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Year : 2021  |  Volume : 8  |  Issue : 2  |  Page : 64-67

A case of IgA pemphigus in a five-year-old child

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 Submission29-Nov-2020
Date of Decision04-Jul-2021
Date of Acceptance23-Jul-2021
Date of Web Publication06-Oct-2022

Correspondence Address:
Sebastian Criton
Department of Dermatology, Amala Institute of Medical Sciences, Thrissur, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijdpdd.ijdpdd_122_20

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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 Top

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.[1] 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.[1]

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 Top

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 2: Subcorneal blister containing neutrophils (H&E 40x×)

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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 Top

Unlike typical pemphigus vulgaris, IgA pemphigus is characterized by tissue bound and circulating IgA autoantibodies that target desmosomal proteins of the epidermis.[2] Two distinct types of IgA pemphigus have been described: the subcorneal pustular dermatosis type and the intraepidermal neutrophilic type.[3] Both these forms may be clinically indistinguishable from subcorneal pustular dermatosis (Sneddon–Wilkinson disease).[1] 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.[3] 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.[4]

Some cases may resemble pemphigus foliaceous or pemphigus herpetiformis. Flexural, oozing, verrucous plaques mimicking pemphigus vegetans have also been reported.[1] 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.[5],[6],[7],[8],[9],[10],[11],[12],[13]

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.[2] 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.[1]

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.[2] 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.[5] 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.

  References Top

Schmidt E, Groves R Immunobullous disease. In: Griffiths C, Barker J, Bleicker T, Chalmers R, Creamer D, editors. Rook’s textbook of dermatology. 9th ed.2016; Vol. 2. Chichester; Hoboken, NJ: Wiley-Blackwell. p. 50.7.  Back to cited text no. 1
Amagai M Pemphigus, section 5—Vesiculobullous diseases. In: Bolognia JL, Schaffer JV, Cerroni L, editors. Dermatology. 4th ed. Philadelphia, PA: Elsevier Saunders; 2018. Vol. 1. pp. 502-9.  Back to cited text no. 2
Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, et al. IgA pemphigus. Clin Dermatol 2011;29:437-42.  Back to cited text no. 3
Chapman CM, Kwock J, Cresce N, Privette E, Cropley T, Gru AA IgG/IgA pemphigus in a patient with a history of pemphigus vulgaris: An example of epitope spreading? J Cutan Pathol 2019;46:380-2.  Back to cited text no. 4
Caputo R, Pistritto G, Gianni E, Carminati G, Crupi A, Berti E, et al. IgA pemphigus in a child. J Am Acad Dermatol 1991;25: 383-6.  Back to cited text no. 5
Bruckner AL, Fitzpatrick JE, Hashimoto T, Weston WL, Morelli JG Atypical IgA/IgG pemphigus involving the skin, oral mucosa, and colon in a child: A novel variant of IgA pemphigus? Pediatr Dermatol 2005;22:321-7.  Back to cited text no. 6
Suzuki M, Karube S, Kobori Y, Usui K, Murata S, Kato H, et al. IgA pemphigus occurring in a 1-month-old infant. J Am Acad Dermatol 2003;48:S22-4.  Back to cited text no. 7
de Oliveira JP, Gabbi TV, Hashimoto T, Aoki V, Santi CG, Maruta CW, et al. Two Brazilian cases of IgA pemphigus. J Dermatol 2003;30:886-91.  Back to cited text no. 8
Neethu KC, Rao R, Balachandran C, Pai S Juvenile IgA pemphigus: A case report and review of literature. Indian J Dermatol Venereol Leprol 2016;82:439-42.  Back to cited text no. 9
Weston WL, Friednash M, Hashimoto T, Seline P, Huff JC, Morelli JG A novel childhood pemphigus vegetans variant of intraepidermal neutrophilic IgA dermatosis. J Am Acad Dermatol 1998;38:635-8.  Back to cited text no. 10
Saurat JH, Merot Y, Salomon D, Didierjean L Pemphigus-like IgA deposits and vesiculo-pustular dermatosis in a 10-year-old girl. Dermatology 1987;175:96-100.  Back to cited text no. 11
Gooptu C, Mendelsohn S, Amagai M, Hashimoto T, Nishikawa T, Wojnarowska F Unique immunobullous disease in a child with a predominantly IgA response to three desmosomal proteins. Br J Dermatol 1999;141:882-6.  Back to cited text no. 12
Teraki Y, Amagai N, Hashimoto T, Kusunoki T, Nishikawa T Intercellular IgA dermatosis of childhood. Selective deposition of monomer IgA1 in the intercellular space of the epidermis. Arch Dermatol 1991;127:221-4.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3]


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