an autoimmune blistering skin disorder: paraneoplastic pemphigus (pnp) in association with a breast...

1
923 An Autoimmune Blistering Skin Disorder: Paraneoplastic Pemphigus (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 Pathology Skin and Anterior Palate Biopsy-Dir ect Immunofluor escence IgG - 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 Skin Testing T. L. Johnson, II 1 , P. McCleskey 2 , M. Rathkopf 1 , J. Meffert 2 , L. L. Hagan 1 ; 1 Allergy/Immunology, Wilford Hall Medical Center, Lack land AFB, TX, 2 Dermatology, 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. 925 Profiles and Prognosis of Severe Infantile Atopic Dermatitis with Food Allergy M. Iguchi 1 , M. Ebisawa 2 , H. Tachimoto 2 ; 1 Jikei University school of Medicine, Tokyo, JAPAN, 2 18-1 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 Dermatitis in Infancy C. Grüber 1 , M. Wendt 2 , S. Lau 1 , M. Kulig 1 , U. Wahn 1 , T. Werfel 3 , B. Niggemann 1 ; 1 Dept. of Pediatric Pneumology/Immunology, Charité Uni- versitätsmedizin Berlin, Berlin, GERMANY, 2 Department 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 (5x10 9 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 J ALLERGY CLIN IMMUNOL Abstracts S239 VOLUME 117, NUMBER 2 MONDAY

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Page 1: An Autoimmune Blistering Skin Disorder: Paraneoplastic Pemphigus (PNP) In Association With A Breast Neoplasm

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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