Pemphigus herpetiformis L10.81

Floden & Gentale, 1955

Special form of pemphigus disease with herpetiform, very itchy or burning, papulovesicular, anular or circinate skin manifestations on an erythematous base, usually in clinically manifest pemphigus foliaceus (variant of pemphigus foliaceus?), rarely in pemphigus vulgaris.

Rare (5-8% of all pemphigus diseases), panethnic, no sex predilection.

Occurring in adults (30.-80. LJ), more common around the 60th LJ.

Intermittent course of the disease with dense, grouped, cluster-like arranged, tight, clear, 0.1-0.5 cm large blisters, which are localized on red spots or plaques. Burning or itching, 1-6 mm large, reddish-brown papules are also visible. With increasing duration of the acute episode, eroded and encrusted lesions and scratch excoriations are impressive. Only rarely does involvement of the oral mucosa occur.

Superficial blistering: eosinophilic spongiosis. Intraepidermal and subcorneal microabscesses with neutrophils and eosinophils. Acantholysis may be absent!

IgG, e.g. against desmoglein 1 (Dsg-1) and desmocollin 3 (Dsg-3) in the intercellular space of the epidermis. Antibodies against desmocollins are rarer.

Clinical, histology, immunohistology, ELISA (IgG against desmoglein 1, IgG against desmocollin 3 as well as IgA against desmoglein 1 are detectable).

Dermatitis herpetiformis.

See pemphigus vulgaris below. Systemic glucocorticoids such as prednisolone (e.g. Decortin H) initially 60-90 mg/day, then reduction to maintenance dose.

Alternatively: DADPS (e.g. dapsone-fatol) 50-150 mg/day p.o. Good response is reported in the literature (Kühn J et al. (2023).

Chronic recurrent course.

1. Ahmed AR, Sami N (2002) Intravenous immunoglobulin therapy for patients with pemphigus foliaceus unresponsive to conventional therapy. J Am Acad Dermatol 46: 42-49
2. Enk AH, Knop J (1998) Adjuvant therapy of pemphigus vulgaris and pemphigus foliaceus with intravenous immunoglobulins. Dermatologist 49: 774-776
3. Floden C, Gentale H (1955) A case of clinically typical dermatitis herpetiformis (M. Duhring) presenting acantholylysis. Acta Derm Venereol (Stockh) 35: 128
4. Goebeler M et al (2003) Blistering autoimmune diseases of childhood. Dermatologist 54: 14-24
5. Kozlowska A et al. (2003) Pemphigus herpetiformis with IgA and IgG antibodies to desmoglein 1 and IgG antibodies to desmocollin 3. J Am Acad Dermatol 48: 117-122
6. Kühn J et al. (2023) Transformation of pemphigus foliaceus into pemphigus herpetiformis. J Dtsch Dermatol Ges 21:655-658.
7. 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 Hautarzt 50: 372-374
8. Marinovic B et al. (2003) Coexistence of pemphigus herpetiformis and systemic lupus erythematosus. J Eur Acad Dermatol Venereol 17: 316-319
9. Robinson ND (1999) The new pemphigus variants. J Am Acad Dermatol 40 (5 Pt 1): 649-671