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Year : 2013  |  Volume : 16  |  Issue : 3  |  Page : 124-126

Diagnosing linear immunoglobulin - A dermatosis of childhood in a resource-constrained area: Case report and review of literature

1 Department of Medicine, Aminu Kano Teaching Hospital and Bayero University, Kano, Nigeria
2 Department of Pathology, Aminu Kano Teaching Hospital and Bayero University, Kano, Nigeria

Date of Web Publication22-Nov-2013

Correspondence Address:
Muhammad S Mijinyawa
Department of Medicine, Aminu Kano Teaching Hospital and Bayero University, Kano
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DOI: 10.4103/1118-8561.121924

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Linear immunoglobulin A (IgA) dermatosis (LAD) of childhood is a self-limiting blistering eruption. At histology, the blisters are subepidermal and may be reported as resembling dermatitis herpetiformis or bullous pemphigoid. Direct immunofluorescence (DIF) of normal or perilesional skin shows a linear band of IgA at the basement membrane. Many of the patients also have a circulating IgA antibody against the basement membrane. Diagnosis of this condition in a resource constrainedcenter, where immunofluorescence is unavailable, may pose diagnostic challenges, and may therefore require high index of suspicion. We describe a case of LAD of childhood in a 7-year-old boy in this report.

Keywords: Blisters, dermatosis, linear IgA, rash

How to cite this article:
Mijinyawa MS, Yusuf SM, Mohammed AZ, Nalado AM. Diagnosing linear immunoglobulin - A dermatosis of childhood in a resource-constrained area: Case report and review of literature. Sahel Med J 2013;16:124-6

How to cite this URL:
Mijinyawa MS, Yusuf SM, Mohammed AZ, Nalado AM. Diagnosing linear immunoglobulin - A dermatosis of childhood in a resource-constrained area: Case report and review of literature. Sahel Med J [serial online] 2013 [cited 2021 Jul 31];16:124-6. Available from: https://www.smjonline.org/text.asp?2013/16/3/124/121924

  Introduction Top

Linear immunoglobulin A (IgA) dermatosis (LAD) is a self-limiting, benign chronic bullous dermatosis of childhood that usually starts before the age of 6 years. The rashes are characterized by tense, usually large, and often clustered blisters that arise on normal skin. The blisters may be generalized or grouped in the pelvic and perioral regions. This case report highlights the challenges in diagnosing blistering disorders in resource-poor settings.

  Case Report Top

A 7-year-old boy presented with a year's history of widespread blistering eruptions the appearance of which was abrupt. He complained of sudden appearance of large itchy blisters all over the body including the limbs, about a year before we saw him. He had no history of administration of any drugs prior to the onset of the rashes. There were no symptoms or signs of malabsorption. Review of systems was unremarkable. He had visited several centers where prescriptions of several topical and oral steroids as well as oral and topical antibiotics were given but without improvement.

On examination, the blisters were tense and large with additional groups of small vesicles. Most of the blisters arose on normal skin. They were more profuse on the face, neck, upper chest, and pelvic region. The palms and soles were not affected and no mucosal lesion was seen. Typical "jewel-like" clustering around healing lesions was seen around the chest [Figure 1]. There were post-inflammatory hypo- and hyper-pigmentation with no scarring.

The patient's blood sample showed normal hematologicalparameters and serological evaluation for hepatitis B surface antigen (HBsAg) and hepatitis C virus (HCV) antibodies were negative.
Figure 1: The patient on the day of presentation and a week after treatment

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Histopathology of the affected skin specimen showed a subepidermal blister and an edematous papillary dermis infiltrated with inflammatory cells, mainly neutrophils and eosinophils [Figure 2]. Direct immunofluorescence (DIF) and indirect immunofluorescence were not done due to non-availability in the center. A presumptive diagnosis of LAD of childhood was made based on the typical clinical characteristics and distribution of the lesions. Therapeutic trail with dapsone, 100 mg/day, resulted in a dramatic improvement of both the skin eruptions and the itching. The resolution of the rashes was complete by 2 weeks [Figure 1]. The dosage of the drug was maintained for 2 months and was then gradually tapered off over another month.
Figure 2: Subepidermal bulla with eosinophils and neutrophils and papillary abscesses

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  Discussion Top

Linear immunoglobulin A (IgA) dermatosis (LAD) is an acquired autoimmune subepidermal blistering disorder, which was first described and defined as a disease entity by Chorzelski et al. in 1979. [1] Both chronic bullous dermatosis of childhood and linear IgA bullous dermatosis of adults are considered as being the same disease; hence, the proposed name, linear bullous dermatosis of adults and childhood. [2] The disease in children begins usually in the preschool age, with a mean of 4-5 years. [3],[4] Typically, the lesions are localized in the lower abdomen and anogenital areas with frequent involvement of the perineum. The other sites include face, especially the perioral area. Lesions may be symmetrically or asymmetrically distributed. [5] The clinical presentation and the histopathologic appearance of LAD is similar to that of both dermatitis herpetiformis and bullous pemphigoid. Immunofluorescence studies are very important for the diagnosis of LAD. Direct immunofluorescence of perilesional and normal skin shows typically a predominant linear deposition of IgA along the basement membrane. [6] Complement (C3) deposition is frequent and immunoglobulin G (IgG) or immunoglobulin M (IgM) can also be present at the same time as IgA antibodies. In some cases, the DIF can be negative and the dermatosis can be mistaken for an infectious disease leading to a considerable delay in the treatment. [7] The most important difference between dermatitis herpetiformis with granular IgA deposits and linear IgA bullous disease is the absence in the latter of nontropical sprue. [6],[7] A gluten-free diet, which is helpful in dermatitis herpetiformis if strictly observed, is of no benefit in cases of linear IgA bullous dermatosis. [7] It is generally accepted that dapsone is the drug of choice in linear IgA bullous dermatosis. Most patients will respond to 100-150 mg of dapsone with clearing of the skin lesions. The dosage is then gradually lowered to a maintenance level of 50 mg every second day is given. The response is rapid, usually within 72 h. In patients who do not respond adequately, prednisone in small doses may be added as adjunctive therapy. [8],[9] Our patient was started on 100 mg of dapsone and by the second week most of the lesions had cleared and no new eruptions were observed.

The diagnosis of LAD is based on the following criteria: Itchy vesiculobullous lesions on skin and/or mucous membranes, subepidermal bulla with neutrophilic infiltrate, linear IgA deposits along the epidermal basement membrane, and prompt response to treatment with dapsone. Remission is usually seen in a majority of patients with linear IgA bullous dermatosis of childhood within 2-4 years, and almost always before puberty. [10]

In a resource-poor center like ours, where facilities for immunofluorescence studies are not readily available, prompt response to treatment with dapsone in the presence of characteristic clinical and histological features may clinch the diagnosis.

  References Top

1.Chorzelski T, Jablonska S, Beutner E. Adult form of linear IgA bullous dermatosis. In: Beutner E, Chorzelski T, Bean S, Editors. Immunopathology of the Skin. New York: Wiley; 1979. p. 316-9.  Back to cited text no. 1
2.Wojnarowska F, Bhogal BS, Black MM. Chronic bullous disease of childhood and linear IgA disease of adults are IgA1-mediated diseases. Br J Dermatol 1994;131:201-4.  Back to cited text no. 2
3.Nanda A, Dvorak R, Al-Sabah H, Alsaleh QA. Linear IgA bullous disease of childhood: An experience from Kuwait. Pediatr Dermatol 2006;23:443-7.  Back to cited text no. 3
4.Hruza LL, Mallory SB, Fitzgibbons J, Mallory GB Jr. Linear IgA bullous dermatosis in a neonate. Pediatr Dermatol 1993;10:171-6.  Back to cited text no. 4
5.Brenner S, Mashiah J. Autoimmune blistering diseases in children: Signposts in the process of evaluation. Clin Dermatol 2000;18:711-24.  Back to cited text no. 5
6.Chorzelski T, Jablonska S. IgA lineadermatosis of childhood (chronic bullous disease of childhood). Br J Dermatol 1979;101:535-42.  Back to cited text no. 6
7.Bhogal B, Wojnarowska F, Marsden R, Das A, Black MM, McKee PH. Linear IgA bullous dermatosis of adults and children: An immunoelectron microscopic study. Br J Dermatol 1987;117:289-96.  Back to cited text no. 7
8.Marsden RA. The treatment of benign chronic bullous dermatosis of childhood, and dermatitis herpetiformis and bullous pemphigoid beginning in childhood. Clin Exp Dermatol 1982;7:653-63.  Back to cited text no. 8
9.Wojnarowska F, Marsden RA, Bhogal B, Black MM.Chronic bullous disease of childhood, childhood cicatricialpemphigoid, 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. 9
10.Monia K, Aida K, Amel K, Ines Z, Becima F, et al. (2011) Linear IgA bullous dermatosis in Tunisian children: 31 cases. Indian J Dermatol 56: 153-159.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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