Pruritic, Pink Scaling Plaques on the Face and Trunk

Pritchett, Ellen; Hejazi, Emily Z.; Cusack, Carrie Ann

doi:10.1001/jamadermatol.2015.2103

Cited by 4 publications

(6 citation statements)

References 5 publications

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“…Lesions typically appear on sun-exposed cutaneous areas such as the head, neck, and upper trunk. Pemphigus erythematosus may also present in a malar distribution mimicking the facial-cutaneous presentation of SLE [5].…”

Section: Discussionmentioning

confidence: 99%

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A Recalcitrant Case of Senear-Usher Syndrome Treated With Rituximab

de Vries,

Moody,

Ojha

et al. 2023

Cureus

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A 51-year-old uninsured, otherwise healthy male who works in the fishing industry presented with a twomonth history of pruritic scaly plaques on his face, scalp, and trunk and mild photosensitivity. A biopsy of a scalp lesion revealed acantholysis consistent with pemphigus foliaceus. Laboratory testing demonstrated elevated anti-desmoglein 1, positive antinuclear antibodies (ANA and anti-dsDNA), and elevated Sjögren's anti-SS-A antibodies. The patient was diagnosed with pemphigus erythematosus. The patient was not optimally responsive and was unable to discontinue systemic corticosteroids despite a maximum dosage of mycophenolate mofetil of 3000 mg/day. Hence, rituximab was added as a rescue treatment with the rheumatoid arthritis protocol. Three months after starting rituximab, there was a marked improvement in symptoms with complete resolution of cutaneous lesions.

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Section: Discussionmentioning

confidence: 99%

“…Treatment includes systemic corticosteroids, dapsone, and immune-suppressing treatments. Rituximab is an established and successful therapy for different subtypes of acquired pemphigus [5]. However, there is little information available on the efficacy of this therapy or other biological treatments in treating PE [6].…”

Section: Introductionmentioning

confidence: 99%

A Recalcitrant Case of Senear-Usher Syndrome Treated With Rituximab

de Vries,

Moody,

Ojha

et al. 2023

Cureus

View full text Add to dashboard Cite

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“…Clinically, the patient often has malar involvement that mimics a severe seborrheic dermatitis with well-defined erythematous, scaly, crusted plaques [4]. Nonfacial lesions may begin as small, flaccid bullae with positive Absoe-Hansen and Nikolsky signs, favoring the upper trunk and face, although lesions have been reported to extend to the feet [2,4]. Lesions often resolve with hyperpigmentation.…”

Section: Case Discussionmentioning

confidence: 99%

“…Positive antinuclear antibody serology is variably positive; 30-80% of patients have positive ANA titers. PE patients rarely meet the full diagnostic criteria for systemic lupus erythematosus [2]. Cases have been reported with normal lupus serologies, normal complement studies, and normal inflammatory markers [3].…”

Section: Case Discussionmentioning

confidence: 99%

“…Senear-Usher syndrome or pemphigus erythematosus (PE) is a rare autoimmune blistering disease that is a more localized form of pemphigus foliaceus (PF). Classically, patients present with small, flaccid bullae with scaling and crusting; mucous involvement is rare [1,2]. Diagnosis is made via a combination of pathological findings of PF and direct immunofluorescence (DIF) demonstrating immunoglobulin G (IgG) and complement deposition both intercellulary and at the dermoepidermal junction (DEJ), [3].…”

Section: Introductionmentioning

confidence: 99%

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