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CORRESPONDENCE Table of Contents  
Ahead of print publication
Intraepidermal neutrophilic dermatosis-type immunoglobulin A pemphigus

1 Department of Dermatology, Taipei Veterans General Hospital, Taipei, Taiwan
2 Department of Dermatology, Taipei Veterans General Hospital; Department of Dermatology, National Yang-Ming University, Taipei, Taiwan
3 Department of Dermatology, Taipei Veterans General Hospital; Department of Public Health, Institute of Public Health, National Yang-Ming University, Taipei, Taiwan

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Date of Submission12-May-2020
Date of Decision04-Jul-2020
Date of Acceptance08-Jul-2020
Date of Web Publication05-Feb-2021

How to cite this URL:
Hong H, Chang TH, Wu CY, Chang YT. Intraepidermal neutrophilic dermatosis-type immunoglobulin A pemphigus. Dermatol Sin [Epub ahead of print] [cited 2021 Feb 28]. Available from: https://www.dermsinica.org/preprintarticle.asp?id=308747

Dear Editor,

Immunoglobulin (Ig) A pemphigus is a rare type of autoimmune bullous dermatosis characterized by intercellular deposits of IgA in the epidermis.[1],[2] IgA pemphigus can be primarily classified into subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic (IEN) dermatosis.[3] Herein, we report a HLA-B*13:01-positive case of IEN-type IgA pemphigus successfully treated with oral corticosteroids.

A 63-year-old male presented with a 1-week history of a blistering eruption. The eruption started as pruritic papules, vesicles, and pustules on the chest and buttocks. The lesions gradually increased in number and appeared on the face, scalp, axillae, back, inguinal folds, and proximal extremities. Many lesions expanded peripherally to form oval or round plaques with central crusting and peripheral beading of pustules [Figure 1]. The patient had dyslipidemia and was started on regular use of rosuvastatin 1 month before the development of eruption. He had no known food or drug allergies.
Figure 1: Multiple pustules coalescing into annular plaques with central crusting on (a) the face and scalp, (b) chest, and (c) back. (d) A close-up view of the annular pustules on the chest

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Histologically, there were intraepidermal clefts filled with a dominant number of neutrophils, along with a few lymphocytes and eosinophils [Figure 2]. Some acantholytic keratinocytes were also present. There was moderate perivascular lymphohistiocytic and neutrophilic infiltration in the upper dermis. No pathogens were identified by Gram stain, periodic acid-Schiff stain, or acid-fast stain. Direct immunofluorescence microscopy demonstrated intercellular deposition of IgA autoantibodies throughout the whole layer of the epidermis. There was no deposition of C3, IgG, and IgM in the epidermis. The workup for monoclonal gammopathy showed a normal serum level of IgA antibodies. Results of serum electrophoresis were unremarkable.[4]
Figure 2: Microscopic findings of the specimen taken from a pustular lesion on the chest. (a) The scanning view showed an intraepidermal cleft with abundant infiltration. (b and c) The inflammatory cells were primarily neutrophils, along with some lymphocytes and eosinophils. Acantholytic keratinocytes were present. (d) Direct immunofluorescence microscopy demonstrated an intercellular pattern of immunoglobulin A deposition throughout the whole layer of the epidermis

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We intended to treat the patient with dapsone. Therefore, the patient was examined for the glucose-6-phosphate dehydrogenase (G6PD) level, and the allele HLA-B*13:01 was determined. The results showed no G6PD deficiency but the presence of the allele HLA-B*13:01.[5],[6] To avoid the occurrence of severe cutaneous adverse reactions, we treated the patient with oral prednisolone. The initial dose of prednisolone was 1 mg/kg/day. The pustules gradually crusted, without the development of new lesions after initiating the therapy. Two weeks later, the patient was discharged with a tapering course of prednisolone. He has been regularly followed up at our clinic for 2 years, and there are no signs of recurrence. Rosuvastatin was continued during the follow-up period, and drug-induced pemphigus was excluded.

The most commonly encountered subtypes of IgA pemphigus are SPD and IEN. Histologically, the SPD type is characterized by the presence of subcorneal neutrophilic pustules, whereas the IEN type is characterized by neutrophilic abscesses distributed in any layer of the epidermis. In the immunofluorescence study, the SPD type is characterized by intercellular deposition of IgA autoantibodies in the upper epidermis, whereas the IEN type is characterized by intercellular deposition of IgA autoantibodies in the lower or entire epidermis.

Dapsone is effective in inhibiting neutrophilic chemotaxis and is regarded as a first-line treatment for IgA pemphigus.[7],[8] However, dapsone should be used with caution owing to its common pharmacologic and idiosyncratic adverse effects in vulnerable individuals. Evidence has indicated a strong association between HLA-B*13:01 and dapsone-induced severe cutaneous adverse reactions.[5],[6],[9] The prevalence of HLA-B*13:01 varies across races, with the highest prevalence among Chinese population, which is up to 15%–20%.[6] Therefore, routine screening of HLA-B*13:01 may be necessary for Chinese people before the dapsone therapy. HLA-B*13:01-positive patients with IgA pemphigus should be alternatively treated with corticosteroids or other less frequently used immunosuppressants.[1]

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kridin K, Patel PM, Jones VA, Cordova A, Amber KT. IgA pemphigus: A systematic review. J Am Acad Dermatol 2020;82:1386-92.  Back to cited text no. 1
Yang SJ, Lee HE, Chang CH, Chen CB. IgG/IgA pemphigus presenting with mixed clinical features of pemphigus erythematosus and IgA pemphigus. Dermatol Sin 2019;37:226-8.  Back to cited text no. 2
  [Full text]  
Wallach D. Intraepidermal IgA pustulosis. J Am Acad Dermatol 1992;27:993-1000.  Back to cited text no. 3
Aste N, Fumo G, Pinna AL, Biggio P. IgA pemphigus of the subcorneal pustular dermatosis type associated with monoclonal IgA gammopathy. J Eur Acad Dermatol Venereol 2003;17:725-7.  Back to cited text no. 4
Zhang FR, Liu H, Irwanto A, Fu XA, Li Y, Yu GQ, et al. HLA-B*13:01 and the dapsone hypersensitivity syndrome. N Engl J Med 2013;369:1620-8.  Back to cited text no. 5
Tangamornsuksan W, Lohitnavy M. Association between HLA-B*1301 and dapsone-induced cutaneous adverse drug reactions: A systematic review and meta-analysis. JAMA Dermatol 2018;154:441-6.  Back to cited text no. 6
Grossman S, Budinsky R, Jollow D. Dapsone-induced hemolytic anemia: Role of glucose-6-phosphate dehydrogenase in the hemolytic response of rat erythrocytes to N-hydroxydapsone. J Pharmacol Exp Ther 1995;273:870-7.  Back to cited text no. 7
Wozel G, Blasum C. Dapsone in dermatology and beyond. Arch Dermatol Res 2014;306:103-24.  Back to cited text no. 8
Chen WT, Wang CW, Lu CW, Chen CB, Lee HE, Hung SI, et al. The function of HLA-B*13:01 Involved in the pathomechanism of dapsone-induced severe cutaneous adverse reactions. J Invest Dermatol 2018;138:1546-54.  Back to cited text no. 9

Correspondence Address:
Yun-Ting Chang,
Department of Dermatology, Taipei Veterans General Hospital, No. 201, Sec. 2, Shih-Pai Road, Taipei
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ds.ds_33_20


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