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Pemphigus foliaceusL10.2

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.12.2023

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Synonym(s)

Cazenave's disease

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HistoryThis section has been translated automatically.

Cazenave, 1844

DefinitionThis section has been translated automatically.

(Minus-) variant of pemphigus vulgaris with high intraepidermal (subcorneal) continuity separation and thus very thin, volatile, easily tearing blister cover. In contrast to pemphigus vulgaris, there are always no mucous membrane changes in pemphigus foliaceus.

ClassificationThis section has been translated automatically.

A distinction is made between:

Occurrence/EpidemiologyThis section has been translated automatically.

Incidence: 0.5-1/1 million inhabitants/year.

EtiopathogenesisThis section has been translated automatically.

Autoimmune disease with formation of autoantibodies against desmoglein 1. non-specific factors such as stress or sunlight have a provocative effect

ManifestationThis section has been translated automatically.

Mainly occurring between the ages of 30 and 60 (rarely possible in children).

LocalizationThis section has been translated automatically.

Face seborrheic zones, seborrheic zones on the trunk (front and back sweat gutter) and capillitium. No mucous membrane infestation!

Clinical featuresThis section has been translated automatically.

Initially circumscribed onset mostly on the head (especially the face) or in the area of the front or rear sweat duct (seborrheic zones). Flat red papules and plaques with flaky scaly crusts, hyperkeratotic scales, weeping, sticky, moist erosions and unpleasant foetor due to bacterial decomposition. Rarely, vesicles or pustules may be observed at the edges of the plaques. Sudden exacerbations with spread to erythroderma are possible (change of the typically localized disease to the generalized stage of pemphigus vulgaris).

The Nikolski phenomenon I is positive.

In contrast to vulgar pemphigus and paraneoplastic pemphigus, the oral mucosa remains free (explanation: only desmoglein-1-AK; desmoglein-1 is not expressed by mucosal epithelia).

Alopecia and painful paronychia are common.

LaboratoryThis section has been translated automatically.

Pemphigus antibodies (desmoglein-1-AK) are not always detectable.

HistologyThis section has been translated automatically.

Acantholytic blister formation in the upper stratum spinosum or stratum granulosum. Acanthosis, papillomatosis, hyperkeratosis or dyskeratotic changes are also present.

Remark: Regarding the special features of the biopsy technique see below. pemphigus vulgaris

Indirect immunofluorescenceThis section has been translated automatically.

Pemphigus antibodies not always detectable.

Differential diagnosisThis section has been translated automatically.

Seborrheic eczema; lupus erythematosus; pemphigus erythematosus.

Complication(s)This section has been translated automatically.

Sepsis with secondary infection.

External therapyThis section has been translated automatically.

Consistent textile sun protection as well as the use of physical or chemical (e.g. Anthelios) sun protection agents with a high sun protection factor (SPF > 15). Disinfecting or anti-inflammatory or astringent bath additives with synthetic tanning agents (e.g. Tannolact, Tannosynt) are necessary for the treatment of secondary infections. Furthermore, application of medium-strength glucocorticoids like 0.1% triamcinolone cream, 0.25% deoximethasone ointment (e.g. Topisolon) or 0.25% prednicarbate cream (e.g. Dermatop). S.u. Pemphigus vulgaris.

Internal therapyThis section has been translated automatically.

In case of extensive involvement, immunosuppressive therapy with glucocorticoids such as prednisolone (e.g. Decortin® H) initially 1.0-2.0 mg/kg bw/day p.o. in combination with

Rituximab (MabThera®, anti-CD20 AK, dosage: 375 mg/m2 KO on day 1 and 14(-21) i.v. Time until therapy response about 7 weeks. Possibly repeat therapy regime after 1 year),

or azathioprine (e.g. Imurek®, 1.0-1.5 mg/kg bw/day p.o.).

Reduction of glucocorticoids to 2.5-10 mg/day according to clinical symptoms.

Progression/forecastThis section has been translated automatically.

Cheaper than pemphigus vulgaris. In adults chronic course. In children occasionally spontaneous healing.

LiteratureThis section has been translated automatically.

  1. Abreu Velez AM et al (2003) Detection of mercury and other undetermined materials in skin biopsies of endemic pemphigus foliaceus. Am J Dermatopathol 25: 384-391
  2. Abreu-Velez AM (2003) Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. J Am Acad Dermatol 49: 609-614
  3. Cazenave PL (1844) Pemphigus chronique, générale; forme rare de pemphigus foliacé; mort: autopsie; alteration du foie. Les Annales des maladies de la peau et de la syphilis. 18: 583-585
  4. Cummins DL et al (2003) Oral cyclophosphamide for treatment of pemphigus vulgaris and foliaceus. J Am Acad Dermatol 49: 276-280
  5. Eming, R et al.(2015) S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid. JDDG 13: 833-844.
  6. Hirsch R et al (2003) Neonatal pemphigus foliaceus. J Am Acad Dermatol 49: S187-189.
  7. Jarzabek-Chorzelska M et al (2002) Immunopathological diagnosis of pemphigus foliaceus. Dermatology 205: 413-415
  8. Luther H, Kastner U, Altmeyer P (1999) Comment on the contribution by Alexander H. Enk and Jurgen Knop. "Adjuvant therapy of pemphigus vulgaris and pemphigus foliaceus with intravenous immunoglobulins". Dermatologist 50: 372-374
  9. Schmidt E et al (2000) Pemphigus. Loss of desmosomal cell-cell contact. Dermatologist 51: 309-318
  10. Schmidt E et al (2015) S2k guideline on the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid. JDDG 13: 713-726
  11. Whittock NV et al (2003) Targeting of desmoglein 1 in inherited and acquired skin diseases. Clin Exp Dermatol 28: 410-415

Authors

Last updated on: 07.12.2023

Author: Prof. Dr. med. Peter Altmeyer

Editor: Julian Baur