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Subacute cutaneous lupus erythematosus in Adult
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Subacute cutaneous lupus erythematosus in Adult

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Contributors: Mehdi Rashighi MD, Belinda Tan MD, PhD, Susan Burgin MD
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Synopsis

Cutaneous lesions of lupus erythematosus can be classified into specific and nonspecific types. There are 3 main specific subtypes based on morphology and distribution, chronicity, association with systemic lupus erythematosus (SLE), and histologic features including location / depth of inflammatory infiltrate. They are as follows:

Acute cutaneous lupus erythematosus (ACLE)
  • Transient cutaneous findings typified by malar erythema without scarring
  • Strongly associated with systemic findings
  • Inflammatory infiltrate seen in the superficial dermis on biopsy
Subacute cutaneous lupus erythematosus (SCLE)
  • Photosensitive cutaneous eruption lasting longer than ACLE but without scarring
  • 10%-15% of patients go on to have systemic findings
  • Inflammatory infiltrate seen in the upper dermis on biopsy
Chronic cutaneous lupus erythematosus (CCLE)
  • Often interchangeably used to refer to its most common variant, discoid lupus erythematosus (DLE), but also includes the less common variants lupus tumidus, lupus panniculitis, and chilblain lupus
  • DLE presents with chronic discoid lesions with permanent disfiguring scars most often seen on the scalp, face, and ears
  • 5%-15% of patients go on to develop systemic findings
  • In DLE, significant inflammatory infiltrate seen in superficial and deep dermis as well as prominent involvement of the adnexa on biopsy
SCLE is characterized by annular plaques with raised borders and central clearing or papulosquamous lesions that are restricted to sun-exposed skin. The sides of the face, the lower neck, the upper trunk, and the extensor surfaces of the arms are the most commonly affected sites. Although scarring is not a characteristic finding, dyspigmentation is common sequelae.

While the etiology remains poorly understood, there is a strong association with anti-Ro/SSA antibodies and SCLE. It is hypothesized that a complex interplay between genetic proclivity and environmental influences leads to a perpetuated autoimmune response. In addition, specific HLA types (1, B8, DR3, DRw52, DQ1, DQ2, and DRw6) have been shown to be associated with this specific subtype of cutaneous lupus erythematosus. Risk factors for developing cutaneous lesions include sex (3:1 female-to-male ratio, especially during childbearing years) and ethnicity, with individuals of African descent demonstrating a higher incidence compared with individuals of Northern European descent. Epidemiologic studies have shown that people of Hispanic, Asian, African American, and African Caribbean descent have an increased incidence of lupus in addition to excess morbidity.

Of note, certain drugs such as antihypertensives (hydrochlorothiazide, calcium channel blockers, and angiotensin-converting enzyme [ACE] inhibitors), antifungals (terbinafine), nonsteroidal anti-inflammatory drugs (NSAIDs), proton pump inhibitors (omeprazole), and, more recently, various chemotherapeutic agents (such as paclitaxel) and tumor necrosis factor (TNF)-alpha antagonists have been reported to trigger SCLE. These drug-induced SCLE lesions run an unpredictable course, and they may not clear completely after discontinuing the offending drug.

Photosensitivity is a prominent feature. Approximately 50% of patients meet the American Rheumatism Association (ARA) criteria of SLE, largely based on laboratory values and mucocutaneous findings. However, the incidence of severe systemic involvement such as renal or central nervous system involvement is relatively low in this population of lupus patients. About 10% of patients can develop acute cutaneous lupus or lesions of discoid lupus (chronic cutaneous lupus). In addition, anti-Ro/SSA antibodies are also seen in Sjögren syndrome, and some patients can have both SCLE and Sjögren syndrome.

Codes

ICD10CM:
L93.1 – Subacute cutaneous lupus erythematosus

SNOMEDCT:
239891002 – Subacute cutaneous lupus erythematosus

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Annular variant:
  • Granuloma annulare – Mainly seen in children and young adults. Biopsy will help differentiate granuloma annulare and SCLE; facial lesions are extremely rare.
  • Tinea corporis – Usually has scale at the leading edge. Check potassium hydroxide (KOH) prep.
  • Erythema marginatum – Seen more commonly in children; cutaneous feature of acute rheumatic fever.
  • Polymorphous light eruption – Most lesions resolve within several days.
  • Erythema multiforme – Characteristic targetoid lesions; tends to involve the palms.
  • Annular psoriasis – Biopsy will assist in differentiating psoriasis from SCLE.
  • Erythema annulare centrifugum (EAC) – Mostly seen on hips and thighs in patients in their 50s; biopsy can help differentiate EAC from SCLE. Usually has scale trailing the leading edge.
  • Urticaria multiforme
Papulosquamous variant:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 07/31/2018
Last Updated: 10/09/2018
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Subacute cutaneous lupus erythematosus in Adult
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Subacute cutaneous lupus erythematosus : Rash, Arm, Neck, Photosensitivity, Superior chest, Upper back, Annular configuration, Thick scaly plaques
Clinical image of Subacute cutaneous lupus erythematosus
A close-up of annular and arcuate, scaly, erythematous plaques and nearby similar papules.
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