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Bullous pemphigoid is often difficult to treat with the limited therapies available. Here, we describe clinical outcomes among 30 adults with bullous pemphigoid patients treated with dupilumab. We performed a multicenter, retrospective case series between March 2020 to August 2022. Patients received a loading dose of dupilumab 600 mg, followed by 300 mg maintenance dose with varying administration frequency tailored to individual patient response. All patients experienced at least some improvement in blister formation and pruritus, with 23 (76.7%) of patients demonstrating either complete clearance of blistering or marked response. Complete clearance of pruritus or marked response was noted in 25 (83.3%) of patients. Eight patients were effectively maintained solely on dupilumab. One (3.3%) patient reported an injection site reaction. Thirty patients represent a small sample, however, to our knowledge, this is the second largest group of BP treated with dupilumab. Furthermore, we provide an understandable framework for clinicians outside of academics to follow and assess treatment responses in their BP patients treated with dupilumab. Dupilumab should be considered as a therapeutic option in patients with bullous pemphigoid given its ability to induce sustained blistering and pruritus response in both typical and refractory cases while maintaining a favorable safety profile. J Drugs Dermatol. 2024;23(6):e144-e148. doi:10.36849/JDD.8258e.

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Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion affecting the skin and mucous membranes, predominantly affects females in their fifth and sixth decades of life. Due to its rare occurrence in children and adolescents, there is often a delay in diagnosis and treatment in this age group. PV should always be considered in the differential diagnosis of oral ulcerative and vesiculobullous lesions in both children and adolescents.

Hailey‒Hailey disease is a rare chronic autosomal-dominant blistering disease characterized by erosions, fissures, and vegetations occurring in intertriginous regions. To date, there is no specific treatment and there are no therapeutic guidelines, which makes management of the disease challenging. We present the case of a 43-year-old man unsuccessfully treated for Hailey‒Hailey disease with topical and systemic corticosteroids, antibiotics, and surgical debridement. At presentation he had erosions, vegetations, and infection in the axillae and groin. We introduced oral methotrexate, 10 mg weekly, and complete remission was achieved in 3 weeks. After 8 weeks, we decided to discontinue methotrexate due to lesion absence. Over 3 years of follow-up, mild flares were effectively managed with topical miconazole or mild steroid creams. We conclude that oral methotrexate is safe and effective for achieving long-term remission in Hailey‒Hailey disease.

BACKGROUND: Pemphigus diseases are a subgroup of autoimmune bullous diseases characterized by autoantibodies against desmogleins and occasionally desmocollins. Desmocollin 3 is the main desmocollin isoform that contributes to cell adhesion in the epidermis.
OBJECTIVE: To evaluate the presence and level of anti-desmocollin 3 antibodies in pemphigus diseases, and to investigate whether their presence is associated with a specific type, presentation, or clinical pattern.
METHODS: Forty patients with pemphigus diseases and forty healthy controls were enrolled. Medical history, clinical examination, and pemphigus disease area index (PDAI) scoring were recorded for all patients. Serum samples were collected from both groups for assessment of anti-desmocollin 3 antibody reactivity by ELISA.
RESULTS: The presence of anti-desmocollin 3 antibodies was significant among patients with pemphigus compared with controls (P=0.003). The level of anti-desmocollin 3 antibodies was also significantly higher in patients with pemphigus compared with controls (P=0.01). There was no significant relationship between the presence of anti-desmocollin 3 antibodies and any of the clinical presentations of pemphigus (type, severity, duration, activity, presence of annular pattern, or site of affection - mucosal, cutaneous, on the scalp, palmoplantar, or flexural).
CONCLUSIONS: Anti-desmocollin 3 antibodies are upregulated in pemphigus diseases and can contribute to the pathogenesis of pemphigus. No specific clinical type, presentation, or pattern was found to be associated with the presence of anti-desmocollin 3 antibodies.

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Early intervention is imperative for potentially fatal dermatologic diseases such as pemphigus vulgaris. In rural Nepal, limited public awareness, home remedies, and delays in healthcare access lead to poor outcomes. Although biopsy confirms the diagnosis, experienced dermatologists can make an accurate clinical diagnosis when characteristic skin lesions are present.

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UNASSIGNED: Paraneoplastic pemphigus (PNP) is an uncommon autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphous skin eruptions, and the presence of underlying neoplasms. Unique histopathological features include suprabasal acantholysis and clefts with scattered necrotic keratinocytes.
UNASSIGNED: A 27-year-old female patient presented with a 3-month history of a painless swelling, approximately the size of a pea, on the left lateral aspect of her neck and axillary area. This swelling progressively increased in size and number. Additionally, she had reddish, itchy, raised skin lesions over her elbows bilaterally, which gradually spread to involve most of her body, including her lips, tongue, and buccal mucosa. These skin lesions were associated with difficulty swallowing both liquid and solid foods. A diagnostic test, including a biopsy, confirmed the diagnosis of PNP. Subsequently, the patient was managed with chemotherapy and other supportive measures, leading to improvement and eventual discharge.
UNASSIGNED: PNP is a rare blistering disorder associated with neoplasms, often presenting diagnostic and treatment challenges. Patients with PNP may develop a diverse range of lesions. It is crucial to promptly recognize and manage the underlying malignancy for improved patient outcomes.
UNASSIGNED: This case highlights the rare association between T-cell lymphoma and PNP. Clinicians should also remain vigilant for the possibility of PNP in lymphomas that are not of B-cell lineage.