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

Introduction: Lupus panniculitis (LP) is an unusual type of cutaneous lupus erythematosus (CLE) wherein the cutaneous inflam- matory reaction involves primarily the deeper dermis and subcutaneous fat. It is characterized by the appearance of recurrent, mostly asymptomatic, firm, nodules or plaques, involving the face, upper limbs, and buttocks. Case report: In our case, a 30-year-old female presented with a non-tender, non-movable nodule on the left breast, 6 weeks prior to her admission. She had fever, chills, and joint pains. The patient later developed hyperpigmented plaques on the infra- clavicular area, and left flank extending to the abdomen. Urinalysis showed proteinuria, and RBC cast. She also had leukopenia, and anemia on CBC. Chest computerized tomography (CT) scan revealed a heterogeneously enhancing soft tissue mass in the base of the neck at the right infraclavicular region with malignant features. ANA titer was normal, while skin biopsy on two sites and direct immunofluorescence studies were compatible with lupus panniculitis. She was managed as a case of systemic lupus erythematosus (SLE) using a combination of hydroxychloroquine, and oral corticosteroids, which afforded temporary relief of symptoms. The patient however was lost to follow-up and opted for alternative medicine, and subsequently succumbed to the complications of SLE. Conclusion This case highlights the importance of a carefully made assessment after an accurate clinicopathological cor- relation was done. This case also emphasizes that although LP if associated with SLE may signify a milder condition, judicious monitoring and follow-up must still be undertaken since management is based on the disease activity.
Panniculitis, Lupus Erythematosus

Introduction: Squamous cell carcinoma (SCC) is a rare complication of long-standing discoid lupus erythematosus (DLE). The reported risk factors associated with the malignant transformation include chronic inflammation, scarring, immunosuppression, and the dysfunctional healing already evident in discoid lesions. If underdiagnosed and left untreated, this may lead to higher rates of recurrences, metastases and death. Objective: The main objective of this article is to report the diagnosis and management of a DLE-associated SCC on the upper lip of an elderly Filipino female. A short discussion on the pathogenesis and treatment of this disease entity is also presented.ugs/topical medications, family history and accompanying systemic symptoms were reviewed and analyzed. Case: A 78-year-old female, pawid maker, developed a rapidly growing verrucous tumor measuring 4.0 x 2.0 x 2.0 cm from a depigmented atrophic plaque located on the upper lip. Initial histopathologic findings were consistent with SCC and DLE. Considering the tumor size and the challenging anatomical location, the surgical oncology and plastic surgery team carried out a wide excision with 5 mm margin of the entire upper lip mass and surrounding plaque, followed by lip reconstruction. Biopsy findings of the excised mass were consistent with the initial findings. Three months post-operation, there was significant wound healing with no recurrences. Conclusion: Although the incidence is rare, SCC occurring in DLE is considered to be high-risk and should warrant early recognition and prompt treatment. In this paper, the authors also highlight the use of a standard wide excision in lieu of Mohs Microscopic Surgery as an alternative option where resources are limited.
surgical flaps ; Lupus Erythematosus, Discoid ; Carcinoma, Squamous Cell

Imiquimod, a toll-like receptor agonist, is a topical immunomodulator that induces the production of several cytokines including interferon-alpha, which shows antifibrotic properties. We hypothesized that the antifibrotic effect of imiquimod would soften fibrotic skin lesions. Therefore, we applied topical imiquimod with topical tacrolimus or systemic acitretin in patients with refractory lesions of myxedema, angiolymphoid hyperplasia, and generalized discoid lupus erythematosus and confirmed improvement in fibrotic lesions in these patients. Thus, we conclude that use of imiquimod improves fibrotic skin lesions. This report describes our experience with the treatment of this condition along with a review of the related literature.
Acitretin ; Angiolymphoid Hyperplasia with Eosinophilia ; Cytokines ; Fibrosis ; Humans ; Hyperplasia ; Interferon-alpha ; Lupus Erythematosus, Discoid ; Myxedema ; Skin ; Tacrolimus ; Toll-Like Receptors

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

Monogenic autoimmune diseases (AD) present as lupus-like clinical manifestations with recurrent fever or various vasculopathies. Recurrent fever with an elevation of acute phase reactants and various skin lesions are similar in monogenic AD and autoinflammatory disease. The molecular pathogenesis of adult systemic erythematosus can be understood through monogenic AD based on gene defects: complement, apoptosis, interferonopathy via nucleic acid sensing, tolerance, rasopathies, and others. Skin vasculopathy with chilblains and livedo reticularis, interstitial lung disease, and panniculitis are common occurrences in type I interferonopathy. Some syndromes have been reported to present with autoimmune inflammation and the general clinical findings, including cerebral calcification. Various clinical manifestations in monogenic AD present in accordance with the gene loss- or gain-of-function mutations involved. The monogenic AD for the early onset of more severe lupus-like symptoms or vasculopathy needs to be considered. Furthermore, clinical trials were conducted via targeted therapy for related molecular pathways, because conventional treatments were not effective in managing monogenic AD.
Acute-Phase Proteins ; Adult ; Apoptosis ; Autoimmune Diseases* ; Chilblains ; Complement System Proteins ; Fever ; Humans ; Inflammation ; Interferons ; Livedo Reticularis ; Lung Diseases, Interstitial ; Lupus Erythematosus, Systemic ; Panniculitis ; Skin

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

Vitiligo is a multifactorial disorder. Neural, biochemical, and autoimmune mechanisms have been hypothetically suggested as etiopathological contributors to this condition. Autoimmunity focuses primarily on genetic factors and the association between vitiligo and other autoimmune disorders including autoimmune thyroid disease, rheumatoid arthritis, psoriasis, type 1 diabetes, pernicious anemia, and Addison's disease. We describe a 35-year-old man with systemic lupus erythematosus who developed concurrent vitiligo and discoid lupus erythematosus suggesting the possible autoimmune association between these 2 different diseases.
Addison Disease ; Adult ; Anemia, Pernicious ; Arthritis, Rheumatoid ; Autoimmunity ; Humans ; Lupus Erythematosus, Discoid ; Lupus Erythematosus, Systemic* ; Psoriasis ; Thyroid Diseases ; Vitiligo*

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