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Pemphigus erythematosusL10.4

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Lupus erythematosus pemphigoides; pemphigoéde séborrhéique; pemphigus seborrhoicus; Senear-Usher Syndrome

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HistoryThis section has been translated automatically.

Senear and Usher, 1926

DefinitionThis section has been translated automatically.

Clinically harmless variant of pemphigus foliaceus with clinical and immunological (concomitant) features of subacute cutaneous lupus erythematosus and increased photosensitivity.

ManifestationThis section has been translated automatically.

Usually occurs after the 60th LJ.

LocalizationThis section has been translated automatically.

Seborrheic zones of the face, head, chest and back are affected. Mainly symmetrical infestation. Mucous membranes are rarely involved.

Clinical featuresThis section has been translated automatically.

Butterfly-shaped, crust-covered red plaques in the centrofacial parts of the face and in the seborrhoeic zones on the chest and back. Fatty, crumbly layered scales, scale crusts, individual (rarely detectable) blisters on reddened or unaltered skin and erosions appear. The Nikolski phenomenon I positive. Exacerbation can be triggered by UV exposure.

HistologyThis section has been translated automatically.

S.u. Pemphigus foliaceus. Leukocytes in the dermis like in lupus erythematosus.

Direct ImmunofluorescenceThis section has been translated automatically.

Detection of IgG antibodies in the intercellular substance and at the basement membrane, up to 60% positive lupus band test.

Indirect immunofluorescenceThis section has been translated automatically.

Pemphigus antibodies, occasionally antinuclear antibodies.

DiagnosisThis section has been translated automatically.

Light stairs ( MED), DIF after UV provocation.

Differential diagnosisThis section has been translated automatically.

Seborrheic eczema; subacute cutaneous lupus erythematosus.

General therapyThis section has been translated automatically.

Consistent textile sun protection, additionally sun protection agent with high sun protection factor (SPF > 15; e.g. Anthelios).

External therapyThis section has been translated automatically.

For moderately severe or low skin infestation, medium-strength glucocorticoids such as 0.25% prednicarbate cream(e.g. Dermatop), 0.1% hydrocortisone buteprate (e.g. Pandel), 0.025% fluocinolone cream (e.g. Jellin) are usually sufficient.

Internal therapyThis section has been translated automatically.

For extensive infestation, internal glucocorticoid therapy (1.0-2.0 mg/kg bw/day prednisone equivalent). Monotherapy usually sufficient. Depending on clinical symptoms, reduction to 2.5-10 mg/day. If a glucocorticoid dose below the Cushing's threshold cannot be achieved without recurrence, additional administration of azathioprine (e.g. Imurek) 1.0-1.5 mg/kg bw/day. As an alternative to azathioprine (the first choice), a trial with DADPS (e.g. Dapson Fatol) 50-100 mg/day can be performed.

Progression/forecastThis section has been translated automatically.

Transition to pemphigus foliaceus is possible. Rare coexistence with myasthenia gravis, thymoma or systemic lupus erythematosus.

Note(s)This section has been translated automatically.

Many authors dispute the entity of this disease, which should have the immunological parameters of both pemphigus and systemic lupus erythematosus. Otherwise, the clinical picture is compatible with a blunt pemphigus foliaceus.

LiteratureThis section has been translated automatically.

  1. Amerian AL, Achmed A (1985) Pemphigus erythematosus. Int J Dermat 24: 16-25
  2. Gomi H et al (1999) Pemphigus erythematosus: detection of anti-desmoglein-1 antibodies by ELISA. Dermatology 199: 188-189
  3. Kindle SA et al (2015) Dermatologic disorders in 118 patients with autoimmune (immunoglobulin G4-related) pancreatitis: a retrospective cohort analysis. At J Clin Dermatol. 201 At J Clin Dermatol 16: 125,130
  4. Makino T et al (2014) Induction of skin lesions by ultraviolet B irradiation in a case of pemphigus erythematosus. Acta Derm Venereol 94:487-488
  5. Oktarina DA et al (2012) The IgG "lupus-band" deposition pattern of pemphigus erythematosus: association with the desmoglein 1 ectodomain as revealed by 3 cases. Arch Dermatol 148:1173-117
  6. Scheinfeld NS et al (2003) Pemphigus erythematosus associated with anti-DNA antibodies and multiple anti-ENA antibodies: a case report. Cutis 71: 303-306
  7. Senear FE, Usher B (1926) An unusual type of pemphigus combining features of Lupus erythematosus. Arch Derm Syph 13: 761

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer