Acta Dermato-Venereologica 99-6CompleteContent | Page 6
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REVIEW ARTICLE
Drug-induced Linear IgA Bullous Dermatosis: A Case Report and
Review of the Literature
Judith LAMMER, Rüdiger HEIN, Sophie ROENNEBERG, Tilo BIEDERMANN and Thomas VOLZ
Department of Dermatology and Allergology, Technical University Munich, Munich, Germany
Linear IgA bullous dermatosis (LABD) is a rare subepi-
dermal autoimmune blistering disease characterized
by linear deposition of IgA along the basement mem-
brane zone. Although most reported cases are idiopa-
thic, there is a subset of patients with drug-induced
LABD. Various drugs have been associated with the
drug-induced form of the disease. This paper reviews
the literature on drugs reported to elicit linear IgA der-
matosis and its specific clinical presentation. In addi-
tion, a case report of a 77-year-old male patient with
linear IgA dermatosis induced by vancomycin is des-
cribed. The aim of this paper is to emphasize the need
to include this differential diagnosis in cases of sus-
pected adverse drug reactions, as well as to highlight
the role of drugs in LABD.
Key words: drug-induced linear IgA bullous dermatosis; vanco-
mycin; autoimmunity.
Accepted Feb 26, 2019; E-published Feb 27, 2019
Acta Derm Venereol 2019; 99: 508–515.
Corr: Thomas Volz, Department of Dermatology and Allergology, Techni-
cal University Munich, Biedersteiner Str. 29, DE-80802 Munich, Germany.
E-mail: [email protected]
L
inear IgA bullous dermatosis (LABD) is a rare
immune-mediated vesiculobullous disease with an
incidence of 0.2–2.3 cases per million-population per
year (1). LABD can occur both in children and in adults.
In children it is also named chronic bullous disease of
childhood, and presents, on average, at approximately
4.5 years of age (2, 3). In adults, 2 peaks are described
with regard to disease onset: teenage years and the 60s
(4). The childhood onset form is characterized by deve-
lopment of annular or polycyclic plaques and papules
with blistering around the edges (string-of-pearls sign),
primarily around the mouth and eyes, lower abdomen,
thighs, buttocks, genitals, wrists and ankles. In contrast,
the adult-onset form of LABD presents with lesions on
the trunk, head and limbs (3). In both children and adults,
the mucosa can be affected. The string-of-pearls sign is
less common in adults in comparison to the childhood
form. The subjective symptoms vary from almost none
or mild pruritus to severe burning (3). Applying direct
immunofluorescence to a skin biopsy, which shows
the presence of linear deposition of immunoglobulin A
(IgA) at the dermoepidermal junction, is mandatory to
confirm the diagnosis (5–9). In addition, deposition of
IgG and C3 may be detected (6). While most cases of
doi: 10.2340/00015555-3154
Acta Derm Venereol 2019; 99: 508–515
SIGNIFICANCE
Linear IgA bullous dermatosis (LABD) is a rare blistering
autoimmune disease. Although most cases are idiopathic,
numerous drugs have been identified as capable of elicit-
ing LABD. This paper provides an overview of the patho-
genesis, diagnosis and treatment of drug-induced LABD,
together with a report of a representative case of vancomy-
cin-induced LABD. In addition, the results of a comprehen-
sive literature review are reported to provide an overview
of putative culprit drugs and their frequency, in order to
enable clinicians to diagnose patients with putative adverse
drug reactions presenting with blistering.
LABD are idiopathic, an association with either drug
intake or systemic autoimmune diseases, such as rheu-
matoid arthritis, psoriasis, systemic lupus erythematodes,
Crohn’s disease and ulcerative colitis, has been reported,
the latter displaying the most frequent association with
LABD (10–15). Regarding the drug-induced form, drugs
such as antibiotics, analgesics, antihypertensives, antie-
pileptics or immunosuppressants have been identified as
potential causative agents (16). We report here a patient
with vancomycin-induced linear IgA dermatosis and
review the literature regarding drug-induced LABD by
searching the PubMed database using the key words ‘’li-
near immunoglobulin A disease’’, ‘’linear IgA disease’’,
‘’linear IgA bullous disease’’, ‘’linear IgA bullous der-
matosis’’, and ‘’LAD’’, ‘’LABD’’, both alone and in
association with the terms ‘’drug’’, ‘’drug-induced’’ and
‘’drug-associated’’.
CASE-REPORT
A 77-year-old man was referred to our department with
a 4-day history of bullous dermatosis. Previously he
underwent implantation of a hip joint endoprosthesis,
complicated by prosthetic joint infection. Due to micro-
biological detection of multidrug resistant Gram-negative
bacteria the patient was treated with vancomycin. Ap-
proximately 2 weeks after initiating intravenous van-
comycin, he developed a polymorphic, erythematous
vesiculobullous rash. Except for light itching at the
beginning, the patient reported no other symptoms. Vital
parameters were stable on presentation. Past medical his-
tory included coxarthrosis, coronary heart disease, atrial
fibrillation, spastic hemiparesis and hemiplegia after
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