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CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 160-162

A rare case of childhood mucous membrane pemphigoid involving both skin and mucosa


1 Department of DVL, SCB Medical College and Hospital, Cuttack, Odisha, India
2 Department of DVL, Institute of Medical Sciences and SUM Hospital, S ‘O' A University, Bhubaneswar, Odisha, India
3 Department of Paediatrics, Institute of Medical Sciences and SUM Hospital, S ‘O' A University, Bhubaneswar, Odisha, India

Date of Web Publication29-Mar-2019

Correspondence Address:
Dr. Chinmoy Raj
Department of DVL, Institute of Medical Sciences and SUM Hospital, Kalinga Nagar, Bhubaneswar - 751 003, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_71_18

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  Abstract 


Mucous membrane pemphigoid has been documented previously in only 19 patients under the age of 20 years. We report a 14-year-old female who had erosions of the oral, ocular, anal and genital mucosa, congestion, hoarseness of voice, dysphagia, and recurrent cough. Symblepharon formation in both eyes necessitated surgical correction. Biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidermal bulla. Direct immunofluorescence showed the deposition of IgG in linear pattern along the dermoepidermal junction. Therapy was begun, consisting of prednisolone at 1 mg/kg/day, in combination with dapsone at 2 mg/kg/day. Azathioprine was added later for control of the disease. The patient showed good clinical response.

Keywords: Adolescent, cicatricial pemphigoid, mucocutaneous


How to cite this article:
Pattnaik M, Panda M, Dash M, Raj C. A rare case of childhood mucous membrane pemphigoid involving both skin and mucosa. Indian J Paediatr Dermatol 2019;20:160-2

How to cite this URL:
Pattnaik M, Panda M, Dash M, Raj C. A rare case of childhood mucous membrane pemphigoid involving both skin and mucosa. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Apr 25];20:160-2. Available from: http://www.ijpd.in/text.asp?2019/20/2/160/255211




  Introduction Top


Mucous membrane pemphigoid (MMP) is an autoimmune bullous disorder that primarily affects the mucous membranes and occasionally the skin. Oral and ocular involvements are common. Blindness and other life-threatening complications may occur.[1] It is found almost exclusively among middle-aged and elderly patients, and it has been described in 19 children, with an important female predominance (15 girls and 4 boys).[2],[3] None of them had the involvement of all mucosal surfaces. We present a case of MMP with all mucosae as well as cutaneous involvement in a 14-year-old female child.


  Case Report Top


A 14-year-old female presented with congestion, photophobia, and lacrimation for six month duration followed by the appearance of blisters in the oral cavity, throat pain, recurrent dry cough, pain in perianal and vulval area, and blisters on the body. Physical examination revealed tense bullae on the skin which healed with hypopigmentation, mild scarring, and milia formation. Erosions with thick hemorrhagic crusts were present in both nasal cavities. Symblepharon was present in both eyes on the lateral aspect [Figure 1], [Figure 2], [Figure 3]. For complaint of hoarseness of voice and pain on deglutition, laryngoscopic evaluation was done that revealed edema, congestion in vocal folds, vocal cords, and arytenoids. Multiple anal fissures with scarring were present. Few healed scars were present in the vulval area. Biopsy was taken from vesicle on the left cheek and trunk for histologic and immunofluorescence studies. Histology showed a cleft below the basal cell layer of dermis and dermal infiltration consisting of lymphocytes and few eosinophils. Direct immunofluorescence (DIF) showed linear IgG deposit along the dermoepidermal junction. Based on the clinical and immunofluorescence findings, the diagnosis of MMP was established. She was started on prednisolone at 1 mg/kg/day along with dapsone 2 mg/kg/day (after doing the hematological profile and glucose 6 phosphate dehydrogenase levels). New blisters still kept appearing for which azathioprine at 100 mg/day was added later. Partial release of symblepharon was done. Dose was maintained until new lesions stopped appearing and old ones healed.
Figure 1: Involvement of ocular, nasal, and oral mucosa along with erosions on the facial skin

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Figure 2: Erosions seen on the upper eyelid, nasal bridge, and concha

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Figure 3: Active erosions along with healed lesions in the form of postinflammatory hyperpigmentation

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  Discussion Top


MMP, previously called as cicatricial pemphigoid (CP), is a rare condition in children as only 19 cases have been reported so far in the English literature.[1] The majority of the cases have occurred in female patients, with 15 girls and 4 boys, aged between 20 months and 18 years.[2],[3] To the best of our knowledge, this is the only case of childhood MMP reported yet which had involvement of skin as well as all the mucosa. Childhood MMP shares similar clinical, histologic, and immunofluorescence features with the adult form. Anatomic differences in affected areas have been described; however, the oral cavity has been frequently involved. Desquamative gingivitis, synechia in MMP may be observed but may rarely lead to functional disturbance, as in our patient who had difficulty in buccal opening.[4],[5] Pruritus, discomfort, dysuria, and vulvar pain are the main symptoms of vulvar MMP.[6] While oral lesions often do not lead to scars, vulvar MMP may lead to synechia of the labia minora and majora with narrowing of the vaginal orifice and may compromise sexual activity.[4],[5],[6],[7],[8],[9],[10] Ocular involvement in MMP may be the most functionally significant, severe disorder, as it can lead to blindness or visual loss (as in our patient) because of fibrosis and scar formation. Ocular lesions consist of chronic conjunctivitis, progressive subepithelial fibrosis on the conjunctival fornix, symblepharon formation, obstruction of the Meibomian ducts, sicca syndrome, and entropion associated with consequent trichiasis and corneal ulcers.

DIF shows linear IgG and often C3 deposits on the basement membrane zone, as observed in our patient and less commonly IgA or IgM. Using indirect immunofluorescence, preferably performed on salt-split skin, the majority of patients' sera bind to the epidermal aspect of the split skin and in a minority bind dermally. The target antigens found in immunoblotting and immunoprecipitation studies are the common bullous pemphigoid antigens (BP 180 and BP 230), the anchoring filament component laminin 5, and also the cytoplasmic domain of b4 integrin.[5],[9]

No absolutely effective agent for MMP has been proposed and the prognosis for childhood MMP varies according to the localization of the disease. Few case reports show good response to topical corticosteroid therapy or oral dapsone. Mild cases of childhood vulvar CP respond well to potent topical steroids. Topical tacrolimus can also be used. However, more severe cases had required systemic therapy with prednisolone, dapsone, or azathioprine, or associated therapy with two or three of these drugs. Cyclophosphamide is shown best ocular remission; however, its use in pediatric age group for long time has been related to especially growth retardation, cancer, and infertility. Other drugs that may be used are intravenous immunoglobulin and rituximab. Surgical intervention may be needed in the presence of severe scarring, deformation, or to allow normal sexual function. In ocular lesions in CP, early diagnosis and treatment are necessary to avoid scarring and visual loss.

We are reporting this case of adolescent MMP due to its rarity in this age group and involvement of all the mucosal surfaces as well as cutaneous involvement.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. The first international consensus on mucous membrane pemphigoid: Definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 2002;138:370-9.  Back to cited text no. 1
    
2.
Wojnarowska F, Marsden RA, Bhogal B, Black MM. Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear igA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol 1988;19:792-805.  Back to cited text no. 2
    
3.
Kharfi M, Khaled A, Anane R, Fazaa B, Kamoun MR. Early onset childhood cicatricial pemphigoid: A case report and review of the literature. Pediatr Dermatol 2010;27:119-24.  Back to cited text no. 3
    
4.
Roche C, Field EA. Benign mucous membrane pemphigoid presenting as desquamative gingivitis in a 14-year-old child. Int J Paediatr Dent 1997;7:31-4.  Back to cited text no. 4
    
5.
Lourenço SV, Boggio P, Agner Machado Martins LE, Santi CG, Aoki V, Menta Simonsen Nico M, et al. Childhood oral mucous membrane pemphigoid presenting as desquamative gingivitis in a 4-year-old girl. Acta Derm Venereol 2006;86:351-4.  Back to cited text no. 5
    
6.
Farrell AM, Kirtschig G, Dalziel KL, Allen J, Dootson G, Edwards S, et al. Childhood vulval pemphigoid: A clinical and immunopathological study of five patients. Br J Dermatol 1999;140:308-12.  Back to cited text no. 6
    
7.
Cheng YS, Rees TD, Wright JM, Plemons JM. Childhood oral pemphigoid: A case report and review of the literature. J Oral Pathol Med 2001;30:372-7.  Back to cited text no. 7
    
8.
Schoeffler A, Roth B, Causeret A, Kanitakis J, Faure M, Claudy A, et al. Vulvar cicatricial pemphigoid of childhood. Pediatr Dermatol 2004;21:51-3.  Back to cited text no. 8
    
9.
Hoque SR, Patel M, Farrell AM. Childhood cicatricial pemphigoid confined to the vulva. Clin Exp Dermatol 2006;31:63-4.  Back to cited text no. 9
    
10.
Iovine A, Fimiani F, Vassallo P, Alessio M, Magli A. Ocular manifestations in a case of childhood cicatricial pemphigoid. Eur J Ophthalmol 2008;18:636-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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