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Subacute cutaneous lupus erythematosus in Child
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Subacute cutaneous lupus erythematosus in Child

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

In children, 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) associated with systemic lupus erythematosus:
  • 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.
  • Systemic findings are mild and less common compared to adults
  • Inflammatory infiltrate seen in the upper dermis on biopsy
Chronic cutaneous lupus erythematosus (CCLE; discoid lupus erythematosus):
  • Chronic discoid lesions with permanent disfiguring scars
  • Up to 25% of children go on to develop systemic findings
  • In discoid LE, significant inflammatory infiltrate seen in superficial and deep dermis as well as prominent involvement of the adnexa on biopsy
In children, SCLE is very rare and 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, and the extensor surfaces of the arms are the most commonly affected sites. Although scarring is not a characteristic finding, hypo- and hyperpigmentation and persistent telangiectasia are common sequelae.

There is a strong association with anti-Ro/SSA antibodies and SCLE. In a small number of cases reported, both sexes seem equally involved, and there is no association with particular ethnic groups. Although drugs are commonly associated with SCLE in adults, this has not been the case in children.

Sinopulmonary infections and meningitis have been associated with the 2 reported cases involving C2 deficiency. A rare patient had factor H deficiency, a protein controlling C3 catabolism.

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 in adults. There are no reports to date of drug-induced SCLE in the pediatric population.

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 – 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.
  • Erythema multiforme – Characteristic target-like lesions; tends to involve the palms.
  • Annular psoriasis – Biopsy will assist in differentiating psoriasis from SCLE.
  • Annular urticaria – Wheals that are characteristically pruritic.
  • 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.
  • Polymorphous light eruption – Most lesions resolve within several days.
  • Sarcoidosis – More infiltrative plaques.
Papulosquamous variant:

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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
Copyright © 2018 VisualDx®. All rights reserved.
Subacute cutaneous lupus erythematosus in Child
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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
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