Eccema herpeticatum B00.0

Author: Prof. Dr. med. Peter Altmeyer

Co-Autors: Dr. Elisabeth Hanf, Hadrian Tran

Our authors

Last updated on: 29.10.2020

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Synonym(s)

Eccema herpetiforme Kaposi; Eccema herpetiformis; eczema herpeticatum; eczema herpeticum; Eruption Kaposi varizelliforme; Kaposi's varicelliform eruption; Kaposis varicelliform eruption; Kaposi varicelliform eruption; pustulosis acuta varicelliformis; Pustulosis acuta varioliformis Juliusberg; Pustulosis herpetica infantum; pustulosis vacciniformis acuta; Varicelliform eruption Kaposi

History
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Kaposi, 1887; Juliusberg, 1898

Definition
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Mostly acute, febrile, regionally localized or generalized herpes simplex infection (HSV infection) in eczematized skin, mostly in patients with extensive atopic eczema.

Also described in M. Darier, pityriasis rubra pilaris, mycosis fungoides, erythrodermic psoriasis, M. Grover . Some cases were observed during treatment with vemurafenib and other biologicals.

Eccema herpeticatum can also occur after burns and after cutaneous ablative cosmetic procedures.

Etiopathogenesis
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HSV infection through autoinoculation or heteroinfection via an infected contact person. The cause is usually a defective epidermal barrier, often combined with a general or local immunodeficiency (e.g. through long-term local pre-treatment with gluocorticoid externa); the most common underlying disease is atopic eczema! Triggering by UV-exposure is described.

Localization
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Especially face, neck, neck, chest, arms.

Clinical features
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Usually fulminant, generalized, symmetrical exanthema of 0.1-0.2 cm large, red or brown-red, crusty, uniform erosions, running in a single acute thrust. Vesicles are usually not (no longer) found. The dotted, often grouped, even once linearly arranged erosions appear in an almost harmonized, uniform distance to each other. They are all in an identical stage of development (apparently, in contrast to the relapsing-active varicella -starry sky-, only 1 single disease flare occurs). Rarely confluent, more densely staggered erosions, to larger, pyodermicised wound surfaces. Bacterial superinfections are typical side effects of these generalised herpes simplex infections.

There is an extremely strong feeling of illness, high fever, headache. Regional painful lymphadenopathy.

Laboratory
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Mostly only low leukocytosis, inconstant lymphopenia. Significantly increased CRP.

Diagnosis
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  • S.u. Herpes simplex. Cultural cultivation of the virus from vesicle contents (gold standard; specific and safest, but costly method).
  • In the Tzanck test of the vesicle base, detection of multinuclear, epidermal giant cells.
  • Electron microscopy: detection of virus from vesicle contents using negative contrast.

Differential diagnosis
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Eccema vaccinatum, acute exacerbation of atopic eczema, pyoderma,

Complication(s)
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Spread of HSV (viremia) to other organs. The most common complications occur in the CNS (aseptic meningitis and encephalitis). Cave! HSV encephalitis is lethal in 55-70% of cases if untreated.

Other rare systemic complications with possible lethal outcome: rhabdomyolysis, bronchopneumonia.

Ocular involvement in the form of keratitis herpetica.

External therapy
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  • Drying measures, antiseptic and antibiotic dry brushes: Lotio alba, possibly with addition of 0.5-2.0% Clioquinol R050. In case of very painful skin tensions, careful cream treatment (e.g. Ungt. emulsif. aq.), but no ointments or fatty ointments (re-creaming after treatment with Clioquinol-Lotio).
  • Externals with antiviral additives such as idoxuridine solution(zostrum, apply 4 times/day on affected skin, do not use for longer than 4 days) are used especially in the first 48 hours after the appearance of blisters.

Internal therapy
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  • Aciclovir (e.g. Zovirax) i.v. (dose: 5 mg/kg bw/day, in immunocompromised patients 10 mg/kg bw/ every 8 h) for 5-8 days. If the findings are not very extensive, oral therapy with acyclovir 5 times/day 200 mg at intervals of 4 hours may be sufficient. Alternatively Famciclovir (Famvir Filmtbl.) 3 times/day 250 mg.
  • Pregnancy: Studies have shown no evidence of a damaging effect of acyclovir. Therefore, despite the lack of approval, a therapy in pregnancy is recommended after individual risk assessment ( off-label use!).
  • In case of existing resistance to acyclovir therapy or in case of immunosuppressive disease foscarnet (foscavir) 3 times/day 40 mg/kg bw as a 1-hour drip infusion.
  • In case of bacterial superinfection (mostly Staphylococcus aureus) antibiotics such as Flucloxacillin (e.g. Staphylex) 3-4 times/day 0.5-1.0 g p.o. or i.m. or Dicloxacillin (e.g. InfectoStaph) 4 times/day 0.5-1.0 g p.o. or Erythromycin (e.g. Erythromycin Wolff) 4 times/day 500 mg/day. Antibiosis as soon as possible after antibiogram.
  • Possible therapy with immunoglobulins (see IVIG below) or immune stimulants (e.g. isoprinosins, dose: 6-8 tbl/day p.o.), especially for prophylaxis.

Progression/forecast
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The mortality of Eccema herpeticatum, caused by viremia and multiple organ failure, was 10-50% before the introduction of acyclovir in 1977.

Literature
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  1. Cavalié M et al (2013) Kaposi's varicelliform eruption in a patient with pityriasis rubra pilaris (pityriasis rubra pilaris herpeticum). J Eur Acad Dermatol Venereol 27:1585-1586

  2. Bunce PA et al (2013) Grover's disease secondarily infected with herpessimplex
    virus and Staphylococcus aureus: case report and review. Australas JDermatol
    54:88-91

  3. Garg G et al(2012) Psoriasis Herpeticum due to Varicella Zoster Virus:
    AKaposi's Varicelliform eruption in erythrodermic psoriasis. Indian J Dermatol 57:213-214

  4. Gupta M et al (2012) Unusual complication of vemurafenib

  5. treatment of metastatic melanoma: exacerbation of acantholytic dyskeratosiscomplicated
    by Kaposi varicelliform eruption. Arch Dermatol 148:966-968

  6. Juliusberg F (1898) About Pustulosis acuta varioliformis. Arch Derm Syph 46: 21-28
  7. Kaposi M (1887) Pathology and therapy of skin diseases. Urban & Schwarzenberg, Vienna & Leipzig, S. 483
  8. Mathes EF et al (2013) "Eczema coxsackium" and unusual cutaneous findings in an enterovirus outbreak. Pediatrics 132:149-157

  9. Rappersberger K (1999) Infections with herpes simplex and varicella zoster viruses during pregnancy. Clinical manifestation in mother, fetus and newborn - therapeutic options. dermatologist 50: 706-714
  10. Walker D et al (2012) A painful eruption in a woman with Darier disease. J Am Acad Dermatol 67:1089-1990
  11. Wolf R, Tamir A, Weinberg M et al (1992) Eczema herpeticatum induced by sun exposure (letter). Int J Dermatol 31: 298-299
  12. Wollenberg A et al (2003) Predisposing factors and clinical features of eczema herpeticum: a retrospective analysis of 100 cases. J Am Acad Dermatol 49: 198-205

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