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Linear immunoglobulin A (IgA) bullous dermatosis

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K Rajendran
K Rajendran
K Rajendran
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Abhijit Patil
Abhijit Patil
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Abstract

A 3 year old boy presented with blisters all over the body. Skin biopsy taken from the leg was consistent with linear IgA bullous dermatosis. Direct immunofluorescence showed a linear basement membrane zone band with IgA, confirming linear IgA disease. He was successfully treated with intravenous methylprednisolone (1.5mg/kg/day) for 5 days followed by oral steroid (1mg/kg) and oral dapsone (1mg/kg). He is on regular follow up and his skin lesions have improved.
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Linear immunoglobulin A (IgA) bullous dermatosis Sri Lanka Journal of Child Health, 2018; 47: 271-272
271
Linear immunoglobulin A (IgA) bullous dermatosis
*K Rajendran
1
, Abhijit Anil Patil
1
Sri Lanka Journal of Child Health, 2018; 47: 271-272
DOI: http://dx.doi.org/10.4038/sljch.v47i3.8552
(Keywords: Linear IgA dermatosis, immunofluorescence, dapsone)
Introduction
Linear immunoglobulin A (IgA) bullous dermatosis
(LABD), first described by Bowen in 1901, is an
autoimmune disease occurring in both children and
adults
1,2
. Around two thirds of cases are induced by
antibiotics, nonsteroidal anti-inflammatory agents
and diuretics
2
. Childhood LABD is synonymous
with chronic bullous disease of childhood (CBDC)
3
.
Case report
A 3 year old boy presented with blisters all over the
body, more over the lower limbs, upper limbs and
ear lobes, since 10 days associated with severe
itching and mild pain (Figure 1).
Ruptured blisters were also present. There was no
history of fever. The blisters started as small papules
on the lower limbs and spread all over the body.
Total white blood cell count was 11,200 /cu mm
(24% polymorphs, 50% lymphocytes, 24%
eosinophils, 2% monocytes). Erythrocyte
sedimentation rate was 8 mm in the first hour,
_________________________________________
1
Department of Paediatrics, Kovai Medical Centre
and Hospital, Coimbatore, Tamil Nadu, India
*Correspondence:
drrajendrantk@gmail.com
(Received on 02 January 2017: Accepted after
revision on 20 February 2017)
The authors declare that there are no conflicts of
interest
Personal funding was used for the project.
Open Access Article published under the Creative
Commons Attribution CC-BY License
the haemoglobin (Hb) level 12.7 g/dl and the platelet
count 458,000 /cu mm. Serum immunoglobulin E
(IgE) level was 112.4 IU/ml (normal up to 60
IU/ml), serum immunoglobulin A (IgA) level 72
mg/dl (normal 19-220 mg/dl), serum
immunoglobulin M (IgM) level 167 mg/dl (normal
40-180 mg/dl) and the serum immunoglobulin G
(IgG) level 800 mg/dl (normal 500-1300 mg/dl). The
gram stain of blister fluid showed a few pus cells but
no organisms. Skin biopsy taken from the leg was
consistent with linear IgA bullous dermatosis. Direct
immunofluorescence showed a linear basement
membrane zone band with IgA, confirming linear
IgA disease. Glucose-6-phosphate-dehydrogenase
(G6PD) level was 11 units/g Hb (normal range 4.6-
13.5 units/g Hb). He was successfully treated with
intravenous methylprednisolone (1.5mg/kg/day) for
5 days followed by oral steroid (1mg/kg) and oral
dapsone (1mg/kg). He is on regular follow up and
his skin lesions have improved (Figure 2).
Discussion
Prevalence of LABD in children is unknown. Age of
onset in children ranges from 1 to 10 years
4
. Our
patient was 3 years old.
Childhood LABD has rarely
been associated with ulcerative colitis and
autoimmune lymphoproliferative disease
5,6
. LAPD
characteristically has linear IgA deposits in the
basement membrane zone of the skin, circulating
basement membrane zone antibodies being detected
in over 80% of patients
3
. Our patient showed a linear
basement membrane zone band with IgA on
immunofluorescence. How drugs stimulate the
immune system to produce IgA antibodies against
the basement membrane in LABD is still unknown
5
.
Both idiopathic and drug-induced LABD can be
triggered by physical trauma, burns, or infections
3
.
There were no triggering factors noted in our case.
Typically, there is an interval of 2 to 28 days
between the drug involved and the bullous eruption
2
.
Linear immunoglobulin A (IgA) bullous dermatosis Sri Lanka Journal of Child Health, 2018; 47: 271-272
272
Lesions of drug-induced LABD resolve
spontaneously within 2 to 7 weeks following
withdrawal of the drug. No drug was incriminated in
our patient. In a study by Wojnarowska F, et al. of
25 cases of childhood LABD, 64% remitted within
2 years and only in 12% was the disease active after
puberty
8
. The treatment of choice in LABD is
dapsone alone or in combination with systemic
steroids
9,10
. Our patient was treated with dapsone
and steroids. Dapsone may cause haemolysis in
G6PD deficient individuals. In our patient the G6PD
level was normal. Flucloxacillin may be considered
as an alternative therapy
11
. In dapsone resistant
cases, tacrolimus ointment may be beneficial
12
.
References
1. Bowen JT. Six cases of bullous dermatitis
following vaccination, and resembling
dermatitis herpetiformis. Journal of
Cutaneous Disease 1901; 19: 401-23.
2. Ho JC, Ng PL, Tan SH, Giam YC. Childhood
linear IgA bullous disease triggered by
amoxicillin-clavulanic acid. Paediatric
Dermatology 2007; 24(5): E40-3.
https://doi.org/10.1111/j.15251470.2007.004
38.x
PMid: 17958778
3. Polat M, Lenk N, Kurekci E, Oztas P, Artuz
F, Alli N. Chronic bullous disease of
childhood in a patient with acute
lymphoblastic leukemia: possible induction
by a drug. American Journal of Clinical
Dermatology 2007; 8(6): 389-91.
https://doi.org/10.2165/00128071200708060
-00010
PMid: 18039023
4. Nantel-Battista M, Dhaybi RA, Hatami A,
Marcoux D, DesRoches A, Kokta V.
Childhood linear IgA bullous disease induced
by trimethoprim-sulfamethoxazole. Journal
of Dermatological Case Reports 2010; 4(3):
33-5.
https://doi.org/10.3315/jdcr.2010.1053
PMid: 21886746 PMCid: PMC3157814
5. Wong CS, Arkwright PD, Rieux-Laucat F,
Cant AJ, Stevens RF, Judge MR. Childhood
linear IgA disease in association with
autoimmune lymphoproliferative syndrome.
British Journal of Dermatology 2004; 150(3):
578-80.
https://doi.org/10.1111/j.13652133.2004.058
50.x
PMid: 15030346
6. Handley J, Shields M, Walsh M, Bingham A.
Chronic bullous disease of childhood and
ulcerative colitis. British Journal of
Dermatology 1992; 127:67–8.
https://doi.org/10.1111/j.13652133.1992.tb0
1256.x
7. Egan CA, Zone JJ. Linear IgA bullous
dermatosis. International Journal of
Dermatology 1999; 38:818–27.
https://doi.org/10.1046/j.13654362.1999.008
13.x
8. 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. Journal of the
American Academy of Dermatology 1988;
19(5 Pt 1): 792-805.
https://doi.org/10.1016/S01909622(88)7023
6-4
9. Nanda A, Dvorak R, Al-Sabah H, Alsaleh
QA. Linear IgA bullous disease of childhood:
an experience from Kuwait. Pediatric
Dermatology 2006; 23(5): 443-7.
https://doi.org/10.1016/S01909622(88)7023
6-4
https://doi.org/10.1111/j.15251470.2006.002
79.x
PMid: 17014638
10. Monia K, Aida K, Amel K, Ines Z, Becima F,
et al. () Linear IgA bullous dermatosis in
Tunisian children: 31 cases. Indian Journal of
Dermatology 2011; 56: 153-9.
PMid: 21716539 PMCid: PMC3108513
11. Alajlan A, Al-Khawajah M, Al-Sheikh O, Al-
Saif F, Al-Rasheed S, et al. Treatment of
linear IgA bullous dermatosis of childhood
with flucloxacillin. Journal of the American
Academy of Dermatology 2006; 54: 652-6.
https://doi.org/10.1016/j.jaad.2005.11.1102
PMid: 16546588
12. Dauendorffer JN, Mahé E, Saiag P.
Tacrolimus ointment, an interesting
adjunctive therapy for childhood linear IgA
bullous dermatosis. Journal of the European
Academy of Dermatology and Venereology
2008; 22(3): 364-5.
https://doi.org/10.1111/j.14683083.2007.023
15.x
PMid: 18269605
ResearchGate has not been able to resolve any citations for this publication.
Linear IgA Bullous Disease of Childhood: An Experience from Kuwait
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Linear IgA bullous disease of childhood is a rare autoimmune blistering disease. We report eight patients with this disease seen in our autoimmune bullous diseases clinic over a span of 12 years. They constituted 89% of the total number of those with linear IgA bullous disease of childhood seen during this period, with an age-adjusted minimum estimated incidence of 2.3 cases/million population/year. Males outnumbered females by a 1.7:1 ratio. The age at onset ranged between 10.5 months and 13 years, with a mean of 6.8 +/- 4.17 years. The majority of patients (62.5%) had moderately severe disease. Fifty percent of patients were observed to have an association with either an autoimmune disease (Crohn disease and post-streptococcal glomerulonephritis in one each) or an infection (beta-hemolytic streptococcal and hepatitis A virus infection in one each). The treatment of choice was dapsone alone or in combination with systemic steroids. Seventy-one percent of patients achieved complete remission by the end of 2 years. The study highlights the significance of systematic clinicoepidemiologic surveys from different regions.
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Childhood linear IgA bullous disease induced by trimethoprim-sulfamethoxazole
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Linear IGA bullous dermatosis in Tunisian children: 31 Cases
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Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa. We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs) in Tunisian children. We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population. Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F) of 2.4. Most of the children had generalized eruption (28/31), more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%). The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal-epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients. This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.
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Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults
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Linear IgA bullous dermatosis of childhood is a rare autoimmune bullous disease that mainly affects preschool-aged children. Dapsone is considered the first-line therapy with prompt response from most patients. However, it may be contraindicated in certain conditions such as glucose-6-phosphate dehydrogenase deficiency. We sought to assess the efficacy of flucloxacillin in the treatment of linear IgA bullous dermatosis. This is an observational study in which all confirmed cases of linear IgA bullous dermatosis (by both histological and immunofluorescence studies) will be treated with flucloxacillin. Flucloxacillin will be continued according to the response or otherwise will be discontinued after 8 weeks in the case of resistance. We describe 7 patients with linear IgA bullous dermatosis of childhood treated with flucloxacillin. In 4 cases, it induced complete remission within 3 to 4 months of starting therapy with no relapses. In the other 3 cases, it successfully controlled the disease but with prompt relapse on discontinuation of the treatment. This is a case series study with a small number of patients. Flucloxacillin may be considered among the first alternative therapies for linear IgA bullous dermatosis of childhood. Further evaluation of the efficacy and safety of the long-term use is required.
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Childhood Linear IgA Bullous Disease Triggered by Amoxicillin-Clavulanic Acid
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Linear immunoglobulin A bullous disease is an autoimmune subepidermal blistering disease that has been described in both children and adults. Reports have shown that as many as two-thirds of occurrences may be drug-induced. The offending drugs include antibiotics, predominantly vancomycin, nonsteroidal anti-inflammatory agents and diuretics. We report childhood linear immunoglobulin A bullous dermatosis developing following amoxicillin-clavulanic acid administration. The patient presented with characteristic blisters in an annular fashion, likened to a ''crown of jewels.'' The diagnosis was confirmed by the presence of a linear band of immunoglobulin A at the dermoepidermal junction on direct immunofluorescence. The lesions resolved with withdrawal of the drug, and systemic therapy was not required. We review the current literature and concepts of drug-induced linear immunoglobulin A bullous disease.
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