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CASE REPORT
Year : 2017  |  Volume : 18  |  Issue : 4  |  Page : 306-309

Erythroderma: An association or a manifestation extraordinary of hyper-immunoglobulin E


1 Dermato-Venereology (Skin/VD) Center, Sehgal Nursing Home, New Delhi, India
2 Department of Pathology, University College of Medical Sciences and Associated Guru Teg Bahadur Hospital, New Delhi, India

Date of Web Publication29-Sep-2017

Correspondence Address:
Virendra N Sehgal
Dermato-Venerology (Skin/VD) Center, Sehgal Nursing Home, A/6 Panchwati, New Delhi - 110 033
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_51_17

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  Abstract 


Erythroderma/generalized exfoliative dermatitis, presenting with intensely itchy, dry, scaly, widespread erythema covering whole of the skin surface is reported, the diagnosis of which was primarily clinical but was supplemented by histopathology. Other investigations such as hemogram, immunoglobulin E (IgE), and serum fluorescent enzyme immunoassay (FEIA) were supportive; while IgE was high, serum FEIA was extraordinary high. Identifying it as a clinical pearl of hyper-IgE syndrome, recovery of heavy coloniz ation of Klebsiella pneumoniae, anaerobic Gram-negative bacteria, was contrastingly unique responding affectively to metronidazole, a drug specific for the purpose.

Keywords: Erythroderma, generalized exfoliative dermatitis, hyper-Immunoglobulin E


How to cite this article:
Sehgal VN, Sehgal D, Syed NH, Sharma S. Erythroderma: An association or a manifestation extraordinary of hyper-immunoglobulin E. Indian J Paediatr Dermatol 2017;18:306-9

How to cite this URL:
Sehgal VN, Sehgal D, Syed NH, Sharma S. Erythroderma: An association or a manifestation extraordinary of hyper-immunoglobulin E. Indian J Paediatr Dermatol [serial online] 2017 [cited 2019 Aug 19];18:306-9. Available from: http://www.ijpd.in/text.asp?2017/18/4/306/215795




  Introduction Top


Atopic dermatitis (AD) is a fascinating, chronically relapsing inflammatory skin condition of a typical distribution. It is an age-related disease, which usually begins in infancy or early childhood. The symptoms of itching, scratching, and sleeplessness are a source of frustration not only for the parents but also for the healthcare providers.[1],[2]

The natural course of the disease is unpredictable and is punctuated by remission and exacerbation. Respiratory allergy, retarded growth, bacterial infections, viral infections, ocular abnormalities, and psychosocial aspects are a few of its complications. Occasionally, erythroderma/generalize exfoliative dermatitis might supervene and present as emergency dermatology warranting evaluation to define its undertones. The reporting of the current case is, therefore, unique for very high level of immunoglobulin E (IgE), its hallmark, being reported for the first time in the literature.


  Case Report Top


An 11-year-old male child was admitted in the indoors with intensely recurrent itchy, dry, extensive erythematous scaly eruptions for the past 3 years. The symptoms had their onset in infancy; the initial eruptions started from behind the ear extended to the neck and cubital and popliteal fossae. Ever since they have been progressing to involve whole of the skin surface, dryness, scaling, and erythema were the disturbing features impelling him to seek expert opinion. It was associated with mild fever, malaise, and recurrent upper respiratory tract infections. Spontaneous remissions and exacerbation were the prominent complaints in the course of the disease.

Skin surface examination was explicit. It was dry, scaly, edematous, and conspicuous diffuse erythema, affecting whole of the skin surface. Lichenification, thick, leathery skin was apparent, affecting the skin including flexors around the neck behind the ears, antecubital and popliteal fossae, and groins. The distribution of the lesions was bilateral and symmetrical. The hair and nails were normal [Figure 1].
Figure 1: Erythroderma/generalized exfoliative dermatitis: Depicting erythema, edema, and scaling and along with the features of atopic dermatitis

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Investigations [Table 1]
Table 1: Depicting name of the test, results, units, and biological reference interval

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Hemogram, IgE, and serum fluorescent enzyme immunoassay were the investigations performed.

Serum electrolyte and liver function tests were normal. Radiograph of the chest anteroposterior was normal.

Culture aerobic, miscellaneous at conventional culture, automated identification, and sensitivity

Skin swab yielded a heavy growth of Klebsiella pneumoniae, anaerobic Gram-negative bacteria resistant to Groups A, B, and C antibiotics, except for gentamicin, tobramycin, amikacin, and tigecycline.

Histopathology

Hematoxylin and eosin-stained skin biopsy sections showed mild hyperkeratosis, focal mounds of parakeratosis, variable psoriasiform hyperplasia with broad rete ridges. Focal areas of spongiosis were seen along with lymphocytic exocytosis. Vertical bands of fibrosis were seen in the papillary dermis. Upper dermal blood vessels showed perivascular edema, dense lymphocytic infiltrate, and focal pigment incontinence. Deeper dermis and subcutis were unremarkable [Figure 2].
Figure 2: Erythroderma/generalized exfoliative dermatitis: Hematoxylin and eosin-stained section showing focus of spongiosis in the epidermis and lymphocytic exocytosis. Vertical bands of fibrosis are evident in the papillary dermis (H and E, ×100)

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Treatment

Ciprofloxacin 100 ml (200 mg) and metronidazole 100 ml (500 mg) along with pheniramine maleate 2 ml (22.75 mg), ranitidine hydrochloride injection 2 ml (27.9 ml), and dexamethasone 8 ml (16 mg) in sodium chloride 100 ml (900 mg) 0.9% w/v injection 10 drops/ml (blood set) were administered in the morning around 6:00 AM and evening around 6:00 PM by slow intravenous infusion for 3 days. An excellent response to the treatment was recorded. He was advised to continue the oral therapy consisting of betamethasone 2 mg (0.5 mg tablet) daily in two divided doses, two tablets in the morning and two tablets in the evening for another 10 days as a maintenance therapy.


  Discussion Top


Erythroderma, also known as generalized exfoliative dermatitis, is an inflammatory skin disease, with erythema and scaling that affects nearly the entire cutaneous surface,[3] described first by Ferdinand Von Hebra in 1868. It is one of the challenging emergencies in dermatology.[4] Its precise prevalence is largely evasive. Nevertheless, the prevalence of preexisting dermatoses [5] including AD accounts for 7.2%, psoriasis 37.8%, ichthyoses 7.8%, pemphigus foliaceus 5.6%, and pityriasis rubra pilaris 0.02%.[6]

The diagnosis of AD was made keeping in view its well-defined criteria [7] complimented by those recommended by the consensus conference on pediatric AD.[8]

Undoubtedly, erythroderma is one of the preeminent complications of AD, the diagnosis of which is made on the basis of cardinal clinical features. The present report is its exquisite illustration, the significance of which is compounded by extraordinary high levels of IgE to 18,832.00 kUA/L along with a couple of collateral changes in the hemogram. This particular presentation embraces immunodeficiency, the hyper-IgE syndrome (HIES)[9] also known by hyper-IgE recurrent infection syndrome, Job syndrome, and autosomal dominant HIES. It is, therefore, worthwhile to recapitulate its clinical characteristics recognized by a triad; elevated serum IgE levels, recurrent staphylococcal skin abscesses, and pneumonia with pneumatocele formation. Erythroderma/generalized exfoliative dermatitis may either be an association or manifestation extraordinary the “clinical pearl” of HIES enlarging its clinical spectrum to tetrad, the diagnosis of which is clinical. Histopathology is mandatory and may help in supplementing the diagnosis.

Histopathology is an essential supplement conforming to psoriasiform [10] pattern, characterized by regular elongation of rete ridges, suprapapillary thinning, mild spongiosis, telangiectasia of blood vessels in the papillary dermis, and fusion of foot processes. It is, therefore, worthwhile to define the outline of the features to enhance its educational importance.

  • A thin layer of compact hyperkeratotic overlying variable acanthosis, diffuse epidermal hyperplasia/thickening of the skin
  • Focal spongiosis/intercellular edema, and lymphocytic exocytosis
  • Mild edema and fibrosis of the papillary dermis
  • A moderately dense perivascular infiltrate of lymphocytes around the superficial plexus.


Abnormal bacterial skin colonization in AD is a cardinal feature, Staphylococcus aureus[11] being the most common pathogen. Special receptors, fibronectin or laminin, are required for its finding to host cells. Specific IgE antibodies to the organisms can be detected in the blood, the clinical relevance of which is still enigmatic. Nevertheless, antimicrobial has been shown to be useful in the treatment of AD.[12] The heavy colonization of K. pneumoniae[13] in the current case is intriguing, a salient revelation which is in striking contrast to that of a heavy colonization S. aureus.[11] The use of metronidazole still a drug of choice for the treatment of anaerobic infections was, therefore, taken cognizance of in the present case.

Although the significance of combined antimicrobial corticosteroids therapy in AD remains speculative the overt secondary infection (s) may respond rapidly. It is unlikely that antibacterials contribute to the management of mild AD. However, in severe cases where higher densities of S. aureus colonization are present, antibacterial corticosteroids combined therapy may prove useful.[14] As in the present case, the role of high-dose intravenous Ig in HIES was also evaluated but was not pursued because of its inconsequential outcome.[15] Besides, it is necessary to replenish the fluids, proteins, and serum electrolytes, the guidelines of which have been provided elsewhere.


  Learning Points/Conclusions Top


  • Erythroderma/generalized exfoliative dermatitis may be an outcome of preexisting dermatoses; AD is one of them, the diagnosis of which is formed around well-established criteria
  • Symptoms of erythroderma/exfoliative dermatitis may include pruritus, widespread erythema, and epidermal sloughing
  • The diagnosis is largely clinical; histopathology may be supplementary
  • Hospitalization is often necessary because the disease may be life-threatening
  • Treatment consists of skin care and combined antimicrobial corticosteroids therapy.


Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Staab D, Diepgen TL, Fartasch M, Kupfer J, Lob-Corzilius T, Ring J, et al. Age related, structured educational programmes for the management of atopic dermatitis in children and adolescents: Multicentre, randomised controlled trial. BMJ 2006;332:933-8.  Back to cited text no. 1
    
2.
Tollefson MM, Bruckner AL; Section on Dermatology. Atopic dermatitis: Skin-directed management. Pediatrics 2014;134:e1735-44.  Back to cited text no. 2
    
3.
Kimgai-Asadi A, Freedberg IM. Exfoliative dermatitis. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors. Fitzpatrick's Dermatology in General Medicine. 6th ed. New York: McGraw-Hill; 2003. p. 436-41.  Back to cited text no. 3
    
4.
Sehgal VN, Srivastava G. Erythroderma/generalized exfoliative dermatitis in pediatric practice: An overview. Int J Dermatol 2006;45:831-9.  Back to cited text no. 4
    
5.
Pal S, Haroon TS. Erythroderma: A clinico-etiologic study of 90 cases. Int J Dermatol 1998;37:104-7.  Back to cited text no. 5
    
6.
Vivier AD. Pityriasis rosea, pityriasis rubra pilaris. Atlas of Clinical Dermatology. 3rd ed. Spain: Elsevier; 2004. p. 91-3.  Back to cited text no. 6
    
7.
Hanifin JM, Rajka G. Diagnostic features of atopic dermatitis. Acta Derm Venereol1980;92:44-7.  Back to cited text no. 7
    
8.
Eichenfield LF, Hanifin JM, Luger TA, Stevens SR, Pride HB. Consensus conference on pediatric atopic dermatitis. J Am Acad Dermatol 2003;49:1088-95.  Back to cited text no. 8
    
9.
Ohameje NU, Loveless JW, Saini SS. Atopic dermatitis or hyper-IgE syndrome? Allergy Asthma Proc 2006;27:289-91.  Back to cited text no. 9
    
10.
Weedon D. Weedon's Skin Pathology. 3rd ed. Beijing, China: Churchill Livingstone Elsevier; 2010. p. 121-2.  Back to cited text no. 10
    
11.
Grice EA. The skin microbiome: Potential for novel diagnostic and therapeutic approaches to cutaneous disease. Semin Cutan Med Surg 2014;33:98-103.  Back to cited text no. 11
    
12.
Ring J, Abeck D, Neuber K. Atopic eczema: Role of microorganisms on the skin surface. Allergy 1992;47 (4 Pt 1):265-9.  Back to cited text no. 12
    
13.
Martins IS, Pessoa-Silva CL, Nouer SA, Pessoa de Araujo EG, Ferreira AL, Riley LW, et al. Endemic extended-spectrum beta-lactamase-producing Klebsiella pneumoniae at an Intensive Care Unit: Risk factors for colonization and infection. Microb Drug Resist 2006;12:50-8.  Back to cited text no. 13
    
14.
Williams RE. The antibacterial-corticosteroid combination. What is its role in atopic dermatitis? Am J Clin Dermatol 2000;1:211-5.  Back to cited text no. 14
    
15.
Wakim M, Alazard M, Yajima A, Speights D, Saxon A, Stiehm ER. High dose intravenous immunoglobulin in atopic dermatitis and hyper-IgE syndrome. Ann Allergy Asthma Immunol 1998;81:153-8.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]



 

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