Pemphigus vegetans type hallopeau L10.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Hallopeau-type pemphigus vegetans; pyoderma vegetans; Pyodermites végétante Hallopeau-Feulard; pyodermite végétante; Pyodermite végétante de Hallopeau

History
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Hallopeau, 1898

Definition
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Suppurative variant of pemphigus vulgaris with formation of papillomatous growths in the eroded areas. S.a. Pemphigus vegetans, type Neumann. Pyostomatitis vegetans is assumed by some authors to be a minimal variant.

Etiopathogenesis
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Variant of pemphigus vulgaris (autoantibodies against desmoglein 1, 3), occasionally triggered by ACE inhibitors. Also described in the context of malignant underlying diseases.

Localization
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Mainly intertrigines, capillitium, stem, genitals, oral mucosa.

Clinical features
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Initially, yellowish-purulent pustules and flabby blisters appear, which after bursting turn into foetal smelling vegetation on eroded ground. Frequently reddened, hyperkeratotic, erosive or encrusted plaques with growth tendency and deep, wrinkle-like relief (especially on the capillitium).

Histology
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Particularly prominent is a pronounced acanthosis with intraepithelial neutrophil and eosinophilic infiltrates which in some places condense like abscesses, with eosinophilic cancellous bone and acantholysis.

Differential diagnosis
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Complication(s)
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Bacterial superinfection.

Therapy
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  • Glucocorticoids such as prednisone (e.g. Decortin) initially 60-120 mg/day and azathioprine (e.g. Imurek) 50-150 mg/day. Glucocorticoid maintenance dose below the Cushing's threshold. Successes under acitretin (neotigason) have been described. S.a. Pemphigus vulgaris.
  • Alternatively, x-ray soft radiotherapy or the intralesional injection of glucocorticoids such as triamcinolone (e.g. Volon A/Scandicain 1:2) may be considered. In case of erosions baths with disinfectant additives such as potassium permanganate (light pink), drying local therapy with lotio alba, possibly with 2-5% clioquinol R050. Also antibiotic or antimycotic additives may be used for external treatment, possibly in combination with glucocorticoid-containing creams or emulsions with hydrocortisone (Hydro-Wolff, Hydrogalen, R120 ) or triamcinolone acetonide (Triamgalen).

Operative therapie
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The papillomatous growths can be removed surgically.

Literature
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  1. Gerharz M, Stadler R (1987) Pemphigus vegetans of the Hallopeau type. Detection of pemphigus antibodies with direct and indirect immunofluorescence. dermatologist 38: 371-374
  2. Hallopeau FH (1898) Nouvelle étude sur une forme pustuleuse et bulleuse de la maladie de Neumann, dite pemphigus végétans Annales de dermatologie et syphiligraphie (Paris) 9: 969
  3. Hashizume H et al (1993) Epidermal antigens and complement-binding anti-intercellular antibodies in pemphigus vegetans, Hallopeau type. Br J Dermatol 129: 739-743
  4. Jansen T et al (2001) Pemphigus vegetans. A historical perspective. dermatologist 52: 504-509
  5. Neumann HA et al (1980) Pyodermite vegetante of Hallopeau. Arch Dermatol 116: 1169-1171
  6. Ngo JT et al (2012) Pemphigus vegetans associated
    withintranasal cocaine abuse. J Cutan Med Surg 16:344-349
  7. Steiner A et al (1985) Pemphigus vegetans (Hallopeau) with eosinophilic cancellous bone - a successful retinoid therapy. dermatologist 36: 356
  8. Virgili A et al (1992) Sudden vegetation of the mouth. Pemphigus vegetans of the mouth (Hallopeau type). Arch Dermatol 128: 398-399, 401-402
  9. Ngo JT et al (2012) Pemphigus vegetans associated
    withintranasal cocaine abuse. J Cutan Med Surg 16:344-349

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Last updated on: 29.10.2020