Korean J Dermatol.  2017 Jun;55(5):293-296.

Systemic Lupus Erythematosus Presenting as Stevens-Johnson Syndrome: A Case Report

• Lee KG
• Koh BY
• Kwon ES
• Myung KB
• Kwon MH
• Cheong SH

Abstract

Acute cutaneous lupus erythematosus (ACLE) on the face is a usual pattern of presentation. However, it can rarely present with a generalized distribution. A hyperacute form of ACLE can mimic Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN). A 33-year-old man presented with erythematous eroded papules and patches on his head, neck, and upper chest over 2 months. Moreover, he showed hemorrhagic crusted erosions on his lips, and buccal and nasal mucosa, in addition to conjunctival injection. A skin biopsy from his cheek showed a mild degree of vacuolar alteration, thickening of the basement membrane, perivascular and periadnexal lymphohistiocytic infiltration, and stromal mucin deposition. Direct immunofluorescence (DIF) demonstrated IgG and IgM deposits along the basement membrane zone. Laboratory investigations demonstrated pancytopenia, positive antinuclear antibody (ANA), anti-double stranded DNA (anti-dsDNA), and anti-Ro antibodies. The patient was diagnosed with systemic lupus erythematosus (SLE) based on clinical, histological, and laboratory markers of autoimmune disease. We report a rare case of SLE presenting as SJS.

Keyword

Systemic lupus erythematosus; Stevens-Johnson syndrome

MeSH Terms

Adult
Antibodies
Antibodies, Antinuclear
Autoimmune Diseases
Basement Membrane
Biomarkers
Biopsy
Cheek
Cytochrome P-450 CYP1A1
DNA
Fluorescent Antibody Technique, Direct
Head
Humans
Immunoglobulin G
Immunoglobulin M
Lip
Lupus Erythematosus, Cutaneous
Lupus Erythematosus, Systemic*
Mucins
Nasal Mucosa
Neck
Necrosis
Pancytopenia
Skin
Stevens-Johnson Syndrome*
Thorax
Antibodies
Antibodies, Antinuclear
Biomarkers
Cytochrome P-450 CYP1A1
DNA
Immunoglobulin G
Immunoglobulin M
Mucins