Varicella B01.9

Authors: Prof. Dr. med. Peter Altmeyer, Dr. med. Franziska Löffel

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Last updated on: 29.10.2020

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Synonym(s)

Chickenpox; chicken pox (e); petite vérole volante; Sheep Leafs; Water pox

Definition
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Initial infection of non-immune individuals with the varicella zoster virus. The infection leaves lifelong immunity. Endogenous reinfection leads to the clinical picture of zoster.

Pathogen
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Occurrence/Epidemiology
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The number of cases of chickenpox (compulsory reporting since 2013 !)

is estimated at 500,000 per year for Germany (before the introduction of vaccination). In total, about 75% of children under the age of 15 fall ill, including about 310,000 (41.5%) aged 0 to 5 years, about 320,000 (42.4%) aged 6 to 12 years and about 65,000 (8.8%) aged 12 to 15 years. The incidence of severe courses in hospitalized children < 16 years is 0.8/100,000 children.

In the age group of 10 to 11 years the rate of infection is 94%. In adolescents and adults up to 40 years of age there are still immunity gaps of about 3 to 4%.

According to international studies, the mortality rate of varicella in immunocompetents is between 0.03 and 0.05 per 100,000 person-years. The actual number of varicella-related deaths in Germany is 20-40 deaths per year (approx. 0.03/100,000 person/year). For adults, the number of deaths is given as 17-30/100,000. In children with leukaemia, the mortality rate is about 10%. Children with bronchial asthma and treatment with corticosteroids are also expected to have a complicated course.

Diaplacental transmission: Transmission of the virus via the placenta is rare, but can lead to congenital infection in 1-2% of cases in pregnant women, provided the disease occurred between the 5th and 24th week of pregnancy.

Etiopathogenesis
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Transmission of the virus from a varicella or zoster patient by droplet or smear infection, in particular by contact with saliva or fluid content of the blisters as well as sneezing or coughing. After infection and replication in the epithelium of the respiratory tract (more rarely the conjunctiva), the first viral phase and further replication in the lymphatic system occurs.

Manifestation
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Mainly occurring in childhood (2/3 of all cases in the 5th to 9th year of life); rarely in adults (Varicellae adultorum); very rarely 2nd occurrence of varicella as a reinfection in adults.

Localization
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Infestation in cranio-caudal direction. First the scalp (often infestation of the hairy scalp!) and the oral mucosa (infestation almost obligatory; note: always inspect the oral mucosa in the case of varicella); then the trunk and extremities. Palms and soles of the feet are usually left out.

Clinical features
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95% of infections are clinically manifest. The incubation period is usually about 14 days (9-23 days). After a short prodromal stage, slight fever, distinct itching and exanthema are observed.

Integument: Gradual appearance of small red spots with rapid transition to papules and vesicles. The content of the vesicles, which is initially clear, becomes cloudy and forms firmly adhering crusts which fall off after 2-3 weeks without leaving scars. Polymorphic exanthema due to the simultaneous presence of different developmental phases of efflorescence(Heubner's star map). The number of bubbles varies from a few to several hundreds. Due to scratching effects and secondary infection, varioliform scars may remain. Hemorrhagic or bullous transformation possible.

Mucous membranes: Yellowish covered erosions surrounded by a red hem. At the oral mucosa the hard palate and the cheek mucous membranes are preferentially affected. Less frequently, conjunctiva, larynx or genital mucous membranes are affected.

In children, usually without complications and without accompanying symptoms, rarely lymph node swelling.

In adults, usually a severe course with pronounced general symptoms, feeling ill, fever, headache, joint pain and swelling of the lymph nodes.

Histology
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Initially multi-chambered, later single-chambered vesicles; ballooning degeneration of basal cells; giant epidermal cells. Vesicle smear: Multinuclear epithelial giant cells, inclusion corpuscles.

Differential diagnosis
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Zoster generalisatus: Elderly persons, segmental infestation pattern is still detectable

Eczema herpeticatum: previously known atopic dermatitis, highly febrile course also in children

Prurigo simplex acuta infantum:urticarial papules, sometimes with vesicles, running in batches, severe itching, no feeling of illness.

Prurigo simplex subacuta: long-term chronicity, scratched chronic papules.

Other virus exanthema (Coxsackie and ECHO viruses; EBV). Serology!

Complication(s)
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Impetiginisation, skin gangrene, bronchitis, varicella pneumonia (20-30/10,000 adults), aseptic meningoencephalitis, acute cerebellar ataxia (2-3/10,000 children). Otitis media, nephritis, arthritis, myocarditis, purpura fulminans, Reye's syndrome, bacterial sepsis originating from the skin (2-3/10,000 children), organ infestation, especially in immunocompromised patients.

Infection during pregnancy: incidence: 1-3/1000 pregnancies. Diaplacental infection about 25%. Congenital Varicella syndrome occurs in 1-12% of all infected persons.

External therapy
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Children: Mainly external treatment.

Accommodation/General: Shorten fingernails, if necessary gloves overnight.

Bubble stage: Only local drying measures, e.g. dry brushing with Lotio alba or synthetic tanning agents (e.g. Tannolact Lotio, Tannosynt), zinc sulphate hydrogel, full baths with potassium permanganate additive(light pink) or quinolinol (e.g. Chinosol 1:1000 or R042 ) as bath additive or rubbing with diluted vinegar water. For itching 2-5% polidocanol in lotio alba or as gel R197 or milk(polidocanol-milk).

In case of superinfection: Clioquinol cream(Linola-Sept) or Lotio alba with 0,5-2% Clioquinol addition R050.

Crust stage: Initially antiseptic ointments e.g. Clioquinol cream R049, later softening ointments promoting healing e.g. dexpanthenol ointment or cream (e.g. Bepanthen).

Notice! Antibiotic-containing topicals should be used with caution if they spread over a large area (germ selection! Risk of sensitization! Systemic side effects!).

In case of mucosal involvement: Mouth rinses (e.g. Dexpanthenol R066 ), Kamillosan R255, or Tormentill Astringens R255, if necessary anaesthetic and/or antibiotic solutions or mouth gels or mouth pastes (e.g. Dolo-Dobendan solution, Acoin solution, periodontal mouth ointment, Hexoral R066, Kamistad gel).

In case of fever: bed rest, conservative antipyretic measures (calf compresses, ice packs in the groin), if necessary antipyretic medication (e.g. Paracetamol 250 mg Supp.). Make sure you drink enough fluids.
No acetylsalicylic acid, Cave!
Reye syndrome.

For itching 2-5% polidocanol in lotio alba or as gel R197 or milk (polidocanol-milk).

Adults:

  • Vesicle stage: Drying disinfecting polidocanol-zinc oxide shaking mixture 3-10% R200. In case of superinfection, antiseptic additives like clioquinol 2-5% or zinc sulphate R298. Alternatively, brush Idoxuridine solution (e.g. Zostrum) 4 times/day onto affected skin. Caution! Idoxuridine solution should not be used for more than 4 days.
  • Crust stage: Initially antiseptic ointments, e.g. 2-5% Clioquinol cream R049, later softening ointments that promote healing (e.g. dexpanthenol ointment).

Internal therapy
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  • Children: Antihistamines p.o. such as Dimetinden (e.g. Fenistil Trp., 3 times/day 10-15 Trp. p.o.), Doxylamine (e.g. Mereprine) 1-3 times/day 1-2 Teas. p.o. or Cetirizine (e.g. Zyrtec Trp./juice, 2-3 times 1/2-1 measuring s/day).
  • Immunosuppressed children: Aciclovir (e.g. Zovirax) 500 mg/m2 KO 3 times/day every 8 hours for 5 days i.v.
  • Adults: Aciclovir (e.g. Zovirax) 5 mg/kg bw as short infusion 3 times/day every 8 hours for 5-7 days. Cave! Blood count control, as well as control of liver and kidney values, are required.
  • Alternatively: Aciclovir 800mg p.o. 5 times/day for 5-10 days
  • Alternatively: Valaciclovir (Valtrex®) /3 times/day 1000 mg p.o. for 7 days.
  • Alternative: Brivudine (Zostex®) once/day 125 mg/day p.o. for 7 days.
  • Immunosuppressed adults: Aciclovir (e.g. Zovirax®) 3 times/day 15 mg/kg bw as a short infusion for 5-10 days. If HIV infection is detected, dosage depends on the number of CD4-positive cells.
  • Pregnancy: Seronegative pregnant women may develop severe symptoms (especially in the 3rd trimester) with a 10% probability of developing varicella pneumonia. Therapy with Aciclovir 3 times/day 10-15 mg/kg bw i.v. (for Aciclovir and Valciclovir there is no evidence of frequent malformations; for forensic reasons it is recommended to sign an "informed consent" declaration of consent).

Prophylaxis
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  • Since August 2004, the STIKO (Standing Vaccination Commission) has recommended varicella vaccination (active immunisation) for all children and adolescents. The vaccination should preferably be administered at the age of 11-14 months, but can also be administered at any time afterwards. Unvaccinated 9 to 17-year-olds without a history of chickenpox should be vaccinated, as the disease is associated with a higher rate of complications. Vaccination is also indicated in seronegative women who wish to have children, in seronegative patients on immunosuppressive therapy, in patients with leukaemia, in patients with severe atopic eczema, and in seronegative health care workers.
  • In unvaccinated persons with a negative history of chickenpox and contact with infected persons, post-exposure vaccination within 5 days of contact or within 3 days of the onset of exanthema should be considered.
  • Prophylaxis after contact with a varicella sufferer by means of passive immunization (varicella zoster immunoglobulin) within 96 hours of contact is recommended for persons with increased risk of complications. It can prevent the onset of disease or significantly reduce its severity. This group of persons includes unvaccinated pregnant women with no history of chickenpox, immunocompromised patients with unknown immunity and newborns whose mother contracted varicella 5 days before to 2 days after delivery.
  • Active immunization (live vaccine): Varilrix® 0.5 ml s.c. e.g. in HIV-positive patients without varicella-AK and with negative varicella history. AK-control and in case of more than 200 CD4 cells/µl, or after consultation with HCV-specialists, after 3 months if necessary repeat in case of insufficient vaccination protection. Alternative preparation: Varivax. Cave! Contraindication: pregnancy, massive immunosuppression, VZV infection.
  • Passive immunization with Varicella zoster hyperimmunoglobulin (e.g. Varitect®, Varicellon®), up to 96 hours after exposure at the latest. Varitect®: Once-only 1 ml/kg i.v. or Varicellon®: At least 0.2 ml/kg bw i.m.

Tables
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Systemic antiviral therapy for immunocompromised patients

Immune status

Active substance

Dosage

CD4 cells > 200/µl

Aciclovir (e.g. Zovirax)

5 times/day 12 mg/kg KG p.o. = 5 times/day 1 tbl Zovirax 800

Famciclovir (Famvir)

3 times/day 250 mg p.o. = 3 times/day 1 tbl. p.o.

Valaciclovir (Valtrex)

3 times/day 1000 mg p.o. = 3 times/day 2 tbl. of 500 mg p.o.

Brivudine (Zostex) Cave: (not approved for immunosuppressed patients)

125 mg p.o. once/day for 7 days; Cave: do not use in combination with 5-fluorouracil!

CD4 cells < 200/µl

Aciclovir (Zovirax)

3 times/day 10 mg/kg bw i.v. every 8 hours for 10 days

Foscarnet (Foscavir) For acyclovir resistance (use only if kidney function is intact)

3 times/day 40 mg/kg bw i.v. = 3 times/day 2400 mg i.v., each on 100 ml glucose 5%, diluted to 500 ml over 1 hour infuse, duration unlimited.

Note(s)
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Since March 29, 2013, doctors and laboratories have been required to report chickenpox throughout Germany in accordance with the Infection Protection Act.

Literature
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  1. Andrei G et al (1995) Comparative activity of selected antiviral compounds against clinically isolated varicella-zoster virus. J Clin Microbiol Infect Dis 14: 318-329
  2. Banz K et al (2003) The cost-effectiveness of routine childhood varicella vaccination in Germany. Vaccines 21: 1256-1267
  3. Fiddian AP (1995) Valaciclovir - an improved treatment for individuals with HIV and AIDS. Antiviral Chemistry & Chemotherapy 6: 51-53
  4. Galil K et al (2002) Outbreak of varicella at a day-care center despite vaccination. N Engl J Med 347: 1909-1915
  5. Gerard L (1995) Pharmacology and clinical use of Foscarnet. Int J Antimicrobial agents 5: 209-217
  6. Gershon A (2004) Varicella zoster virus. In: Feigin RD, Cherry JD, Demmler GJ, Kaplan SL (eds.): Textbook of Pediatric Infectious Diseases. Saunders, Philadelphia, p. 1962
  7. Gross G et al (2003) Herpes zoster guideline of the German Dermatology Society (DDG). J Clin Virol 26: 277-289
  8. Chaplain JE, Masur H et al (2002) Guidelines for preventing opportunistic infections among HIV-infected persons--2002 Recommendations of the U.S. Public Health Service and the Infectious Diseases Society of America. MMWR Recomm Rep 51: 1-52
  9. Kempf W, lute hit S (2001) Infections with varicella zoster virus. dermatologist 52: 359-376
  10. Mazzella M et al (2003) Severe hydrocephalus associated with congenital varicella syndrome. CMAJ 168: 561-563
  11. Mohsen AH et al (2003) Varicella pneumonia in adults. Eur Respir J 21: 886-891
  12. Müllegger RR et al (2010) Skin infections during pregnancy. dermatologist 61: 2066-2069
  13. Rappersberger K (1999) Infections with herpes simplex and varicella zoster virus in pregnancy: clinical manifestations in mother, fetus and newborn - therapeutic options. dermatologist 50: 706-714
  14. Snoeck R et al (1994) Chemotherapy of varicella zoster virus infections. Int J Antimicrobial agents 4: 211-226
  15. Swingler G (2003) Chickenpox. Clin Evid 9: 755-762
  16. Wutzler P et al (2002) Seroprevalence of varicella-zoster virus in the German population. Vaccines 20: 121-124

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