1
433 A Case Of Incontinentia Pigmenti Masquerading As Urticaria Pigmentosa Dr. Erin C. Donahue, MD, Dr. Sonal R. Patel, MD; White Memorial Medical Center, Los Angeles, CA. RATIONALE: Incontinentia Pigmentosi (IP) is an X-linked dominant ectodermal dysplasia often associated with opthalmological and neuro- logical complications. Histopathology depends on the stage of skin lesions but mastocytosis is not a recognized feature. This child was referred to us with a diagnosis of Urticaria Pigmentosa (UP) based on the presence of itchy pigmented rash and mastocytes on biopsy. Here we present a patient with skin findings typical of IP, and potentially atypical finding of mast cells on biopsy, misdiagnosed with UP. METHODS: Punch biopsy of lesion, CD117 and Giemsa stain of tissue sample performed by Quest Diagnostics. RESULTS: This 14 m/o patient with pruritic hyperpigmented whorled rash following the lines of Blaschko was worked up for mastocytosis. Raised hyperpigmented plaques were present at birth, most of which are now flat. Workup for systemic mastocytosis including C-kit mutation analysis, histamine release and tryptase were normal. Plasma Histamine 2.4 ng/ml (reference range 0.1-1.8). Increased mast cells, verrucous hyperplasia with dyskeratotic cells and pigment incontinence on skin biopsy. CONCLUSIONS: Only one previous report from 1967 could be found of mast cells in IP. History and physical exam are classic for IP in this patient, yet she was worked up as UP due to the presence of pruritic hyperpig- mentation and diagnosis was thought to be confirmed by mast cells on biopsy. This case highlights the need for increased awareness of IP and its features given the importance of ophthalmologic evaluation and genetic counseling, and suggests the need for further study regarding mastocytosis as a histological feature of IP. 434 Acute Urticaria Caused By Infection In 72 Chinese Patients Prof. Ting Xiao 1 , Dr. Lin Zhu 2 ; 1 The First Affiliated Hospital, China Medical University, Shenyang, China, 2 The First Affiliated Hospi- tal, China Medical University. RATIONALE: Infection, food and drugs are the most common causes of acute urticaria. To date, the clinical, etiological and prognostic features and the optimal treatment of acute urticaria caused by infection are poorly defined. METHODS: This retrospective study included 72 inpatients diagnosed acute urticaria caused by infection from 2008 to 2012 at the First Affiliated Hospital of China Medical University. The patients’ general information, etiology, clinical manifestations, laboratory examinations, treatment and prognosis were analyzed. RESULTS: Of 204 inpatients with acute urticaria, 72 cases were caused by infections (35.3%), 38 cases caused by food (18.6%), 26 by drugs (12.7%), 16 by other causes (7.8%), and 52 with no obvious causes (25.5%). The infections included 30 upper respiratory infection (bacterial or viral, 41.7%), 12 gastroenteritis (16.7%), 7 mycoplasma pneumonia (9.7%), 4 wound infection (5.6%). WBC and neutrophil counts, erythrocyte sedimentation rate, serum C-reactive protein and procalcitonin were significantly elevated in the patients with acute urticaria caused by infection. Systemic corticosteroid, antibiotic and antihistamine were administered in 39 cases (54.2%), systemic antibiotic and antihistamine in 19 (26.4%), systemic corticosteroid and antihistamine in 14 cases (19.4%). Of the 72 patients, 65% of the patients healed in 1 week, 80.4% cured in 2 weeks, 91.2% cleared in 3 weeks, and 100% resolved in 6 weeks. CONCLUSIONS: Infections accounted for a major cause of acute urticaria. WBC, neutrophil count, ESR, CRP, and procalcitonin were useful for monitoring disease activity. Systemic corticosteroid, antibiotic and antihistamine were the cornstone treatment. The prognosis of acute urticaria caused by infection was good. 435 Assessment Of Acute Urticaria In Pediatric Emergency Department Dr. Raquel Reis Pitchon, Clinical Research 1 , Mr. Daniel Reis, Medical Student 2 , Ms. Adriana Reis, Medical Student 3 , Mr. Andr e Chuster, Med- ical Student 3 , Prof. Teresa Mohallen, Professor 2 , Dr. Jos e Ribeiro, Pedia- trician 4 , Dr. N ıvea Claret, Pediatrician 4 , Dr. Giane Chaves, Pediatrician 4 , Dr. Virgilio Aleixo, Pediatrician 4 , Dr. Maria do Socorro Fernandes, Pedi- atrician 4 , Dr. Fausto Pacheco, Pediatrician 4 ; 1 Mater Dei Hospital, BELO HORIZONTE, Brazil, 2 Universidade Federal de Minas Gerais, BELO HORIZONTE, Brazil, 3 Faculdade de Ciencias M edicas, BELO HORI- ZONTE, Brazil, 4 Hospital Mater Dei, BELO HORIZONTE, Brazil. RATIONALE: Assessment of patients admitted with acute urticaria in the Pediatric Emergency Department(PED). METHODS: Uncontrolled study of 565 children and adolescents aged from 0 to 15 years, from January 2012 to December 2012, with acute urticaria (ICD L50),treated in the PED. RESULTS: In this period were performed a total of 64 203 visits due to several causes, and 565 (0.88%) were admitted with acute urticaria. The incidence was higher in males (56%) and in children aged between 1 and 3 years old, which corresponded to 45.5% of the total of cases diagnosed with acute urticaria. Hospital medication was prescribed to 33.27% of the patients. Sixty one cases(10.79%) reported associated diseases, and the most frequent were infectious diseases in 45 cases (75%). The associated pathologies were otitis, acute rhinofaryngitis , acute sinusitis, influenza and acute tonsillitis (see Table I). In a period of less than 72 hours after the hospital discharge, 19 patients (3.36%) were readmitted . Five patients were hospitalized, one case associated with bacterial pneumonia and another with cellulitis. CONCLUSIONS: Performance measurement of care quality allows the comparison of data between institutions and the creation of incentives for improving care quality standards. After the results, the staff did a protocol for the attendance of acute urticaria and a discharge standard prescription for the patient and his family. 436 OTC Pills and Severe Urticaria Ivan Cherrez 1 , Enrique Loayza 2 , Leonardo Greiding 3 , Jose M. Vilema 4 , Juan Calderon 4 , Erick Calero 4 , Gabriela Martinetti 4 ; 1 Respiralab - Hospital Kennedy, Guayaquil, Ecuador, 2 Respiralab - Hospital Luis Ver- naza, Guayaquil, Ecuador, 3 Instituto Argentino de Alergia e Inmunologia, Buenos Aires, Argentina, 4 Respiralab, Guayaquil, Ecuador. RATIONALE: A lot of weight loss pill are available over the counter (OTC) and online. Most haven’t been proved effective and may contain dangerous substances, causing life-threatening effect. Urticarial Vasculitis (UV) has been reported in association with other drugs but no with OTC diet pills. METHODS: We describe three patients without antecedents with 2-5 days of generalized urticarial with mild constitutional symptoms. Physical examination revealed hives on head, hands, abdomen, groin, all skinfolds and thighs. They were pruriginous, painful and burning, > _0.5 cm with an urticarial severity score of 4-6 points. Lesions lasted < _24 hours, leaving residual pigmentation. They had lymphopenia. The C protein was high, but complement, liver function test, urea and creatinine, plasma sodium, glucose, and thyroid function were in normal ranges. ANA and anti-Tg antibodies were not detected in two of them. They were taking OTC diet pills approximately 15 days ago. The ingredients were: synefrin, guggulsterones, thyroid stimulating matrix, yohimbine and phenylmine. These different ingredients have reported side effect separately but no vasculitis. Skin biopsies, confirmed the diagnosis of Urticarial Vasculitis. RESULTS: Diagnosis of diet pills induced UV was based on the temporal relationship between intake pills and wheals lesion, systemic manifesta- tion, histologically proven vasculitis and finally by reversal of the clinical signs after discontinuation of the therapy. CONCLUSIONS: We believe these are the first reported cases of UV with the use of OTC diet pills. Given the widespread use of these pills, allergist and dermatologist should be able to recognize UV which may be under reported. J ALLERGY CLIN IMMUNOL VOLUME 133, NUMBER 2 Abstracts AB123 SUNDAY

A Case Of Incontinentia Pigmenti Masquerading As Urticaria Pigmentosa

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J ALLERGY CLIN IMMUNOL

VOLUME 133, NUMBER 2

Abstracts AB123

SUNDAY

433 A Case Of Incontinentia Pigmenti Masquerading As UrticariaPigmentosa

Dr. Erin C. Donahue, MD, Dr. Sonal R. Patel, MD; White Memorial

Medical Center, Los Angeles, CA.

RATIONALE: Incontinentia Pigmentosi (IP) is an X-linked dominant

ectodermal dysplasia often associated with opthalmological and neuro-

logical complications. Histopathology depends on the stage of skin lesions

but mastocytosis is not a recognized feature. This child was referred to us

with a diagnosis of Urticaria Pigmentosa (UP) based on the presence of

itchy pigmented rash and mastocytes on biopsy. Here we present a patient

with skin findings typical of IP, and potentially atypical finding of mast

cells on biopsy, misdiagnosed with UP.

METHODS: Punch biopsy of lesion, CD117 and Giemsa stain of tissue

sample performed by Quest Diagnostics.

RESULTS: This 14m/o patient with pruritic hyperpigmented whorled rash

following the lines of Blaschko was worked up for mastocytosis. Raised

hyperpigmented plaques were present at birth, most of which are now flat.

Workup for systemic mastocytosis including C-kit mutation analysis,

histamine release and tryptase were normal. Plasma Histamine 2.4 ng/ml

(reference range 0.1-1.8). Increased mast cells, verrucous hyperplasia with

dyskeratotic cells and pigment incontinence on skin biopsy.

CONCLUSIONS: Only one previous report from 1967 could be found of

mast cells in IP. History and physical exam are classic for IP in this patient,

yet she was worked up as UP due to the presence of pruritic hyperpig-

mentation and diagnosis was thought to be confirmed by mast cells on

biopsy. This case highlights the need for increased awareness of IP and its

features given the importance of ophthalmologic evaluation and genetic

counseling, and suggests the need for further study regarding mastocytosis

as a histological feature of IP.

434 Acute Urticaria Caused By Infection In 72 Chinese PatientsProf. Ting Xiao1, Dr. Lin Zhu2; 1The First Affiliated Hospital,

China Medical University, Shenyang, China, 2The First Affiliated Hospi-

tal, China Medical University.

RATIONALE: Infection, food and drugs are the most common causes of

acute urticaria. To date, the clinical, etiological and prognostic features and

the optimal treatment of acute urticaria caused by infection are poorly

defined.

METHODS: This retrospective study included 72 inpatients diagnosed

acute urticaria caused by infection from 2008 to 2012 at the First Affiliated

Hospital of China Medical University. The patients’ general information,

etiology, clinical manifestations, laboratory examinations, treatment and

prognosis were analyzed.

RESULTS: Of 204 inpatients with acute urticaria, 72 cases were caused by

infections (35.3%), 38 cases caused by food (18.6%), 26 by drugs (12.7%),

16 by other causes (7.8%), and 52 with no obvious causes (25.5%). The

infections included 30 upper respiratory infection (bacterial or viral,

41.7%), 12 gastroenteritis (16.7%), 7 mycoplasma pneumonia (9.7%), 4

wound infection (5.6%). WBC and neutrophil counts, erythrocyte

sedimentation rate, serum C-reactive protein and procalcitonin were

significantly elevated in the patients with acute urticaria caused by

infection. Systemic corticosteroid, antibiotic and antihistamine were

administered in 39 cases (54.2%), systemic antibiotic and antihistamine

in 19 (26.4%), systemic corticosteroid and antihistamine in 14 cases

(19.4%). Of the 72 patients, 65% of the patients healed in 1 week, 80.4%

cured in 2 weeks, 91.2% cleared in 3 weeks, and 100% resolved in 6weeks.

CONCLUSIONS: Infections accounted for a major cause of acute

urticaria. WBC, neutrophil count, ESR, CRP, and procalcitonin were

useful for monitoring disease activity. Systemic corticosteroid, antibiotic

and antihistamine were the cornstone treatment. The prognosis of acute

urticaria caused by infection was good.

435 Assessment Of Acute Urticaria In Pediatric EmergencyDepartment

Dr. Raquel Reis Pitchon, Clinical Research1, Mr. Daniel Reis, Medical

Student2, Ms. Adriana Reis, Medical Student3, Mr. Andr�e Chuster, Med-

ical Student3, Prof. Teresa Mohallen, Professor2, Dr. Jos�e Ribeiro, Pedia-trician4, Dr. N�ıvea Claret, Pediatrician4, Dr. Giane Chaves, Pediatrician4,

Dr. Virgilio Aleixo, Pediatrician4, Dr. Maria do Socorro Fernandes, Pedi-

atrician4, Dr. Fausto Pacheco, Pediatrician4; 1Mater Dei Hospital, BELO

HORIZONTE, Brazil, 2Universidade Federal de Minas Gerais, BELO

HORIZONTE, Brazil, 3Faculdade de Ciencias M�edicas, BELO HORI-

ZONTE, Brazil, 4Hospital Mater Dei, BELO HORIZONTE, Brazil.

RATIONALE: Assessment of patients admitted with acute urticaria in the

Pediatric Emergency Department(PED).

METHODS: Uncontrolled study of 565 children and adolescents aged

from 0 to 15 years, from January 2012 to December 2012, with acute

urticaria (ICD L50),treated in the PED.

RESULTS: In this period were performed a total of 64 203 visits due to

several causes, and 565 (0.88%) were admitted with acute urticaria. The

incidence was higher in males (56%) and in children aged between 1 and 3

years old, which corresponded to 45.5% of the total of cases diagnosed

with acute urticaria. Hospital medication was prescribed to 33.27% of the

patients. Sixty one cases(10.79%) reported associated diseases, and the

most frequent were infectious diseases in 45 cases (75%). The associated

pathologieswere otitis, acute rhinofaryngitis , acute sinusitis, influenza and

acute tonsillitis (see Table I). In a period of less than 72 hours after the

hospital discharge, 19 patients (3.36%) were readmitted . Five patients

were hospitalized, one case associated with bacterial pneumonia and

another with cellulitis.

CONCLUSIONS: Performance measurement of care quality allows the

comparison of data between institutions and the creation of incentives for

improving care quality standards. After the results, the staff did a protocol

for the attendance of acute urticaria and a discharge standard prescription

for the patient and his family.

436 OTC Pills and Severe UrticariaIvan Cherrez1, Enrique Loayza2, Leonardo Greiding3, Jose M.

Vilema4, Juan Calderon4, Erick Calero4, Gabriela Martinetti4; 1Respiralab

- Hospital Kennedy, Guayaquil, Ecuador, 2Respiralab - Hospital Luis Ver-

naza, Guayaquil, Ecuador, 3Instituto Argentino de Alergia e Inmunologia,

Buenos Aires, Argentina, 4Respiralab, Guayaquil, Ecuador.

RATIONALE: A lot of weight loss pill are available over the counter

(OTC) and online. Most haven’t been proved effective and may contain

dangerous substances, causing life-threatening effect. Urticarial Vasculitis

(UV) has been reported in association with other drugs but no with OTC

diet pills.

METHODS: We describe three patients without antecedents with 2-5

days of generalized urticarial with mild constitutional symptoms. Physical

examination revealed hives on head, hands, abdomen, groin, all skinfolds

and thighs. They were pruriginous, painful and burning, >_0.5 cm with an

urticarial severity score of 4-6 points. Lesions lasted <_24 hours, leaving

residual pigmentation. They had lymphopenia. The C protein was high, but

complement, liver function test, urea and creatinine, plasma sodium,

glucose, and thyroid function were in normal ranges. ANA and anti-Tg

antibodies were not detected in two of them. They were taking OTC diet

pills approximately 15 days ago. The ingredients were: synefrin,

guggulsterones, thyroid stimulating matrix, yohimbine and phenylmine.

These different ingredients have reported side effect separately but no

vasculitis. Skin biopsies, confirmed the diagnosis of Urticarial Vasculitis.

RESULTS: Diagnosis of diet pills induced UV was based on the temporal

relationship between intake pills and wheals lesion, systemic manifesta-

tion, histologically proven vasculitis and finally by reversal of the clinical

signs after discontinuation of the therapy.

CONCLUSIONS:Webelieve these are the first reported cases of UVwith

the use of OTC diet pills. Given the widespread use of these pills, allergist

and dermatologist should be able to recognize UV which may be under

reported.