|Year : 2021 | Volume
| Issue : 2 | Page : 64-67
A case of IgA pemphigus in a five-year-old child
Anjali Rose Jose1, Sebastian Criton1, Usha Mary Abraham2
1 Department of Dermatology, Amala Institute of Medical Sciences, Thrissur, Kerala, India
2 Department of Pathology, Amala Institute of Medical Sciences, Thrissur, Kerala, India
|Date of Submission||29-Nov-2020|
|Date of Decision||04-Jul-2021|
|Date of Acceptance||23-Jul-2021|
|Date of Web Publication||06-Oct-2022|
Department of Dermatology, Amala Institute of Medical Sciences, Thrissur, Kerala
Source of Support: None, Conflict of Interest: None
Immunoglobulin A (IgA) pemphigus is a group of autoimmune intraepidermal blistering diseases presenting with vesiculopustular eruptions, neutrophilic infiltration of the skin, and in vivo bound and circulating IgA autoantibodies against the cell surface of keratinocytes. It usually occurs in middle-aged or elderly persons. We report a case of childhood IgA pemphigus in a 5-year-old male child, with direct immunofluorescence confirmation, which is an uncommon occurrence.
Keywords: Childhood immunobullous diseases, childhood pemphigus, IgA pemphigus
|How to cite this article:|
Jose AR, Criton S, Abraham UM. A case of IgA pemphigus in a five-year-old child. Indian J Dermatopathol Diagn Dermatol 2021;8:64-7
|How to cite this URL:|
Jose AR, Criton S, Abraham UM. A case of IgA pemphigus in a five-year-old child. Indian J Dermatopathol Diagn Dermatol [serial online] 2021 [cited 2023 Feb 8];8:64-7. Available from: https://www.ijdpdd.com/text.asp?2021/8/2/64/357931
| Introduction|| |
Immunoglobulin A (IgA) pemphigus is a rare autoimmune blistering disease to occur in childhood. It is characterized by painful and pruritic vesiculopustular eruptions, due to circulating IgA antibodies against keratinocyte cell surface components. In IgA pemphigus, patients clinically present with flaccid vesicles or pustules on either erythematous or normal skin. The main sites of predilection are the axillae and groin, but can also involve the trunk, face, scalp, and proximal limbs.
Direct immunofluorescence (DIF) in IgA pemphigus shows IgA deposition on cell surfaces of epidermal keratinocytes, and many patients have detectable circulating IgA autoantibodies, as shown by indirect immunofluorescence microscopy.
Herein, we report a case of IgA pemphigus in a 5-year-old male child, who was presented with multiple blisters over various sites
| Case History|| |
A 5-year-old boy presented with 6 months history of recurrent blisters over various parts of the body. The child who was well 6 months back initially developed a few small blisters over the neck and then over axillae and groin. Blisters ruptured easily leaving erosions and crust. There was no history of any constitutional symptoms and mucosal involvement. The child showed no response to various antibiotics, given during illness.
General and systemic examinations were unremarkable. On dermatologic examination, there were clear small vesicles over the neck and few over the face, forearm, and (L) thigh [Figure 1]A and B. Vesicles were arranged in a “string of pearl pattern.”
|Figure 1: A. Vesicles arranged in an annular pattern over the forearm. (B) Few vesicles and crusted lesions over the neck|
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Histopathology of the vesicle showed a subcorneal collection of neutrophils in the epidermis, and the dermis showed perivascular and perifollicular infiltrate of neutrophils and lymphocytes [Figure 2]. DIF microscopy of peri-lesional skin showed intercellular IgA staining in the granular layer [Figure 3]. A final diagnosis of subcorneal pustular type IgA pemphigus was made.
|Figure 3: DIF showing positive (3+) IgA deposition in the intercellular zone|
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The child was started on oral prednisolone in a dose of 0.5 mg/kg/day. He showed excellent therapeutic response with almost complete resolution of lesions within 2 weeks of initiating treatment. However, he had a single episode of relapse, which was developed on tapering prednisolone dose. Hence, the dose of prednisolone was increased. The child became better and on regular follow-up now. There is no new blister formation.
| Discussion|| |
Unlike typical pemphigus vulgaris, IgA pemphigus is characterized by tissue bound and circulating IgA autoantibodies that target desmosomal proteins of the epidermis. Two distinct types of IgA pemphigus have been described: the subcorneal pustular dermatosis type and the intraepidermal neutrophilic type. Both these forms may be clinically indistinguishable from subcorneal pustular dermatosis (Sneddon–Wilkinson disease). The target antigen in SCPD type is desmocollin 1, whereas in IEN type desmoglein is the normal target.
The exact mechanism of disease is still unclear. It is suggested that IL-5 is activated in IgA pemphigus. Besides, γδ T cells are also reported to be involved. Another proposed mechanism is the epitope-spreading phenomenon, in which an inflammatory event releases a new target antigen, exposes them to the immune system, and then induces autoimmunity to new antigens.
Some cases may resemble pemphigus foliaceous or pemphigus herpetiformis. Flexural, oozing, verrucous plaques mimicking pemphigus vegetans have also been reported. Mucosal involvement is unusual. Most runs in a chronic indolent course.
The disease mainly affects adults, although childhood cases have also been reported. Only nine cases of childhood IgA pemphigus have been reported in the literature so far.,,,,,,,,
Unlike the usual sunflower-like configuration of pustules in IgA pemphigus, in our case, the child was presented with clear vesicles, which were arranged in a string of pearl pattern.
The characteristic histologic feature of IgA pemphigus is an intraepidermal pustule or vesicle containing predominantly neutrophils. The two forms of the disease are described based on the level of the intraepidermal blister. In the SCPD type, pustules are located subcorneally in the upper epidermis, whereas in the IEN type, suprabasal pustules involve the lower or entire epidermis. DIF will show IgA deposition on the keratinocyte cell surface. Histology and DIF features in our case were consistent with those of the characteristic findings in IgA pemphigus.
The subcorneal type has been reported to be most frequently associated with monoclonal IgA gammopathy. Other cases have been linked with HIV infection, inflammatory bowel disease, rheumatoid arthritis, and thiol drugs.
Dapsone is the drug of choice for patients with IgA pemphigus. Other treatment options include sulphapyridine, acitretin, low-to medium-dose prednisone as well as photochemotherapy (PUVA) or colchicine. Our patient was managed with low dose systemic steroids and he responded well to the treatment.
The main differential diagnosis to be considered in case of IgA pemphigus includes subcorneal pustular dermatosis, pemphigus foliaceous, bullous impetigo, dermatitis herpetiformis, linear IgA bullous dermatosis, and pustular psoriasis. Unlike IgA pemphigus, linear IgA dermatosis is characterized by tense blisters which are subepidermal and here IgA autoantibodies are directed against extracellular domain of BP 180 antigen.
Through this article, we report a case of juvenile IgA pemphigus in a 5-year-old male child, which responded well to oral steroids. The child is under follow-up for 6 months now and the disease is well controlled.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]