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CASE REPORT
Year : 2022  |  Volume : 49  |  Issue : 2  |  Page : 201-203

Severe eczematous dermatitis with hyperimmunoglobulin E syndrome


Department of Dermatology, Venereology and Leprosy, Datta Meghe Institute of Medical Sciences, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha, Maharashtra, India

Date of Submission01-Apr-2022
Date of Acceptance23-May-2022
Date of Web Publication23-Aug-2022

Correspondence Address:
Raavi Ramapure
Department of Dermatology, Jawaharlal Nehru Medical College, Sawangi, Belgaum, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jss.jss_53_22

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  Abstract 


Primary immunodeficiency syndrome represents a heterogeneous group of inherited disorders caused by mutations in genes encoding functional proteins of the immune cells and characterized by an increased risk of infections, autoimmunity, autoinflammation, malignancy, and allergic disorders. Hereby, we report an 8-year-old-male child with complaints of severe itchy lesions over the body since infancy. Thorough investigations showed he had primary immunodeficiency with underlying hyperimmunoglobulin E syndrome.

Keywords: Atopic dermatitis, eosinophilia, hyperimmunoglobulin E syndrome,


How to cite this article:
Ramapure R, Pugalia N, Ganjre S, Singh A, Madke B. Severe eczematous dermatitis with hyperimmunoglobulin E syndrome. J Sci Soc 2022;49:201-3

How to cite this URL:
Ramapure R, Pugalia N, Ganjre S, Singh A, Madke B. Severe eczematous dermatitis with hyperimmunoglobulin E syndrome. J Sci Soc [serial online] 2022 [cited 2022 Sep 27];49:201-3. Available from: https://www.jscisociety.com/text.asp?2022/49/2/201/354278




  Introduction Top


Primary immunodeficiency syndrome (PIS) represents a heterogeneous group of inherited disorders caused by mutations in genes encoding functional proteins of the immune cells and characterized by an increased risk of infections, autoimmunity, autoinflammation, malignancy, and allergic disorders.[1]

A recent systematic review observed both infectious and noninfectious skin disorders being common in PIS and may be among the presenting clinical manifestations. Staphylococcus aureus-induced skin infections are the most common infectious skin disorders in many primary immunodeficiencies. On the other hand, dermatitis is one of the most prominent noninfectious skin manifestations and may be part of the atopic diathesis. Patients with an atopic constitution show atopic dermatitis (AD) tendency toward the development of food allergies, asthma, and rhinoconjunctivitis.[2]

In a prospective study on 290 Tunisian children with PIS, 32% of cases were found to have bacterial skin infections whereas eczematous dermatitis was found in 21.38%.[3]

Al Herz and Nanda found that 100% of patients with hyper-immunoglobin E (IgE) syndromes (HIES) and Wiskott–Aldrich syndrome (WAS) had eczematous skin rashes. Netherton syndrome, severe combined immunodeficiency, Omenn syndrome, immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome, hyper-IgM syndrome, selective IgA deficiency, common variable immunodeficiency (CVID), and ataxia–telangiectasia commonly presents as eczematous dermatitis in the infancy period. Eczema with underlying immunodeficiency syndrome tends to be more severe, extensive, and often recalcitrant to conventional treatment.[4]


  Case Report Top


An 8-year-old male child presented extensive itchy lesions over most of his body. Parents reported that the skin lesions started from the 1st year of life and there were frequent episodes of remission and relapses. Parents revealed that the child had frequent episodes of upper respiratory tract infections which were resolved with a course of oral antibiotics and symptomatic treatment. There were few episodes of ear infection and seropurulent discharge from the ear in the past. The child had to be admitted on two occasions in the past for severe pneumonia.

Cutaneous examination showed multiple small to large eczematous patches and plaques on trunk extremities. The lesions had linear erosions due to severe scratching [Figure 1] and [Figure 2]. The erosion in the groin was secondarily infected which made walking difficult. A complete hemogram showed increased white blood cell count (19,700cells/cu.mm) with raised eosinophil count. IgE level was markedly raised (>2500IU/ml).
Figure 1: Severe dry eczematous patches and plaques on the posterior trunk and buttocks

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Figure 2: Well-defined eczematous and xerotic patches and plaques on chest, shoulder, upper limbs and groin

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We made a diagnosis of primary immunodeficiency disease with HIES according to the criteria defined by the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. The diagnosis of eczematous dermatitis was made based on the UK Working Party criteria and the severity was assessed using (Eczema Area and Severity Index).

The patient was admitted in dermatology and was started on oral steroid (1 mg/kg), parenteral antibiotic, local emollients, and topical steroid antibiotic. After a week, the child showed significant improvement in the severity of eczema.


  Discussion Top


Eczematous dermatitis is associated with several immunodeficiencies including selective IgA deficiency, CVID, WAS, X-linked agammaglobulinemia, HIES, and Omenn syndrome.[2]

In our case, the diagnosis of primary immunodeficiency with underlying HIES was made based on the investigations.

HIES can be diagnosed by the clinical laboratory triad of recurrent staphylococcal skin infections, recurrent sinopulmonary tract infections, and high serum level of IgE (>2000 IU/mL).[5],[6] Most of the patients with HIES patients present with papulopustular skin rashes in their neonatal period beginning on the face/scalp. There are several reports that showed the copresence of HIES and AD manifestations.[7],[8] Differentiation between the HIES and AD is critical since delay in the diagnosis of underlying primary immunodeficiency may delay in initiation of an appropriate treatment and portend a poor prognosis.[9],[10],[11]

Our patient was managed with parenteral antibiotics, oral steroids, and local emollients which controlled the flare of severe eczema. We are currently exploring the option of using JAK inhibitor (tofacitinib/baracitinib) in our patient since long-term steroids are not indicated to maintain remission of eczema. Long-term bland emollients are the mainstay of treatment with need-based topical steroids to achieve remission (e.g., weekend therapy).

Our case highlights that severe and recalcitrant cases of eczema with a history of frequent upper respiratory tract infection should be investigated for underlying primary immunodeficiency disorders.

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.
Bousfiha AA, Jeddane L, Ailal F, Benhsaien I, Mahlaoui N, Casanova JL, et al. Primary immunodeficiency diseases worldwide: More common than generally thought. J Clin Immunol 2013;33:1-7.  Back to cited text no. 1
    
2.
de Wit J, Brada RJ, van Veldhuizen J, Dalm VA, Pasmans SG. Skin disorders are prominent features in primary immunodeficiency diseases: A systematic overview of current data. Allergy 2019;74:464-82.  Back to cited text no. 2
    
3.
Dhouib NG, Ben Khaled M, Ouederni M, Ben-Mustapha I, Kouki R, Besbes H, et al. Cutaneous manifestations of primary immunodeficiency diseases in tunisian children. Mediterr J Hematol Infect Dis 2018;10:e2018065.  Back to cited text no. 3
    
4.
Al-Herz W, Nanda A. Skin manifestations in primary immunodeficient children. Pediatr Dermatol 2011;28:494-501.  Back to cited text no. 4
    
5.
Picard C, Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Conley ME, et al. Primary immunodeficiency diseases: An update on the classification from the international union of immunological societies expert committee for primary immunodeficiency 2015. J Clin Immunol 2015;35:696-726.  Back to cited text no. 5
    
6.
Williams HC, Jburney PG, Hay RJ, Archer CB, Shipley MJ, Ahunter JJ, et al. The UK working party's diagnostic criteria for atopic dermatitis. I. Derivation of a minimum set of discriminators for atopic dermatitis. Br J Dermatol 1994;131:383-96.  Back to cited text no. 6
    
7.
Hanifin JM, Thurston M, Omoto M, Cherill R, Tofte SJ, Graeber M. The eczema area and severity index (EASI): Assessment of reliability in atopic dermatitis. EASI Evaluator Group. Exp Dermatol 2001;10:11-8.  Back to cited text no. 7
    
8.
Grimbacher B, Holland SM, Puck JM. Hyper-IgE syndromes. Immunol Rev 2005;203:244-50.  Back to cited text no. 8
    
9.
Aghamohammadi A, Moghaddam ZG, Abolhassani H, Hallaji Z, Mortazavi H, Pourhamdi S, et al. Investigation of underlying primary immunodeficiencies in patients with severe atopic dermatitis. Allergol Immunopathol (Madr) 2014;42:336-41.  Back to cited text no. 9
    
10.
Appalasamy JR, Sariff A. A survey of drug-related-problems among patients treated for allergy symptoms in community pharmacies at Negeri Sembilan, Malaysia. Int J Pharm Pharm Sci 2014;6:313-6.  Back to cited text no. 10
    
11.
Bonilla FA, Bernstein IL, Khan DA, Ballas ZK, Chinen J, Frank MM, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol 2005;94:S1-63.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]



 

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