an autoimmune blistering skin disorder: paraneoplastic pemphigus (pnp) in association with a breast...
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925 Profiles and Prognosis of Severe Infantile Atopic Dermatitiswith Food Allergy
M. Iguchi1, M. Ebisawa2, H. Tachimoto2; 1Jikei University school of
Medicine, Tokyo, JAPAN, 218-1 Sakuradai, Department of pediatrics,
National Organization Sagamihra National Hospital, Sagamihara city,
Kanagawa, JAPAN.
RATIONALE: The aim of this study was to clarify the profiles and prog-
nosis of severe infantile atopic dermatitis (AD) (n=67) with food allergy
(FA) against more than 2 food antigens.
METHODS: All subjects were admitted to the Sagamihara National Hos-
pital to control AD symptoms and to receive diagnosis of FA from 1997
to 2003. We analyzed their patients’ profiles and the prognosis of atopic
condition for 5 years.
RESULTS: 97% of their chronic eczema started by 4 months old. The
number of food antigens determined by food provocation was decreased
by 1.1 antigens (from 4.6 to 3.5) compared with the number of sensitized
food antigens proved by specific IgE or SPT. Hen’s egg was the most
common, followed by cow’s milk, wheat, and soybean. The number of
food antigens had become less and less, finally it reached 1.4 items at the
age of five. The release of egg elimination at the age of five was still only
30%. In addition the age when the release rate of cow’s milk elimination
reached 50% was at 5 y, however, those for wheat and soybean were at
2 y. Although most of the subjects used steroid ointment regularly at the
time of discharge, the percentage of “regular use” at 5 years old decreased
by 10%. Incidence of bronchial asthma from the subjects at the age of four
reached 53%.
CONCLUSIONS: Their prognosis was relatively fair except for the pro-
longed egg and cow’s milk FA and the high incidence of BA from the
subjects.
Funding: Ministry of Health, Labor, and Welfare
926 Randomized, Placebo-Controlled Trial of Lactobacillus rham-nosus GG as Treatment of Mild to Moderate Atopic Dermatitisin Infancy1 2 1 1 1 3
J ALLERGY CLIN IMMUNOL Abstracts S239VOLUME 117, NUMBER 2
923 An Autoimmune Blistering Skin Disorder: ParaneoplasticPemphigus (PNP) In Association With A Breast Neoplasm
P. Buddiga; Baz Allergy, Asthma & Sinus Center, Fresno, CA.
RATIONALE: Paraneoplastic pemphigus (PNP) is a potentially rare
complication usually associated with a neoplasm and is characterized by
intractable ulcerative stomatitis, mucosal surface erosions and polymor-
phic cutaneous eruptions.
METHODS/CASE: A 50-year-old African-American female with inva-
sive breast carcinoma and supraclavicular lymph node metastasis was
treated with a chemotherapeutic regimen of Cyclophosphamide and Dox-
orubicin. During her multiple cycle course she developed severe oral
mucositis leading to the discontinuation of chemotherapy. The oral lesions
progressed and she developed a diffuse polymorphic cutaneous eruption
represented by erythematous papules, bullae and erosions that responded
to oral corticosteroid treatment with Prednisone 60 mg/day for 2 weeks
and tapered down to 20 mg everyday. Oral mucosal and skin lesion biop-
sies were performed and interpreted as consistent with PNP.
RESULTS: Surgical PathologySkin and Anterior Palate Biopsy-Direct ImmunofluorescenceIgG - 4+ linear intraepidermal intercellular deposit, complete epidermis
IgA, IgM, C1q - negative
C3D - 4+ linear epidermal basement membrane deposit, 3+ linear
intraepidermal intercellular deposit , bottom one-fourth of epidermis.
Breast biopsy- Invasive mammary carcinoma, poorly differentiated with
focal mucinous features.
CONCLUSIONS: Despite initial short-term success of aggressive
immunosuppressant therapy for controlling the polymorphic cutaneous
eruptions of PNP, the relative poor prognosis and high mortality rate in
those with malignant tumors (10 % - 2 year survival) suggests failure of
inducing a remission, despite persistent use of various immunomodulat-
ing agents. This demonstrates the need for further studies to elucidate the
disease pathogenesis by which neoplasia induces autoimmunity so that
this may facilitate the arrival of an immunotherapeutic efficacious inter-
vention.
924 Induction of a Pathergy-Like Reaction Following Prick SkinTesting
T. L. Johnson, II1, P. McCleskey2, M. Rathkopf1, J. Meffert2, L. L.
Hagan1; 1Allergy/Immunology, Wilford Hall Medical Center, Lack
land AFB, TX, 2Dermatology, Wilford Hall Medical Center, Lackland
AFB, TX.
RATIONALE: A 46-year-old female who received 6 months of
immunotherapy for perennial and seasonal allergic rhinitis 5 years prior to
presentation returned for consideration of restarting immunotherapy sec-
ondary to uncontrolled rhinoconjunctivitis. The patient had no other
chronic illnesses and lacked symptoms suggestive for Behcet’s disease.
Past history was negative for local or systemic reactions to previous skin
testing and immunotherapy injections.
METHODS: Prick skin testing was performed utilizing the Quintest®
device to an aeroallergen panel consisting of 53 antigens to assess for the
development of new sensitizations.
RESULTS: The patient had positive results to multiple trees, weeds,
molds, grasses, dust mite and cat. Five days after the testing procedure the
patient called and stated that she developed pustules and pruritus at many
of the skin test sites. The pustules were first noted on the day following
testing. Upon examination the patient had round erythematous patches
with central hyperpigmentation along with erosions and crusts. Lesions
were present at 37 of 40 sites that had a 1+ or greater reaction (including
histamine control). Out of 15 test sites (including negative control) that
lacked reactivity at the time of initial reading; 2 had the development of
the above lesions. Histopathologic examination revealed confluent
epidermal necrosis, extensive interface vacuolar changes, and focal
subepidermal vesicle formation. Dermal changes included a perivascular
lymphocytic inflammatory infiltrate with endothelial swelling and few
eosinophils or neutrophils.
CONCLUSIONS: This report may represent a previously uncharacter-
ized pathergy-like reaction induced by skin testing in a patient that to date
lacks symptoms of known pathergy associated illnesses.
C. Grüber , M. Wendt , S. Lau , M. Kulig , U. Wahn , T. Werfel , B.
Niggemann1; 1Dept. of Pediatric Pneumology/Immunology, Charité Uni-
versitätsmedizin Berlin, Berlin, GERMANY, 2Department of Dermatology
and Allergology, Hanover Medical University, Hanover, GERMANY, 3(2)
Department of Dermatology and Allergology, Hanover Medical University,
Hanover, GERMANY.
RATIONALE: The increase of atopic dermatitis prevalence has been
associated with a decrease of “healthy” microbials such as lactobacilli in
the intestinal flora. Some studies suggested that supplementation of food
with lactobacilli may improve atopic dermatitis in children. This study
was designed to investigate the therapeutic effect of Lactobacillus rham-nosus GG (LGG) in infants suffering from atopic dermatitis.
METHODS: Infants aged 3-12 months suffering from mild to moderate
atopic dermatitis (SCORAD index of 15-40) without current antiinflam-
matory treatment were randomised to receive LGG (5x109 CFU BID) or
placebo as a food supplement for 12 weeks. SCORAD index and use of
hydrocortisone 1% ointment as rescue medication (2 points per applica-
tion) were recorded at 4, 8, and 12 weeks of treatment and combined as
symptom load (SL).
RESULTS: 54 infants (LGG group, mean± SD SCORAD index 24.6±
8.8) and 48 infants (placebo group, SCORAD index 23.6± 7.8) were ran-
domised and completed the treatment period (intention to treat analysis).
SL generally improved over time at 4 weeks (LGG vs placebo, 23.8± 12.4
vs 20.6± 9.9), 8 weeks (22.5± 14.6 vs 17.9± 13.1), and 12 weeks (19.6±
15.4 vs 15.1± 12.1), without statistical significant group differences. No
significant group differences were found for use of rescue medication
(0.8g± 45.0g vs 3.5g ± 29.8g), increase in median total serum IgE (0.17±
0.30kU/L vs 0.26± 0.45kU/L), and new developed allergic sensitization
against hen’s egg or cow’s milk (18.8% vs 10.0%).
CONCLUSIONS: This randomised controlled trial showed no therapeutic
effect of LGG on mild to moderate atopic dermatitis in infancy.
Funding: Infectopharm Arzneimittel, Heppenheim, Germany
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