INTRODUCTION
============

Blistering diseases are facing the danger of being finished, since our understanding of the pathogenesis and therapeutic approaches are undergoing a major revision. A wide spectrum of skin disorders can manifest as a blistering process. A blister is an event associated with tissue injury and fluid accumulation within a specific layer of skin due to either genetic mutations or autoimmune response. Blisters can also occur secondary to bacterial/viral infections, chemical/physical burns or skin necrosis/dermatitis.^[@B1][@B2]^ Here, the focus of interest is a bullous dermatoses in the child based on a case of pemphigus vulgaris (PV).

The PV is an autoimmune blistering disease of elderly (3rd-5th decade), which was previously fatal before the advent of steroid therapy, mainly due to dehydration or secondary systemic infection.^[@B3][@B4]^ The PV is characterized by the presence of circulating autoantibodies immuno-globulin G against desmogleins 3^[@B5][@B6]^ which result in loss of cell to cell adhesion and blister formation that rupture and progress to form painful erosions.^[@B4]^ The PV in children aged less than 12 years is known as childhood PV and in those aged between 12 and 18 years as juvenile PV. Data on incidence and prevalence of childhood PV are scarce because in literature only a few cases are reported. In a study, children aged less than 15 years accounted for 3.7% of cases.^[@B7]^ Several environmental factors, medications, and acantholytic substances superimposed on genetic predisposition may play a role in the onset of this disease in children.^[@B8]^

CASE REPORT
===========

An 11-year-old girl presented to the department of pedi-atric dentistry, with a complaint of multiple eruptions and blisters all over the mouth, which increased in size gradually over a period of 2 to 3 months and ruptured to form a crusty erosive surfaces with watery discharge ([Fig. 1](#F1){ref-type="fig"}). Later, similar sores appeared on limbs, trunk, and the genital area which were painful and led to considerable discomfort ([Figs 2](#F2){ref-type="fig"} and [3](#F3){ref-type="fig"}).

Entire oral mucosa including the tongue was eroded and erythematous, causing extreme discomfort and pain during eating. There was no history of any drug intake during the past 6 months nor any systemic condition identified. The child presented with such a condition for the first time and there was no such disorder noted in the family. Nikolsky's perilesional sign was positive.

The girl was hospitalized in the medical unit and comprehensively managed with the help of a dermatologist ([Tables 1](#Table1){ref-type="table"} and [2](#Table2){ref-type="table"}). Direct immunofluorescence was positive and perilesion biopsy containing intact lesion, revealed Tzanck cells, intraepidermal blister and suprabasilar acantholysis ([Fig. 4](#F4){ref-type="fig"}). The connective tissue stroma showed dense mononuclear infiltration. A significant improvement in the condition was observed after 3 to 4 weeks following the standardized steroid treatment regime ([Figs 5](#F5){ref-type="fig"} to [7](#F7){ref-type="fig"}).

![Multiple crushed lesions with superficial erosions on lips](ijcpd-10-196-g001){#F1}

![View of lesions on the limbs](ijcpd-10-196-g002){#F2}

![View of wide spread lesions all over the body](ijcpd-10-196-g003){#F3}

DISCUSSION
==========

Unusual childhood occurrence, though quick response to treatment, however potentially life-threatening nature with substantial morbidity, justifies its consideration in routine dental practice. These chronic recurrent and painful lesions interfere with the daily activities of life, such as eating, drinking, talking, and personal relationships.^[@B9]^ Pediatric dentists have the unique opportunity since initial lesions occur in the oral cavity and complete remission is possible only with early diagnosis.^[@B10]^

Prompt diagnosis and early initiation of aggressive therapy can combat the malignant course of disease in children. The treatment strategies should be based on the understanding of underlying pathogenic processes and recurrence^[@B3][@B11]-[@B14]^ ([Tables 3](#Table3){ref-type="table"} and [4](#Table4){ref-type="table"}). Systemic corticosteroids and immunosuppressive therapy are the mainstay treatments for PV. Apart from steroids, adjuvant therapies include azathioprine, mycophenolate mofetil, dapsone, and rituximab in refractory cases.^[@B4][@B7][@B8]^ These modern therapies can effectively reduce the circulating antibodies, allowing patients to lead a normal life. Adverse effects associated with long-term use of steroids, such as weight gain, menstrual irregularities, growth retardation, osteoporosis, and hormonal disturbances in adolescence^[@B4][@B5]^ have always led to the search for newer steroid sparing and novel avenues for eradication of blisters at the molecular level.^[@B1][@B2]^ As we probe deeper into molecular aspects of the disease, our understanding of the patho-genesis begins to gain focus, offering new novel, and improved methods of therapy or even an opportunity to achieve a cure, which should mark the end of an era of blistering diseases.

###### 

**Table 1:** Systemic treatment regime

  ---------------- ------ ---------------------------------------- ------ ------------------------------------------------------- ------
  *Drugs*                 *Dose, route, and duration\**                   *Action*                                                    
  Dexamethasone           0.5 mL Inj IM (50-100 mg) 3 to 4 weeks          Modification of immune response (immunosuppresion)          
  Roxithromycin           150 mg Tab BID - 2 to 3 week                    Antibacterial for secondary infection                       
  Prednisolone            10-20 mg Tab tapering to 5 mg                   Anti-inflammatory and modification of immune response       
                          BID - 2 to 3 months                                                                                         
  Hematopoietics          Oral capsule OD - 1 month                       Nutritional supplement                                      
  NaCl saline             IV fluid                                        Electrolytic balance                                        
  ---------------- ------ ---------------------------------------- ------ ------------------------------------------------------- ------

\*Minimum duration is 3 to 4 weeks, may be extended depending on response and recurrence; IM: Intramuscular injection; BID: Twice (two times) a day; OD: Once daily; IV: Intravenous

###### 

**Table 2:** Topical treatment regime

  ---------------------------------------- ------ ----------------------------------------- ------ ----------------------------------------------------- ------
  *Drugs*                                         *Dose, route, and duration'*                     *Action*                                                  
  Triamcinolone                                   Local application for more than 3 weeks          Potent anti-inflammatory and alters immune response       
  Silver sulfadiazine and chlorhexidine           Local application for more than 2 weeks          Broad spectrum antimicrobial                              
  Gentamycin with propyl salicylic acid           Local application for more than 2 weeks          Prevents secondary infections                             
  Saline compresses over erosive lesions          Local application for more than 2 weeks          For soothing effect and control of edema                  
  Chlorhexidine                                   Oral gargle for more than 3 weeks                Oral antimicrobial                                        
  ---------------------------------------- ------ ----------------------------------------- ------ ----------------------------------------------------- ------

![Acantholysis and suprabasilar separation](ijcpd-10-196-g004){#F4}

![Posttreatment view](ijcpd-10-196-g005){#F5}

![Lesions disappear following standard treatment regime](ijcpd-10-196-g006){#F6}

![Healing of lesions all over the body](ijcpd-10-196-g007){#F7}

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**Table 3:** Protocols for preventing recurrence^[@B3][@B12]-[@B14]^

  -- ------ -------------------------------------------------------------- ------
            Maintaining healthy diet and weight                                
            Avoiding sunlight and friction of body folds                       
            Keeping flexural areas clean and dry                               
            Wearing cool garments with absorbent pads                          
            Regular evaluation of secondary infections                         
            Systemic antibiotics, such as tetracycline and erythromycin        
            Topical use of antibacterial creams, such as benzyl peroxide       
            Long-term low-dose steroid maintenance therapy                     
            Controlling side effects of long-term steroids                     
  -- ------ -------------------------------------------------------------- ------

###### 

**Table 4:** The bullous management portfolio^[@B5][@B11]-[@B14]^

  -- ------ --------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------- ------
            Gold line mainstay of therapy - Steroids (Systemic prednisone 1 mg/kg/day and topical triamcinolone)                                                                                                                

            Broad-spectrum antibiotics for control of secondary infections Improving the general health and hygiene of the patient (Fluid replacement, electrolytic balance, and multiple vitamins/ minerals)                   

            Symptomatic relief of pain, discomfort, burning, and itching (Paracetamol, astringents, and aluminium acetate)                                                                                                      

            Steroid sparing immunosuppressant and adjuvants (Mycophenolate mofetil, tracolimus, azathioprine, dapsone,\                                                                                                         
            retenoids methotrexate, cyclophosphamide, gold,\                                                                                                                                                                
            cyclosporine, and chlorambucil)                                                                                                                                                                                 

            Newer vistas - Plasmapheresis, intra venous immunoglobulins, anti-B cell monoclonal antibodies, CO~2~ laser vaporization, dermabrasion, proteinase inhibitors, chimeric molecules, cholinergic agonists, etc.       
  -- ------ --------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------- ------

**Source of support**: Nil

**Conflict of interest**: None
