE (figure 1). Whereas ACLE almost always occurs in association with SLE, other types of lupus-specific skin disease are less strongly associated with SLE. (See 'Association with SLE' above.)
●ACLE may occur as a localized, generalized, or toxic epidermal necrolysis-like eruption. The most common manifestation is the localized facial eruption (malar rash, butterfly rash), which is characterized by the development of erythema over the cheeks and bridge of the nose (picture 1A-B). ACLE may last for hours, days, or weeks and often recurs. (See 'Acute cutaneous lupus erythematosus' above.)
●SCLE may occur as an idiopathic eruption, in association with SLE, or as a drug-induced disorder. SCLE classically presents as psoriasiform or annular erythematous plaques on the shoulders, forearms, neck, or upper torso (picture 4A-B). There is a strong association with Ro/SSA autoantibodies. The possibility of drug-induced SCLE should always be reviewed, particularly when skin involvement is widespread or severe. (See 'Subacute cutaneous lupus erythematosus' above.)
●Discoid lupus erythematosus (DLE) is the most common form of CCLE. Patients with DLE develop erythematous, scaly plaques that may exhibit follicular plugging and heal with scarring (picture 7A-D). Associated hypopigmentation and hyperpigmentation are common. Frequent sites for DLE are the face, neck, scalp, and ears. Patients with the generalized variant of DLE also have involvement of the trunk or extremities. A hypertrophic variant of DLE is characterized by hyperkeratotic, verrucous plaques. LE tumidus, lupus profundus, chilblain LE, and lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome are additional subtypes of CCLE. (See 'Chronic cutaneous lupus erythematosus' above.)
●Cutaneous LE is largely a clinical diagnosis supported by contextual clinical features (such as the presence of known underlying SLE). Confirmatory histopathologic examination is indicated when diagnostic uncertainty remains. Select patients with known SLE and/or classic clinical features of cutaneous LE may not require a biopsy. (See 'Diagnosis' above.)
●The approach to the management of cutaneous LE is influenced by the extent of disease, subtype of cutaneous LE, response to initial therapy, and the presence of underlying SLE. Photoprotection, topical corticosteroids, topical calcineurin inhibitors, and oral antimalarials are common first-line treatments. (See 'Management' above.)
●LE may affect mucous membranes. Oral involvement may manifest as white plaques, erythema, erosions, or ulcers. Oral LE should be distinguished from lichen planus, candidiasis, aphthous stomatitis, intraoral herpes, Behçet's syndrome, bite marks, leukoplakia, and malignancy. (See 'Mucosal manifestations' above.)
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