Subacute cutaneous lupus erythematosus in Child
Alerts and Notices
Synopsis
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
- 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 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
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
Look For
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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.
- Psoriasis
- Lichen planus – Pruritic scaly papules that involve the wrists, forearms, genitalia, and presacral area; biopsy will assist in differentiating lichen planus from SCLE.
- Syphilis (see secondary syphilis) – Check rapid plasma reagin (RPR).
- Drug-induced photosensitive reaction
- Drug-induced photoallergic reaction
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.Subscription Required
References
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Last Reviewed:07/30/2018
Last Updated:10/08/2018
Last Updated:10/08/2018
Subacute cutaneous lupus erythematosus in Child