answer
in text
 graphic
question

Pemphigus Erythematosus (Senear-Usher Syndrome)

Patient's Course
  • Prednisone 60mg/day for 4 weeks: no response
  • Predisone plus Azathioprine 150mg/day for 6 weeks: dramatic improvement
Key Aspects of Pemphigus Erythematosus onset clinical presentation laboratory evaluation immunofluorescence associated diseases drug association treatment
ages 17-84
M=F>
erythematous plaques with vesicles, erosions or scale, or flaccid bullae
+ pemphigus antibody
sometimes + low titer ANA, homogenous or speckled
IgG and Complement within intercellular spaces of the epidermis
80% have IgG and C3 at the dermal-epidermal junction
Lupus Erythematosus
Myasthenia Gravis
Thymoma
Penicillamine
Prednisone +/- immunosuppressive agents
Dapsone
Differentiating Pemphigus erythematosus from Pemphigus foliaceus
  • lesions are restricted to seborrheic areas of face and trunk
  • presence of anti-nuclear antibody (most cases)
  • immunofluorescence reveals IgG and C3 at the dermal-epidermal junction.

1) Senear FE, Usher B. An unusual type of pemphigus combining features of lupus erythematosus. Arch Dermatol Syphilol 1926; 13:761-781. 2) Amerian ML, Ahmed AR. Pemphigus erythematosus: Senear-Usher syndrome.Int J Dermatol.1985;24:16-25. 3) Andrade R. Pemphigus erythematosus in a six-year old girl.Mod Probl Paediatr. 1975;17:190-192. 4) Jablonska S, Chorzelski T, Blaszczyk M, Maciejewski W. Pathogenesis of pemphigus erythematosus. Arch Dermatol Res.1977;258:135-140. 5>) Chorzelski T, Jablonska S, Blaszczyk M. Immunopathological investigations in the Senear-Usher syndrome (coexistence of pemphigus and lupus erythematosus). Br J Dermatol1968;80:211-217. 6) Amerian ML, Ahmed AR. Pemphigus erythematosus: Presentation of four cases and review of the literature. J Am Acad Dermatol 1987;16:472-480. 7) Ngo AW, Straka C, Fretzin D. Pemphigus erythematosus; a unique association with systemic lupus erythematosus. Cutis1986;38:160-163. 8) Cruz PD, Coldiron BM, Sontheimer RD. Concurrent features of cutaneous lupus erythematosus and pemphigus erythematosus following myasthenia gravis and thymoma. J AM Acad Dermatol1987;16:472-480. 9) Ruocco V, Pisani M. Induced pemphigus. Arch Dermatol Res 1982;274:123-140. 10) Basset N, Guillot B, Michel B, Meynadier J, Guilhou JJ. Dapsone as initial treatment in superficial pemphigus. Arch Dermatol1987;123:783-785.