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CASE REPORT
Year : 2018  |  Volume : 19  |  Issue : 1  |  Page : 59-61

Monomorphic bullous hemorrhagic varicella in a patient on methotrexate


1 Department of Dermatology, Venereology and Leprosy, KGMU, Lucknow, Uttar Pradesh, India
2 Department of Microbiology, KGMU, Lucknow, Uttar Pradesh, India
3 Department of General Medicine, KGMU, Lucknow, Uttar Pradesh, India

Date of Web Publication28-Dec-2017

Correspondence Address:
Swastika Suvirya
Department of Dermatology, Venereology and Leprosy, KGMU, Lucknow - 226 003, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_6_15

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  Abstract 

Varicella, a common exanthematous disease of childhood, can have atypical presentation in immunosuppressed patients. We report a case of a 14-year-old female who presented with multiple hemorrhagic tense bullae and a monomorphic presentation that created an initial diagnostic dilemma. Patient's treatment history was remarkable for taking methotrexate for arthritis for the last 2 months. The patient was treated with a prolonged course of intravenous acyclovir that eventually led to her uneventful recovery.

Keywords: Hemorrhagic bullae, methotrexate, varicella


How to cite this article:
Suvirya S, Jain A, Agrawal A, Jain P. Monomorphic bullous hemorrhagic varicella in a patient on methotrexate. Indian J Paediatr Dermatol 2018;19:59-61

How to cite this URL:
Suvirya S, Jain A, Agrawal A, Jain P. Monomorphic bullous hemorrhagic varicella in a patient on methotrexate. Indian J Paediatr Dermatol [serial online] 2018 [cited 2019 Dec 7];19:59-61. Available from: http://www.ijpd.in/text.asp?2018/19/1/59/211821


  Introduction Top


Varicella is a common exanthematous viral disease of childhood having a polymorphic morphology, with all stages of the lesion such as vesicles, papules, and crusting seen at the same time. The lesions usually measure 2–3 mm in diameter.[1]

Monomorphic morphology is seen when all the lesions are at the same stage. Monomorphic and bullous morphology of varicella is an extremely rare manifestation, with only a few cases reported in literature.[2],[3],[4],[5] We report a 14-year-old female who received methotrexate treatment, following which she developed multiple, tense hemorrhagic bullae during varicella. The unique aspect of the case was the monomorphic morphology and large size of the lesions which created a clinical dilemma. One should be aware of the atypical presentation of varicella in patients on immunosuppressive therapy as timely intervention can be lifesaving for the patient.


  Case Report Top


A 14-year-old female was admitted to the Department of Dermatology with a 2-week history of high-grade fever with chills and myalgia with subsequent eruption of multiple bullous lesions on the entire body. The lesions started as multiple ulcers in the oral cavity and vaginal mucosa about 7 days back. Two days later, the patient developed few papular lesions on the forehead which spread to involve the entire body including palms and soles in a span of 2–3 days. After the 2nd day, the papules turned into vesicular lesions which gradually increased in size. The lesions turned hemorrhagic on the 8th day of eruption [Figure 1]a and [Figure 1]b. New lesions continued to appear until day 15. There was no history of varicella infection or vaccination. Her treatment history was remarkable for receiving methotrexate 5 mg every week for 2 months for knee pain from a local practitioner without a proper workup for arthritis. There was neither history of contact with varicella patient nor any history of bullous disorder.
Figure 1: (a and b) Monomorphic bullous hemorrhagic lesions on entire body

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Physical examination revealed multiple tense hemorrhagic bullous lesions ranging in size from 6 to 20 mm in diameter scattered all over the body including palms and soles with superficial 5–10 mm erosions in oral and vaginal mucosa. Hairs and nails did not reveal any abnormality. There was neither active bleeding from any site nor any organomegaly.

Tzanck's smear was positive for multinucleated giant cells. Hemogram, coagulation profile, and liver and kidney function tests were normal and ELISA for HIV 1 and 2 was negative. Skin biopsy revealed intraepidermal vesiculation with multinucleated giant cells; dermis showed moderately dense superficial and deep perivasular and periappendegeal infiltrates of lymphocytes and neutrophils [Figure 2]. Swab for bacterial culture and sensitivity was negative from the vesicular fluid. Further investigations showed the presence of immunofluorescent antibody for varicella zoster virus (VZV). Sera also tested positive for anti-VZV IgM antibody (test value 25.53 NTU) (Novatec Immundiagnostica Gmbh, Germany). Anti-VZV IgG antibody was negative (Novatec Immundiagnostica Gmbh, Germany). Multiplex real-time polymerase chain reaction (PCR) for VZV and herpes simplex virus (HSV) 1 and 2 was done from the bullous fluid which was positive for VZV (Ct value of 14.0) (Taqman chemistry). Multiplex real-time PCR tested negative for HSV 1 and 2.
Figure 2: Skin biopsy showing an intraepidermal vesicle lined by mixed inflammatory infiltrate and acantholytic keratinocytes along with presence of multinucleated epithelial giant cell (a) as well as Herpes affected keratinocytes (b)

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On day 3, intravenous acyclovir was started at a dose of 10 mg/kg body weight 8 hourly and continued for 25 days. The lesions began to crust simultaneously on day 21 and healed with slightly atrophic hypopigmented macules by the 28th day [Figure 3]. Her methotrexate was also discontinued. The patient was followed regularly at interval of a month without any further complication over 4 months.
Figure 3: Healing with scaling and crusted lesions

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  Discussion Top


VZV infection characteristically presents with a polymorphic morphology having all stages of the lesion such as vesicles, papules, and crusting at the same time. This typical presentation may alter significantly in immunocompromised patients as in lymphoma or patients on immunosuppressive therapy with frequent adverse outcome.[6] Monomorphic varicella has been reported only in photodistributed varicella.[2],[3] Our patient had extensive, bullous, hemorrhagic lesions with monomorphic presentation after 2-month long treatment with methotrexate. To the best of our knowledge, we did not find monomorphic presentation in immunosuppressed patients in the literature.

The vesicular lesions of varicella are typically 2–3 mm in diameter with clear fluid. Hemorrhagic varicella with blood in the vesicles and hemorrhagic complications such as purpura and disseminated intravascular coagulation has been reported in immunocompromised individuals as in lymphoma or patients receiving immunosuppressive therapy such as methotrexate.[7] In patients receiving steroids and methotrexate, fatal hemorrhagic varicella zoster has been reported.[8] In most patients with hemorrhagic varicella, there are generalized hemorrhagic complications and varicella becomes fatal. Our case is different as there was the absence of generalized hemorrhagic complication at presentation and with the institution of antiviral therapy the disease resolved uneventfully.

Varicella bullosa is an unusual manifestation of chicken pox. It has been suggested that bullous lesions result from secondary bacterial infection most commonly Staphylococcus.[5] In our case, we could demonstrate the VZV DNA by PCR and antibody by immunofluorescence in the bullous fluid.

In case of our patient, although the cutaneous manifestations portended a serious course, she did not develop any visceral dissemination or coagulopathy, possibly because she was put on intravenous acyclovir relatively early and for a prolonged period.

There is greater risk of VZV pneumonia, hepatitis, meningitis, coagulopathy, progressive visceral dissemination in immunosuppressed patients. Some authors have recommended serological testing of patients in the absence of history of varicella infection before starting immunosuppressive therapy.[6] Seronegative patients may be vaccinated before initiation of immunosuppressive therapy to prevent primary VZV infection which may be progressive and fatal in them. In case VZV is acquired during immunosuppressive treatment, the immunosuppressant should be discontinued and combination of intravenous acyclovir and intravenous immunoglobulin treatment may be lifesaving.[9],[10]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Straus SE, Oxman MN, Schmader KE. Varicella and herpes zoster. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's Dermatology in General Medicine. 7th ed. New York: McGraw-Hill Book Co.; 2008. p. 1885-98.  Back to cited text no. 1
    
2.
Sakiyama M, Maeshima H, Higashino T, Kawakubo Y. Photolocalized varicella in an adult. Br J Dermatol 2014;170:1195-6.  Back to cited text no. 2
[PUBMED]    
3.
Karaca S, Kulac M, Dogru O, Cetinkaya Z. Photodistributed varicella. Eur J Gen Med 2005;2:89-90.  Back to cited text no. 3
    
4.
Peyramond D, Bertrand JL, Bertoye A. Bullous varicella. Association of varicella and a childhood scalded-skin syndrome. Arch Fr Pediatr 1979;36:922-5.  Back to cited text no. 4
[PUBMED]    
5.
Sathynarayana BD. Varicella bullosa. Indian J Dermatol Venereol Leprol 2003;69:56-7.  Back to cited text no. 5
    
6.
Hackett CB, Wall D, Fitzgerald SF, Rogers S, Kirby B. Varicella-zoster virus immunity in dermatological patients on systemic immunosuppressant treatment. Br J Dermatol 2011;164:1387-9.  Back to cited text no. 6
[PUBMED]    
7.
Mashood AA, Salamat N. Fatal hemorrhagic chickenpox in a case of acute promyelocytic leukemia. J Pak Assoc Dermatol 2008;18:119-21.  Back to cited text no. 7
    
8.
Morice AH, Lai WK. Fatal varicella zoster infection in a severe steroid dependent asthmatic patient receiving methotrexate. Thorax 1995;50:1221-2.  Back to cited text no. 8
[PUBMED]    
9.
Lu YC, Fan HC, Wang CC, Cheng SN. Concomitant use of acyclovir and intravenous immunoglobulin rescues an immunocompromised child with disseminated varicella caused multiple organ failure. J Pediatr Hematol Oncol 2011;33:e350-1.  Back to cited text no. 9
[PUBMED]    
10.
Plisek S, Pliskova L, Bostik V, Prasil P, Laco J, Chlibek R, et al. Fulminant hepatitis and death associated with disseminated varicella in an immunocompromised adult from the Czech Republic caused by a wild-type clade 4 varicella-zoster virus strain. J Clin Virol 2011;50:72-5.  Back to cited text no. 10
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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