Authors: Dr Helen Gordon, Dermatology Registrar, New Zealand. Adjunct A/Prof. Amanda Oakley, Dermatologist, New Zealand. July 2019. Minor update Dr Ian Coulson, Dermatologist, June 2023.
Exposure to UVR results in increased expression of the Ro/SSA antigen on the surface of keratinocytes, binding the anti-Ro/SSA antibody and leading to the disease.
It is estimated 20-40% of cases of SCLE are drug-induced. The incubation time widely varies from a few days (such as in the case of the chemotherapy agents capecitabine and paclitaxel) to years before the onset of the rash (eg, a case was reported to arise 5 years after starting a thiazide diuretic).
Malignancy: there are increasing reports of SCLE being temporally associated with underlying cancers, including carcinoma of the lung, oesophagus, prostate, cholangiocarcinoma, and B-cell lymphoma.
What are the clinical features of subacute cutaneous lupus erythematosus?
SCLE most often presents as a non-scarring papulosquamouseruption.
Typically, there are annularplaques with raised erythematous borders and central clearing.
The plaques coalesce to form polycyclic patterns.
They may or may not have an overlying scale.
Sometimes, there are peripheralvesicles, crusting, and bullae.
The rash is typically symmetrically distributed on the sun-exposed sites of the neck, the upper trunk, and the outer arms.
The face is usually unaffected.
Lesions resolve with post-inflammatoryhypopigmentation; normal pigmentation recovers over time.
Other lupus-associated findings in patients with SCLE include:
Drug-induced lupus can be indistinguishable from the non-drug-induced form of SCLE. Specific features of drug-induced SCLE include:
Usually presenting in older patients
Association with a likely drug
Malar rash
Involvement of the legs
Bullous, targetoid, or vasculitic variants.
What are the complications of subacute cutaneous lupus erythematosus?
Around 50% of patients with SCLE meet the American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus (SLE).
A pregnant woman who is Ro/SSA antibody-positive has a risk of delivering an infant suffering from neonatal lupus erythematosus (8–10%) and congenital heart block (1–2%).
How is subacute cutaneous lupus erythematosus diagnosed?
SCLE can be diagnosed clinically, supported by the results of blood tests and a skin biopsy.
Around 60% of patients with SCLE are antinuclear antibody (ANA) positive
More than 80% are Ro/SSA antibody positive
La/SSB, dsDNA, anti-histone, and Sm antibodies are less common.
The histology on biopsy can resemble other forms of cutaneous lupus.
There is a lymphocyticinterfacedermatitis with basal layer degeneration.
Perivascular and periadnexal lymphocytic infiltrate, follicular plugging, basement membrane thickening, and dermalmucin are less prominent than in DLE.
Direct immunofluorescence reveals a granulardeposition of immunoglobulin (Ig)G, IgM, and C3 along the dermal–epidermal junction in approximately two-thirds of patients with SCLE (the lupus band test).
Drug-induced SCLE and non-drug-induced SCLE cannot be distinguished on histology.
What is the differential diagnosis for subacute cutaneous lupus erythematosus?
The differential diagnosis for SCLE includes other types of cutaneous lupus, especially:
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Xie F, Frewen J, Divekar P. Three cases of subacute cutaneous lupus erythematosus associated with malignancy: a late paraneoplastic phenomenon. Clin Exp Dermatol. 2020;45(5):607-608. doi:10.1111/ced.14176 Journal