ABSTRACT
Pemphigus vulgaris (PV) stands as a rare autoimmune disorder characterized by blistering and erosion of mucocutaneous membranes. The pathogenesis of PV implicates both B and T cells, which target cell-to-cell adhesion molecules within the epithelia of the skin and oral mucosa, leading to acantholysis. Typically, the presentation involves blistering of the oral mucosa, often followed by cutaneous lesions. Given the considerable risk of morbidity and mortality associated with PV, early diagnosis is crucial, typically relying on a combination of clinical features, histopathology, and direct immunofluorescence. Bruton tyrosine kinase (BTK) plays a significant role in the pathophysiology of autoimmune diseases and inflammation. Herein, we present a case of PV that demonstrated resistance to first-line therapy with steroids. Subsequently, treatment with the BTK inhibitor ibrutinib was initiated, yielding favorable outcomes. This case underscores the potential of targeted therapies, such as BTK inhibitors, in managing PV refractory to conventional treatment modalities.
ABSTRACT
Lichen planus pigmentosus (LPP) is a rare form of lichen planus that typically affects middle-aged people with darker-pigmented skin. LPP is associated with a longer clinical course than classical lichen planus, which distinguishes it clinically. Its occurrence in children is uncommon, with few reported cases in this population in the literature. We report a rare presentation of unilateral blaschkoid LPP in a seven-year-old Saudi Arabian female patient.
ABSTRACT
Atopic dermatitis (AD) is a chronic immune-mediated disease characterized by intense pruritis, causing inflammation, itching, and redness of the skin. Dupilumab is a human monoclonal antibody that has been approved for the treatment of atopic dermatitis. It has also been linked with various adverse effects, most of them confined to the injection site. Café-au-lait-macules are benign pigmented lesions of the skin, usually seen in people with genetic disorders. We present a case of café-au-lait macules as an adverse effect of dupilumab therapy in a patient with atopic dermatitis. The patient in this case had been receiving dupilumab therapy for atopic dermatitis. The eczematous lesions had seen improvement; however, the patient presented with CALMs on follow-up, which seem to be linked with dupilumab therapy.
ABSTRACT
Lichen planus pigmentosus (LPP) is a rare variant of lichen planus. Due to the scarce number of patients diagnosed with LPP, there are no treatment guidelines. Multiple topical and oral agents are utilized in LPP with varying degrees of response. Isotretinoin has only been investigated in a case report and a single prospective pilot study for managing LPP. Herein, we report the efficacy, safety, and moderate improvement of LPP patients on isotretinoin 20 mg (0.25 mg/kg), topical adapalene gel, 4% hydroquinone cream, and topical sunscreen.
