Senear-Usher syndrome
- VernacularTitle:Синер–Ашерийн хам шинж
- Author:
Nomin B
1
;
Otgonbayar M
1
;
Jambalsuren M
1
;
Khandsuren B
2
;
Tsogzol G
1
Author Information
- Publication Type:Case Reports
- Keywords: Pemphigus erythematosus; Pemphigus sebaceous; Lupus erythematosus; Pemphigus foliaceus
- From: Mongolian Journal of Health Sciences 2026;94(4):133-136
- CountryMongolia
- Language:Mongolian
-
Abstract:
Background:Senear-Usher Syndrome, also known as pemphigus erythematosus, is a rare autoimmune blistering skin disease characterized by overlapping clinical, histopathological, and immunological features of both lupus erythematosus and pemphigus foliaceus.
Case Presentation:We report the case of a 46-year-old male diagnosed with Senear-Usher Syndrome, who presented to the outpatient clinic of the National Dermatology Center of Mongolia and received inpatient treatment.
Diagnosis:The patient exhibited diffuse, thick, pale gray scales and yellowish crusts on the scalp. Multiple brown hyperpigmented macules of various shapes and sizes, vivid erythema, and a few erosions were observed on the nose, cheeks, trunk, and upper and lower extremities. A few pea-sized vesicles containing clear fluid were scattered on the back. Nikolsky's sign was weakly positive, and Besnier-Meshchersky's sign was positive. Histopathology: Histopathological analysis of skin biopsies revealed hyperkeratosis, parakeratosis, and acanthosis in the epidermis. Subcorneal cleft formation with neutrophils within the cleft was noted, along with perivascular infiltration of neutrophils and lymphohistiocytes in the superficial dermis.
Treatment:The patient received treatment with hydroxychloroquine, systemic and topical corticosteroids, and symptomatic therapy.
Outcome:Following treatment, the patient's general condition improved significantly. The erosions re-epithelialized, and the vesicles resolved, leaving behind brown hyperpigmented macules. The patient is currently under regular follow-up by a dermatologist and a family physician.
Conclusion:Senear-Usher Syndrome is a rare disease with a high potential for misdiagnosis. Therefore, an accurate diagnosis relies on a comprehensive evaluation of clinical features, histopathological examination, and direct and indirect immunofluorescence studies. - Full text:202610012306573182427_133-136_Синер–Ашерийн хам шинж.pdf
