Systemic Lupus Erythematosus Presenting as Stevens-Johnson Syndrome: A Case Report.
- Author:
Kyoung Geun LEE
1
;
Byoung Yong KOH
;
Eun Sun KWON
;
Ki Bum MYUNG
;
Mi Hye KWON
;
Seung Hyun CHEONG
Author Information
1. Department of Dermatology and Division of Rheumatology, Konyang University College of Medicine, Daejeon, Korea. mabie@kyuh.ac.kr
- Publication Type:Case Report
- Keywords:
Systemic lupus erythematosus;
Stevens-Johnson syndrome
- MeSH:
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
- From:Korean Journal of Dermatology
2017;55(5):293-296
- CountryRepublic of Korea
- Language:Korean
-
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.
