OBSOLETE: Acute cutaneous lupus erythematosus

Acute cutaneous lupus erythematosus (ACLE) is a dermatological manifestation of systemic lupus erythematosus (SLE) that primarily affects the skin. It is characterized by the classic malar or 'butterfly' rash, which presents as an erythematous eruption across the cheeks and bridge of the nose, typically sparing the nasolabial folds. ACLE can also present in a generalized form with widespread photosensitive maculopapular lesions affecting sun-exposed areas of the body, including the arms, hands, and upper trunk. The skin lesions are often triggered or worsened by ultraviolet light exposure. ACLE is almost always associated with active systemic lupus erythematosus, meaning that patients frequently have involvement of other organ systems including the joints, kidneys, blood, and cardiovascular system. The condition reflects underlying autoimmune dysregulation in which the body produces autoantibodies against its own tissues. Diagnosis is supported by clinical presentation, serological findings (such as anti-double-stranded DNA antibodies and antinuclear antibodies), and skin biopsy. Note: This Orphanet entry (code 163528) is marked as OBSOLETE, meaning it has been retired or reclassified within the Orphanet nomenclature. Patients and clinicians should refer to the broader classification of cutaneous lupus erythematosus or systemic lupus erythematosus for current clinical guidance. Treatment of ACLE typically involves sun protection, topical corticosteroids, antimalarial agents such as hydroxychloroquine, and management of the underlying systemic disease with immunosuppressive therapies when indicated.

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