Discoid Lupus Erythematosus (DLE) is the most common form of chronic cutaneous Lupus Erythematosus (LE). Characteristic lesions cause disfigurement impacting quality of life, with 5% of progress into systemic LE (SLE). Prompt diagnosis avoids sequelae such as scarring, recurrence, and malignancy. Local therapy includes sun protection, steroids, and calcineurin inhibitors (CNIs). Antimalarials and surgical or cosmetic interventions are other options.

Summary Treatment of refractory cutaneous lupus is challenging. When conventional therapy, including hydroxychloroquine (HCQ), corticosteroids and immunosuppressants, has failed, the addition of quinacrine may be a promising option. We describe a case of refractory chronic cutaneous lupus erythematosus (CCLE) who responded well to quinacrine.
Quinacrine ; Lupus Erythematosus, Cutaneous

Child ; Female ; Humans ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Lupus Nephritis

No abstract available.
Herpes Zoster* ; Lupus Erythematosus, Cutaneous*

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.
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

Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.
Antibodies* ; Antibodies, Antinuclear ; Autoantibodies ; Autoimmune Diseases ; Biopsy ; Dermis ; Edema ; Epidermis ; Female ; Heart Block ; Humans ; Infant* ; Infant, Newborn ; Liver ; Lupus Erythematosus, Cutaneous ; Mothers* ; Serologic Tests ; Skin ; Telangiectasis

Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.
Antibodies* ; Antibodies, Antinuclear ; Autoantibodies ; Autoimmune Diseases ; Biopsy ; Dermis ; Edema ; Epidermis ; Female ; Heart Block ; Humans ; Infant* ; Infant, Newborn ; Liver ; Lupus Erythematosus, Cutaneous ; Mothers* ; Serologic Tests ; Skin ; Telangiectasis

The clinical manifestations and immunohistologic findings of drug-induced lupus erythematosus (DILE) are similar to those of idiopathic lupus. However, DILE is different from idiopathic lupus because it is induced after continuous drug exposure and resolves after discontinuation of the causative drug. DILE can be divided into systemic lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus (CCLE). Lupus erythematosus profundus is a subtype of CCLE, and drug-induced CCLE is very rarely reported in the literature. Herein, we report a rare case of adalimumab-induced lupus erythematosus profundus developed in a rheumatoid arthritis patient. The patient is a 43-year-old Korean woman who had multiple tender nodules and plaques on her face, trunk, and both extremities after using adalimumab for rheumatoid arthritis. She was diagnosed with adalimumab-induced lupus erythematosus profundus, and her condition improved after discontinuation of adalimumab.
Adult ; Arthritis, Rheumatoid* ; Extremities ; Female ; Humans ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Panniculitis, Lupus Erythematosus* ; Adalimumab

The clinical manifestations and immunohistologic findings of drug-induced lupus erythematosus (DILE) are similar to those of idiopathic lupus. However, DILE is different from idiopathic lupus because it is induced after continuous drug exposure and resolves after discontinuation of the causative drug. DILE can be divided into systemic lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus (CCLE). Lupus erythematosus profundus is a subtype of CCLE, and drug-induced CCLE is very rarely reported in the literature. Herein, we report a rare case of adalimumab-induced lupus erythematosus profundus developed in a rheumatoid arthritis patient. The patient is a 43-year-old Korean woman who had multiple tender nodules and plaques on her face, trunk, and both extremities after using adalimumab for rheumatoid arthritis. She was diagnosed with adalimumab-induced lupus erythematosus profundus, and her condition improved after discontinuation of adalimumab.
Adult ; Arthritis, Rheumatoid* ; Extremities ; Female ; Humans ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Panniculitis, Lupus Erythematosus* ; Adalimumab

BACKGROUND: Peculiar erythema known as annular erythema associated with Sjogren's syndrome (AESS) can be differentiated from autoimmune annular erythema and subacute cutaneous lupus erythematosus, both clinically and histologically. However, there are no detailed investigations on immune competent cells infiltration. OBJECTIVE: Preferential infiltration of interleukin-17-producing T helper (Th17) cells and regulatory T (Treg) cells into the labial salivary gland is reported to play a role in maintaining mucoepithelitis in patients with Sjogren's syndrome. In this study, we evaluated Th17 and Treg cell infiltration into the lesional skin of AESS. METHODS: We analyzed the numbers and infiltration patterns of Th17 and FoxP3 (+) Treg cells in seven cases of AESS using immunohistochemistry. Seven patients with systemic lupus erythematosus (SLE), atopic dermatitis (AD) and psoriasis vulgaris (PV), which are representatives of Th17 cell-involved skin disorders, were enrolled as disease controls. RESULTS: Periappendageal and epidermal changes, such as follicular plugging and liquefaction, were evident in the annular erythema of SLE, not AESS, tissue samples. In AESS tissue samples, dense perivascular and periappendageal infiltration of lymph cells was observed in the middle-to-deep dermis, as previously described, in contrast to the superficial infiltration pattern observed in both AD and PV samples. While the total number of infiltrated lymphocytes was similar between AESS and SLE tissue samples, Th17 cells were found to be preferentially infiltrated in the middle-to-deep dermis in AESS samples. CONCLUSION: These results suggest that an increased number and distribution of infiltration of Th17 cells is a preferential feature of AESS, rather than a characteristic feature of annular erythema of SLE.
Dermatitis, Atopic ; Dermis ; Erythema* ; Humans ; Immunohistochemistry ; Interleukin-17* ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Lymphocytes ; Psoriasis ; Salivary Glands ; Sjogren's Syndrome* ; Skin ; T-Lymphocytes, Helper-Inducer* ; T-Lymphocytes, Regulatory* ; Th17 Cells