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Concurrent Linear Immunoglobulin A Dermatosis, Hashimoto Thyroiditis, and Immunoglobulin A Nephropathy in an Adult

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Brief Report
226 Ann Dermatol
Received November 19, 2015, Revised February 3, 2016, Accepted for publication April 4, 2016
*Current affiliation: Sue Kyung Kim, Department of Dermatology, Seoul Medical Center, 156 Sinnae-ro, Jungnang-gu, Seoul 02053, Korea. Tel:
82-2-2276-7891, Fax: 82-2-2276-7438, E-mail: skkim@seoulmc.or.kr
Corresponding author: Sue Kyung Kim, Department of Dermatology, Ajou University School of Medicine, 164 WorldCup-ro, Yeongtong-gu, Suwon 16499,
Korea. Tel: 82-31-219-5190, Fax: 82-31-219-5189, E-mail: ksk9167@ajou.ac.kr
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Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology
pISSN 1013-9087eISSN 2005-3894
Ann Dermatol Vol. 29, No. 2, 2017 https://doi.org/10.5021/ad.2017.29.2.226
BRIEF REPORT
Fig. 1. (A) A 45-year-old woman
presented with multiple erythema-
tous collarettes of blisters on her
whole body, mainly on the trunk.
Note the clustered bullae on the
back. (B) New bullae are found
adjacent to old bullae, forming a
string of beads sign.
Concurrent Linear Immunoglobulin A Dermatosis,
Hashimoto Thyroiditis, and Immunoglobulin A
Nephropathy in an Adult
Ji Young Yang, Inwhee Park1, Sue Kyung Kim*
Departments of Dermatology and 1Nephrology, Ajou University School of Medicine, Suwon, Korea
Dear Editor:
A 45-year-old woman presented with a 5-week history of
vesicular eruption over her body. Four years prior, she
was diagnosed with Hashimoto thyroiditis with anti-thyro-
globulin antibody level, 339 U/ml (reference range, 0
100 U/ml) and anti-microsomal antibody level, 1,296
U/ml (reference range, 0100 U/ml). She had been taking
medication for 3 years, discontinuing it on her own. In ad-
dition, she had been taking telmisartan 80 mg/day, amlo-
dipine 5 mg/day, and hydrochlorothiazide 12.5 mg/day
Brief Report
Vol. 29, No. 2, 2017 227
Fig. 2. (A, B) Subepidermal bulla with mixed inflammatory infiltration in the upper dermis (H&E, virtual slide view). (C) Linear deposition
of immunoglobulin (Ig) A along the dermoepidermal junction (direct immunofluorescence [DIF], ×200). (D) Mild widening of the
mesangial matrix with focal and segmental mesangial hypercellularity on renal biopsy (periodic acid-Schiff stain, virtual slide view).
The renal DIF study result showed mild IgA, C3, and minimal IgM deposits at the mesangium (not shown).
for 4 months to control hypertension. Physical examina-
tion revealed multiple erythematous collarettes of blisters
with intense pruritus on her whole body (Fig. 1). Skin bi-
opsy with direct immunofluorescence (DIF) study was per-
formed on her back. Hematoxylin-eosin staining revealed
a subepidermal bulla with mixed inflammatory infiltration
in the upper dermis (Fig. 2A, B). The DIF study revealed
linear deposition of immunoglobulin (Ig) A along the der-
moepidermal junction (Fig. 2C), resulting in the diagnosis
of linear IgA dermatosis (LAD). Laboratory test results
were otherwise normal except for the following: white
blood cell count, 15,000/μl with 85.6% neutrophil con-
centration; hemoglobin level, 10.8 g/dl; blood urea nitro-
gen level, 30.6 mg/dl; creatinine level, 1.44 mg/dl; urinary
protein level, 55.9 mg/dl; urinary creatinine level, 96.9
mg/dl; urinary protein-to-creatinine ratio, 0.58; and uri-
nary red blood cell count, many per high power field. On
referral to the department of nephrology for evaluation,
she was diagnosed with IgA nephropathy by kidney biop-
sy (Fig. 2D). Moreover, further evalution revealed normo-
cytic normochromic anemia with elevated serum ferritin
level, implying anemia of chronic inflammation. During
12-month follow-up, the cutaneous lesions had been fairly
well controlled with dapsone 50100 mg/day, with or
without colchicine 1.2 mg/day.
LAD is an acquired, autoimmune vesiculobullous derma-
tosis characterized by subepidermal blisters with deposi-
tion of linear homogeneous IgA at the basement membrane.
Its pathogenesis is unclear, but associations with malig-
nancies, drugs, and inflammatory diseases, notably ulcer-
Brief Report
228 Ann Dermatol
ative colitis, have been reported in adults1. Several cases
of LAD with IgA nephropathy have been reported in chil-
dren1, but a few in adults2.
Hashimoto thyroiditis is an autoimmune thyroiditis dem-
onstrating high titers of thyroid antibodies. It is associated
with other autoimmune diseases such as Addison disease,
type 1 diabetes mellitus, vitiligo, rheumatoid arthritis, or
systemic lupus erythematosus. To the best of our knowl-
edge, only one case associated with LAD has been re-
ported3. As they share the autoimmune pathogenesis, reg-
ulatory T cells might play a role in LAD and Hashimoto
thyroiditis4.
IgA nephropathy is an immune complex-mediated glomer-
ulonephritis characterized by diffuse mesangial IgA depos-
it, sometimes with IgM, IgG, complement 3, or Ig light
chains. IgA in the mesangium is typically of the polymeric
IgA1 subclass. Pena-Penabad et al.2 suggested a possible
role of the IgA1 subclass in the shared pathogenesis be-
tween LAD and IgA nephropathy. Furthermore, in a ge-
nome-wide association study of IgA nephropathy, six new
genome-wide significant associations were found, most of
which were associated with the risk of inflammatory bow-
el disease5. These loci could be related to LAD, as the as-
sociation between LAD and ulcerative colitis is well
documented.
In conclusion, we report a rare case of concurrent LAD,
Hashimoto thyroiditis, and IgA nephropathy.
CONFLICTS OF INTEREST
The authors have nothing to disclose.
REFERENCES
1. Rositto AE, Cobeñas C, Drut R. Linear IgA disease of
childhood developing IgA nephropathy. Pediatr Dermatol
2008;25:339-340.
2. Pena-Penabad C, Hernández-Vicente I, Hernández-Martín A,
García-Silva J, Flores T, Armijo M. IgA mesangial nephropathy
and autoimmune haemolytic anaemia associated with linear
IgA bullous dermatosis. Br J Dermatol 1995;133:146-148.
3. Horiguchi Y, Ikoma A, Sakai R, Masatsugu A, Ohta M,
Hashimoto T. Linear IgA dermatosis: report of an infantile
case and analysis of 213 cases in Japan. J Dermatol 2008;
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4. Pyzik A, Grywalska E, Matyjaszek-Matuszek B, Roliński J.
Immune disorders in Hashimoto's thyroiditis: what do we
know so far? J Immunol Res 2015;2015:979167.
5. Kiryluk K, Li Y, Scolari F, Sanna-Cherchi S, Choi M,
Verbitsky M, et al. Discovery of new risk loci for IgA
nephropathy implicates genes involved in immunity against
intestinal pathogens. Nat Genet 2014;46:1187-1196.
... About 5% of LAD patients shows concomitant malignancies, with Hodgkin disease, non-Hodgkin's lymphoma and chronic lymphocytic leukemia being the more common [42]. In two case reports LAD was also found to arise together with more than one pathology, among which Hashimoto thyroiditis [60,61]. The significance of such association remains, however, uncertain. ...
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