Senear Usher syndrome, a rare variant of pemphigus foliaceus

Authors

DOI:

https://doi.org/10.29176/2590843X.1818

Keywords:

Pemphigus, blisters, hyperpigmentation

Abstract

Senear-Usher syndrome is a localized variant of pemphigus foliaceus, rare, with a chronic course, manifested by well-defined erythematous squamous plaques, with a seborrheic appearance and distribution, which adopts a butterfly-wing pattern resembling cutaneous lupus erythematosus (characteristic in our case) positive Nikolsky sign. With the presence of fragile and fleeting blisters, it does not affect the mucosa, it is a disease of low incidence and represents 0.5 to 3.2 cases per 100,000 inhabitants per year. In ages 15-84 years, on average at 54 years, without predominance of race or sex. We present the case of a 31-year-old male patient, skin phototype V/VI according to Fitzpatrick, with no significant history, presenting bilateral and symmetrical generalized dermatosis beginning in the central facial region characterized by well-defined erythematous plaques with regular borders, with Presence of thick oily yellow scales and bruises on the surface, which spread to other seborrheic areas of the chest, anterior and posterior, exulcerated areas, hyperpigmented macules of variable sizes, pruritic with a 1-year history. A skin biopsy was performed with findings consistent with this disease. Treatment with topical and systemic steroids and immunosuppressants was started with a good response. A case is reported because it is a rare variant of pemphigus foliaceus, so it is important to report this case in Venezuela.

Author Biographies

Mary Soto, 128245

Médico, residente de segundo año del posgrado de Dermatología. Universidad de Carabobo, Ciudad Hospitalaria “Dr. Enrique Tejera”, Valencia, Estado Carabobo, Valencia, Venezuela.

Neiris Villegas, Universidad de Carabobo

Médico, residente de tercer año del posgrado de Dermatología. Universidad de Carabobo, Ciudad Hospitalaria “Dr. Enrique Tejera”, Valencia, Estado Carabobo, Valencia, Venezuela. 

Sandra Vivas, Universidad de Carabobo

Médico internista y dermatóloga. Magíster en Investigación Educativa. Jefa, Servicio de Dermatología, Universidad de Carabobo, Ciudad Hospitalaria “Dr. Enrique Tejera”. Coordinadora, programa de posgrado de Dermatología. Miembro, Sociedad Venezolana de Dermatología; Sociedad Internacional de Dermatoscopia.

References

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How to Cite

1.
Soto M, Villegas N, Vivas S. Senear Usher syndrome, a rare variant of pemphigus foliaceus. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2024 Apr. 1 [cited 2024 Jul. 3];32(1):77-83. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/1818

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Published

2024-04-01

How to Cite

1.
Soto M, Villegas N, Vivas S. Senear Usher syndrome, a rare variant of pemphigus foliaceus. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2024 Apr. 1 [cited 2024 Jul. 3];32(1):77-83. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/1818
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