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Lupus erythematosus is a multisystem disorder that frequently has cutaneous involvement
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Lupus-specific skin disease can be characterized as acute, subacute, or chronic based on clinical and laboratory features
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Other nonspecific cutaneous changes such as cutaneous vasculitis and Raynaud’s phenomenon occur more commonly in lupus patients
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Prevention involves protection from ultraviolet radiation and smoking cessation
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Topical and intralesional corticosteroids and other topical immunomodulators may be effective for mild or localized disease
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Antimalarials are the first-line systemic treatment, with other systemic agents reserved for more severe or recalcitrant disease
Lupus erythematosus (LE) is a multisystem disorder that encompasses a spectrum from a relatively benign, self-limited cutaneous eruption to a severe, sometimes fatal, systemic disease. Prior to Hargraves’ recognition of the LE cell, LE was diagnosed by a constellation of clinical findings. Ultimately, the American College of Rheumatology (ACR) developed a set of criteria that could be used for the classification of systemic lupus erythematosus (SLE). The criteria were revised in 1982 ( Table 1-1 ). When a patient fulfills four or more of the ACR criteria, either concurrently or serially, during any period of observation, that patient can be classified as having SLE.
| If four or more of the following criteria are present serially or simultaneously during any observation, the patient may be considered to have systemic lupus erythematosus: |
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In the 1940s and 1950s, dermatologists first recognized that most of their patients with chronic, scarring discoid lupus erythematosus (DLE) lesions had few, if any, systemic findings, whereas those with malar erythema and/or photosensitivity frequently had systemic disease. They also recognized a middle group in whom the cutaneous lesions were more transient than in patients with DLE, but for whom the prognosis was not as poor as in those patients with SLE. These patients were later categorized as having subacute cutaneous LE (SCLE). The classification of cutaneous LE subsets was stressed by Gilliam and his coworkers. Gilliam proposed that cutaneous manifestations characterized by interface dermatitis (histopathologically- specific LE) be classified into one of three groups based on clinical features. An individual LE patient can present with more than one subtype of the disease. Gilliam also recognized that LE patients can have a skin disease that is not histopathologically specific ( Table 1-2 ). Although each subset listed in Table 1-2 is generally predictive of outcome, it must be remembered that the full spectrum of LE-associated organ dysfunction is possible in any individual patient.
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The prevalence of SLE is reported to be 17–48/100,000 people. The prevalence of cutaneous LE is not well established, but it appears to be at least as common as SLE. SLE has a strong female preponderance, with a 12:1 female-to-male ratio in the childbearing years. Cutaneous LE appears to be more common than SLE in males and older adults, but remains more common in women, with a 3:1 female-to-male ratio.
Chronic Cutaneous Lupus Erythematosus
Chronic cutaneous LE can have several clinical manifestations. The most common subset is discoid lupus erythematosus (DLE). Patients with DLE may be classified as having either localized DLE, in which lesions are confined to the head and neck, or widespread DLE, in which lesions are found on other body surfaces in addition to the head and neck. DLE can also occur as a manifestation of SLE in approximately 20% of patients. Other less common forms of chronic cutaneous LE include hypertrophic LE, tumid LE, lupus erythematosus panniculitis (LEP, or lupus profundus), oral DLE, as well as DLE lesions on the palms and/or soles.
Discoid Lupus Erythematosus
DLE lesions are characterized by erythema; telangiectasia; adherent scale, which varies from fine to thick; follicular plugging; dyspigmentation; and atrophy and scarring ( Fig. 1-1 ). The lesions are usually sharply demarcated and can be round, thereby giving rise to the term discoid (or disc-like). The presence of scarring and/or atrophy is the characteristic that separates these lesions from those of SCLE. The differential diagnosis most often includes papulosquamous diseases such as psoriasis, lichen planus, secondary syphilis, superficial fungal infection, and sarcoidosis. A histopathologic examination is usually helpful in confirming the diagnosis, and only rarely is immunofluorescence microscopy necessary.
Patients with localized DLE have lesions located solely on the head, neck, or both. These appear to represent the majority of cases of DLE. These patients differ from those with widespread discoid lesions of LE in a number of ways. They have fewer manifestations that suggest systemic disease, and they less frequently demonstrate a positive antinuclear antibody (ANA) or leukopenia. It appears that patients with DLE who progress to develop SLE are generally not in the subset with localized discoid lesions of LE. Those patients with disease localized to the head and neck will frequently (roughly 50%) have a remission, whereas the disease rarely becomes clinically inactive (less than 10%) in those with widespread involvement. Lastly, it also appears that those with widespread disease respond less well to antimalarial treatment. Thus, it seems that it is prognostically worthwhile to separate patients with localized DLE and those with generalized DLE into different subsets.
Hypertrophic Lupus Erythematosus
Hypertrophic or verrucous DLE (HLE) is a unique subset in which the thick, adherent scale is replaced by massive hyperkeratosis, and the resulting lesions resemble verruca or squamous cell carcinomas ( Fig. 1-2 ). These lesions usually occur in the setting of other, more typical DLE lesions. Patients with HLE tend to have chronic disease, to have little in the way of systemic symptoms or abnormal laboratory findings, and to be extremely difficult to treat with conventional therapy. They may respond to oral retinoids.
Palmar/Plantar Discoid Lupus Erythematosus
The lesions of DLE can occur on the palms and/or soles ( Fig. 1-3 ). The frequency of this subset is low, and there is no specific clinical or serologic correlation. Patients with DLE of the palms or soles can have chronic cutaneous disease, or the lesions can be present in patients with SLE. Palmar and/or plantar lesions are often difficult to treat.
Oral Discoid Lupus Erythematosus
Oral DLE lesions are histopathologically and clinically similar to cutaneous discoid lesions of LE ( Fig. 1-4 ). Oral DLE lesions are distinct from the oral and nasal ulcerations that occur in SLE, which are associated with active systemic disease and are histopathologically nonspecific. Lesions that look like those of discoid lesions of LE in the oral mucosa have associations similar to those seen with localized or widespread discoid lesions of LE.
Tumid Lupus Erythematosus
Tumid lupus erythematosus (lupus tumidus) is characterized by erythematous to violaceous papules, plaques ( Fig. 1-5 ), or nodules, that usually occur on sun-exposed surfaces. The lesions classically have no epidermal changes and tend to heal with no residual scarring or atrophy. Patients with tumid LE are photosensitive. Serologic abnormalities are distinctly uncommon in these patients, and patients with tumid LE rarely meet criteria for SLE. The pathology of tumid LE reveals an increase in mucin and a periappendiceal and perivascular dermal infiltrate composed of lymphocytes, but there is little if any change at the dermal–epidermal interface. It is possible that there is overlap, both clinically and histologically, between reticulated erythematous mucinosis and tumid LE.
There are several controversies regarding tumid LE: (1) some authorities believe that tumid LE is not a variant of lupus erythematosus; and (2) as there is no residual scarring or atrophy, it differs from other types of chronic cutaneous lupus. Some have argued that it would fit better as a variant of subacute cutaneous lupus or in a separate classification. Patients with tumid LE are usually responsive to photoprotection and antimalarials.
Lupus Panniculitis
Lupus erythematosus panniculitis (LEP, lupus panniculitis) is a lobular panniculitis that occurs rarely in patients with DLE or SLE ( Fig. 1-6 ). Whether LEP is histopathologically distinct is controversial; thus, in the authors’ opinion, the patient should have documented SLE or DLE to be classified as having LEP. The term lupus profundus is sometimes used as a synonym for LEP, while others reserve this term for LEP with overlying discoid lesions. LEP is often chronic, and it can lead to cutaneous and subcutaneous atrophy, calcification, and occasional ulceration. Lesions preferentially involve the face as well as areas with prominent subcutaneous tissue, such as the upper arms, thighs, and buttocks. Twice as many patients with LEP do not have systemic disease as have systemic disease. It has been postulated that in the patient with LEP, renal disease is rarely present, and when present, it is among the more benign forms.
Chilblains Lupus
Chilblains lupus is a rare manifestation of LE that typically presents as red or dusky papules or plaques in acral locations ( Fig. 1-7 ) that are exacerbated by cold and may improve—but do not always resolve—in warmer weather. This entity can be extremely difficult to distinguish from “common” pernio in the absence of other features of LE, but lesions classically should have histological changes of LE, such as vacuolar interface changes. Lesions of chilblains lupus are often recalcitrant to typical treatments of cutaneous LE, such as antimalarials. Over time, lesions may develop an appearance of more typical DLE. In half of patients, chilblains may be the only presenting manifestation of LE. Long-term follow-up of these patients for signs of systemic disease is warranted.
DLE–SLE Subset
The DLE–SLE subset defines a small group of patients (about 5% to 15%) who, by the nature of their selection, have systemic disease in association with scarring cutaneous disease. Patients whose disease progresses from being purely cutaneous into this group are typically characterized by widespread DLE, the presence of clinically appreciable periungual telangiectases, persistent elevated erythrocyte sedimentation rates, leukopenia, and positive ANA titers. Patients in this group may have DLE alone at the onset, DLE with other symptoms or signs, or systemic disease without cutaneous lesions. The timeframe for developing SLE is variable—in one study, most patients with DLE developed the criteria for SLE within 1.3 years of diagnosis; in a more recent study, however, the mean time to developing SLE was 8.2 years. Patients in the DLE–SLE subset rarely have renal disease, and, when present, it is most often transient and mild. The DLE–SLE population is often considered to be a distinct LE subset because of its relatively benign, albeit chronic, course.
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