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 Table of Contents  
ORIGINAL ARTICLE
Year : 2018  |  Volume : 26  |  Issue : 1  |  Page : 20-23

Exfoliative diseases of the integument and mucous membrane that mimic deep 2nd-degree burns with or without sepsis


1 Department of Plastic and Reconstructive Surgery, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
2 Department of Pediatric Intensive Care, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
3 Department of Pediatric Dermatology, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India

Date of Web Publication11-Mar-2019

Correspondence Address:
Prof. K Mathangi Ramakrishnan
Department of Plastic and Reconstructive Surgery, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijb.ijb_28_18

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  Abstract 


Introduction: Exfoliative skin diseases in children can mimic burns in many ways and require early diagnosis and management. Here we enumerate the different skin conditions with four case based examples and their management.
Clinical Presentation: Varying degrees of skin involvement may be seen with severe involvement in DRESS syndrome and milder involvement in Stevens-Johnson syndrome. Management: The management of this exfoliative lesion is the application of collagen membrane to the wound as we treat burns. Although systemic symptoms can produce mortality (e.g., dress), collagen dressing heals the wound well with good epithelialization. Early and appropriate antibiotic therapy is mandatory.
Conclusion: Careful attention to correct diagnosis, early intervention and use of collagen dressing have improved outcome in these children.

Keywords: Closed dressing in Stevens–Johnson syndrome and toxic epidermal necrolysis, collagen as biological dressing for exfoliative diseases, exfoliative disease management


How to cite this article:
Ramakrishnan K M, Ramachandran B, Ravikumar K G, Ravikumar K, Ramkumar R, Jayaraman V, Mathivanan T. Exfoliative diseases of the integument and mucous membrane that mimic deep 2nd-degree burns with or without sepsis. Indian J Burns 2018;26:20-3

How to cite this URL:
Ramakrishnan K M, Ramachandran B, Ravikumar K G, Ravikumar K, Ramkumar R, Jayaraman V, Mathivanan T. Exfoliative diseases of the integument and mucous membrane that mimic deep 2nd-degree burns with or without sepsis. Indian J Burns [serial online] 2018 [cited 2019 Sep 18];26:20-3. Available from: http://www.ijburns.com/text.asp?2018/26/1/20/253853




  Introduction Top


Significant mortality and morbidity are seen in certain necrotizing diseases of the skin and underlying tissues. Some of these may look like deep 2nd-degree burns, with or without infection, during their evolution.

These conditions include the following:

  • Staphylococcal scalded skin syndrome (SSSS)
  • Erythema multiforme major
  • Steven–Johnson syndrome (SJS)
  • Toxic epidermal necrolysis (TEN)
  • Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome).


Some of these conditions are thought to be similar in pathogenesis, differing only in the severity of involvement. We present our experience with these conditions with case series.


  Clinical Presentation and Description Top


Staphylococcal scalded skin syndrome

The lesions may look like scalds or superficial partial-thickness burns as illustrated in [Figure 1] which compares the two conditions.[1]
Figure 1: (a) An 18-month-old child with superficial burns. (b) The 18-month-old child with lesions of Staphylococcal scalded skin syndrome

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In SSSS, blistering occurs over the skin resembling scalds. This occurs due to toxins produced by Staphylococcus in the affected area. Exfoliative toxin A and superantigens cause breakage of desmoglobin and loss of cell adhesion in the epidermal layer. These toxins can enter the circulation and cause skin erosions at distant sites. The blisters that occur often burst and the wound looks like a burn ulcer. There is tenderness surrounding the lesion. Mucous membranes are spared in SSSS. The condition may lead to dehydration in children.

SSSS usually follows a benign course when diagnosed and treated early.


  Management Top


Treatment consists of supportive care and appropriate antibiotics. Corticosteroids are not indicated.

Immaturity of the immune system makes neonates the most at risk. Usually, staphylococcal skin diseases are seen in children from the poorer groups of society, who have increased risk. Though the incidence is higher in developing countries, some differences exist in the incidence of strains and type of exotoxins produced. Fluid-filled areas appear over the skin and when these rupture, the area looks like burn.

Nose-and-throat cultures may show a growth of Staphylococcus aureus.

Principles of treatment

  • Child should be admitted for acute care
  • As the child may have fluid loss due to exfoliation, rehydration must be initiated
  • Anti-staphylococcal antibiotics, such as cloxacillin, are essential. The incidence of methicillin resistance is high in these conditions, where teicoplanin, linezolid, or vancomycin has to be used.


Erythema multiforme major

Many authors consider erythema multiforme major, SJS, and TEN to be the same disease entity, differing only by the area of involvement of skin (SJS – 10%, TEN >30%). There is a zone of overlap between 10% and 30% total body surface area (TBSA) and is known as “SJS and TEN.”

Stevens–Johnson syndrome

This is a little more extensive than the previous condition. It is characterized by the involvement of <10% of the TBSA. Prognosis is usually good. The main pathophysiology in both SJS and TEN [Table 1] is apoptosis of the keratinocytes due to cell-mediated cytotoxicity.[2] Drug-induced proliferation of the cytotoxic T-cells causes damage to keratinocytes, leading to an extensive epidermal loss.
Table 1: Differences between  Stevens-Johnson syndrome More Details, toxic epidermal necrolysis, and drug reaction with eosinophilia and systemic symptoms syndrome

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[Figure 2] shows the comparison between burns and Stevens-Johnson syndrome.
Figure 2: (a) A 5-year-old boy with Steven–Johnson syndrome. (b) A 4-year-old boy with burns

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Clinically, the use of biological dressing material on the exposed dermis and the sensitive dermal nerve endings seems to minimize pain and physical discomfort of the patients.[3]

The standard treatment includes the removal of all loose skin and coverage of the wound with collagen. At the end of 7–10 days, the membrane falls off or can be removed with an artery forceps dipped in paraffin.[4]

The epithelialization is very satisfactory if we treat like 2nd-degree burns.[5] Application of biologic dressing is a very comfortable way of treating superficial and superficial partial-thickness burns.[6]

Toxic epidermal necrolysis

This is similar to SJS, but is a severe condition involving more than 30% TBSA with systemic symptoms.[7] This has to be treated with supportive measures. [Figure 3] shows differentiation of burns from Toxic epidermal necrolysis.
Figure 3: (a) A 10-year-old child with severe form of toxic epidermal necrolysis. (b) A 7-year-old child with burns

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This occurs in all age groups, but more in the elderly. Mortality is high, and the wounds can get infected with Pseudomonas aeruginosa or S. aureus. Pathogenesis is less understood and complex. The cause could be drugs or viruses.[8] Immunocompromised patients, such as hematopoietic stem cell transplant recipients, are at an increased risk. The superficial epidermis peels off and the red raw area is visible.[9] The wounds are covered with collagen dressing and heal satisfactorily like a 2nd-degree burn. Antibiotics may be required, depending on the extent of involvement and the presence of secondary infection.[10]

Drug reaction with eosinophilia and systemic symptoms

This is a rare but potentially life-threatening drug-induced hypersensitivity reaction which includes skin eruption and eosinophilia with atypical lymphocytosis with internal organ involvement (liver, kidney, and lung). This condition has a long latency (2–8 weeks) period. Accumulation of toxic drug metabolites secondary to defects in metabolizing the drugs coupled with genetic associations is considered as the most important reason for DRESS. Another possibility is the interaction between drug and a concurrent viral infection or reactivation leading to expansion of T-cells and subsequent manifestations.

Numerous terms are used to describe adverse drug reactions, but the one we prefer is “DRESS – Drug Reaction with Eosinophilia and Systemic Symptoms” which seems most appropriate. The offending drugs include carbamazepine, phenytoin, phenobarbitone, lamotrigine, allopurinol, and various antibiotics. The incidence ranges from 1 to 5/10,000 patients who have been exposed to anticonvulsant drugs.[11]

History of one of our cases with drug reaction with eosinophilia and systemic symptoms syndrome (case report [Figure 4])
Figure 4: (a) An 11-year-old child with lesions of drug reaction with eosinophilia and systemic symptoms. (b) The 11-year-old child with severe burns

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A previously well 11-year-old female child was treated elsewhere for fever with multiple antimicrobials and other drugs. She developed a rash that evolved to exfoliation involving the entire body. She was shifted to this hospital for further management. In addition to the skin rash, she had anemia, leukocytosis, elevated immunoglobulin (Ig) E, and hepatitis (severe hyperbilirubinemia with moderately elevated transaminases). There was extensive desquamation and peeling of the skin. A skin biopsy showed epidermal necrosis with infiltration by eosinophils and neutrophils. She was diagnosed as having DRESS syndrome. She was treated with antibiotics, intravenous Ig, and pulse methylprednisolone. In addition, collagen dressing was applied to the skin lesions. She improved initially but developed secondary septic shock caused by Acinetobacter baumanii, from which she could not be saved.


  Conclusion Top


In this study, we have presented four cases of exfoliative diseases of the skin that resembled 2nd-degree burns with infection. The last patient had very severe disease (DRESS) and could not be saved. Although initially they resembled burns, prognosis was different. Sometimes, these patients are admitted in the burns ward and their wounds are treated like burns. We have started using collagen dressing (manufactured in the Central Leather Research Institute of India and patented by the Government of India) which is very useful. Prior to the advent of such skin covers, bulky dressings were done – these were not only labor intensive but also tend to get infected. Clinicians must be aware of these conditions, which mimic burns.

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.
Mathangi Ramakrishnan K, Hanumadass M. Non thermal wounds that mimic burns and their management in children. Pediatric Burns. Paras Medical Publisher; 2011. p. 250-67.  Back to cited text no. 1
    
2.
Ramakrishnan KM, Sankar J, Venkatraman J. Role of biological membranes in the management of Stevens Johnson syndrome – Indian experience. Burns 2007;33:109-11.  Back to cited text no. 2
    
3.
Forman R, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in children: A review of 10 years' experience. Drug Saf 2002;25:965-72.  Back to cited text no. 3
    
4.
Chang YS, Huang FC, Tseng SH, Hsu CK, Ho CL, Sheu HM, et al. Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: Acute ocular manifestations, causes, and management. Cornea 2007;26:123-9.  Back to cited text no. 4
    
5.
Chung WH, Hung SI, Yang JY, Su SC, Huang SP, Wei CY, et al. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nat Med 2008;14:1343-50.  Back to cited text no. 5
    
6.
Mockenhaupt M. Stevens-Johnson syndrome and toxic epidermal necrolysis: Clinical patterns, diagnostic considerations, etiology, and therapeutic management. Semin Cutan Med Surg 2014;33:10-6.  Back to cited text no. 6
    
7.
Ghislain PD, Roujeau JC. Treatment of severe drug reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis and hypersensitivity syndrome. Dermatol Online J 2002;8:5.  Back to cited text no. 7
    
8.
Heimbach DM, Engrav LH, Marvin JA, Harnar TJ, Grube BJ. Toxic epidermal necrolysis. A step forward in treatment. JAMA 1987;257:2171-5.  Back to cited text no. 8
    
9.
Spies M, Sanford AP, Aili Low JF, Wolf SE, Herndon DN. Treatment of extensive toxic epidermal necrolysis in children. Pediatrics 2001;108:1162-8.  Back to cited text no. 9
    
10.
Sheridan RL, Schulz JT, Ryan CM, Schnitzer JJ, Lawlor D, Driscoll DN, et al. Long-term consequences of toxic epidermal necrolysis in children. Pediatrics 2002;109:74-8.  Back to cited text no. 10
    
11.
Kardaun SH, Sekula P, Valeyrie-Allanore L, Liss Y, Chu CY, Creamer D, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): An original multisystem adverse drug reaction. Results from the prospective RegiSCAR study. Br J Dermatol 2013;169:1071-80.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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