- Email: [email protected]
The use of Danazol in the Management of Refractory Chronic Urticaria
Abstracts S205
J ALLERGY CLIN IMMUNOL VOLUME 119, NUMBER 1
Tamoxifen, A Possible Novel Therapy For Systemic Mastocytosis R. Mian, P. E. Korenblat; Washington University School of Medicine, Barnes Jewish Hospital, St louis, MO. A 45 year old Caucasian female presented to the outpatient clinic with diffuse reddish-brown macular rash on her arms, legs, and chest with associated post-prandial cramping with diarrhea, hip pain and episodic hypotension. She had two ER visits for these symptoms. Physical exam was otherwise unremarkable. She had tried several different drug regimens including ranitidine, loratidine and cetirizine, with only intermittent prednisone bringing some relief. NSAIDs made her rash worse. Lab investigations for CBC, CMP, ESR, and DEXA Scan for hip and spine were all within normal range. UniCAP total tryptase was 65 nanogram per ml and mature tryptase was 1 nanogram per ml. Skin biopsy revealed abundant mast cells and small bowel mucosal biopsy revealed mast cells with CD117 expression. Patient was started on doxepin, cetirizine, fexofenadine, ranitidine, montelukast and oral cromolyn with partial relief to her symptoms. During her treatment she was diagnosed with stage 1A left breast intraductal carcinoma that was hormone receptor positive. She underwent lumpectomy and adjuvant chemo and radiation therapy. She was then prescribed Tamoxifen after which her difficult to treat symptoms of systemic mastocytosis improved. Tamoxifen is a nonsteroidal triphenylethylene antagonist of estrogen receptors. It is found to inhibit human mast cell proliferation possibly through ‘‘ion channel modulation.’’ Our patient has been taking Tamoxifen 20 mg per day for the past three years and her rash has improved significantly. This unique case highlights Tamoxifen as a possible novel treatment for systemic mastocytosis. This observation should be investigated further in a prospective clinical trial.
806
Urticaria: Characterization of 375 cases M. D. C. Petrola1, P. Adriana1, F. Maria1, T. Alcira2, Q. Velmar2; 1University of Carabobo, Valencia, VENEZUELA, 2Ciudad Hospitalaria Enrique Tejera, Valencia, VENEZUELA. RATIONALE: Descriptive study, series of cases. objective: characterization of urticarias. METHODS: Revision of medical histories (two authors, same method and approaches): Urticaria and/or Angioedema - unit of immunology (1996/ 06-2006). Classification: 2 groups: I) clinical presentation: a) Urticaria, b) Urticaria - Angioedema, c) Angioedema. II) time-appearance: a) acute recurrent urticaria (ARU): hives and/or edemas associated to unchain factors, repetitively. b) Chronic Urticaria (CU). more than 6 weeks periodically. RESULTS: 375 patients, 71.9 female%; under 12 years: equal prevalence in bouth sexes. Urticaria 1 angioedema: 63%, without difference between sexes. High serum IgE: 61%: without relationship with sex, age, characteristic, time and appearance; 51.42%: intestinal parasitosis. 13.85% (23) with higher IgE levels over 1000 U.I: 69.6% over 22 years old, 65.4%: ARU, 61%: urticaria-angioedema. 25.58%: allergy to drugs, 67.52% NSAIDs. CU: 34.3% (124), 71.3%: female; 29 (23.38%): well-known causes 44.8%: physical, 37.9% autoantibodies, 17.2%: deficit C1q inhibitor, 3.4%: low complement, 3.4%: HIV. 7.54% of the total group: ANA 1 5 92.9 feminine%, 71.2%: UrticariaAngioedema, 21.51%: ARU. 98% ARU and 99% CU: without collagen vascular illnesses. Positive dermografism: 6.9% total group, ARU prevail. 76.3%: foods as unchain factor, 80.8% with skin tests (ST) positive to foods. 45.9% ST positive to house dust, without concomitant allergic illness. Funding: Consejo de Desarrollo Cientı´fico y Humanı´stico U.C.
807
The use of Danazol in the Management of Refractory Chronic Urticaria M. Karakelides, C. R. Weiler; Mayo School of Graduate Medical Education, Rochester, MN. RATIONALE: Stanozolol has been shown to be an effective and safe adjuvant therapy for the treatment of refractory chronic urticaria in one study. There is only a single case report in the literature reporting the use of danazol in a patient with chronic urticaria. METHODS: The Mayo Clinic electronic database was reviewed to identify patients with the diagnosis of CU who were treated with danazol. The patients’ prior therapies, response to treatment and medication side effects were recorded and analyzed. RESULTS: Three patients were treated with danazol for refractory chronic urticaria. Two patients had resolution of their symptoms within two weeks of initiating therapy. The third patient did not have any symptomatic improvement after two weeks of treatment. This patient also started having episodes of dizziness after initiating treatment. CONCLUSIONS: Danazol may be of therapeutic benefit in the management of chronic urticaria refractory to mainstay therapy.
808
A Case of Urticarial Vasculitis Associated with Chronic Lymphocytic Leukemia J. C. Moore, M. B. Fasano; University of Iowa Hospitals and Clinics, Iowa City, IA. RATIONALE: Although cutaneous lesions are frequently seen in patients with hematological malignancies, cutaneous vasculitis is rarely seen in chronic lymphocytic leukemia (CLL). Here we report a case of CLL presenting as urticarial vasculitis. METHODS: Case report. RESULTS: An 83 year old male with long-standing thrombocytopenia presented to our clinic with a four month history of rash and intermittent lip edema. The rash was described as generalized, pruritic, raised, erythematous, persistent lesions with residual hyperpigmentation. Previous treatment with oral corticosteroids and antihistamines was not effective, nor did the rash resolve after stopping aspirin and hydrochlorothiazide. Skin biopsy showed superficial perivascular mixed inflammation, consistent with urticarial vasculitis. Laboratory tests revealed a normal white blood cell count (WBC 5 5.5 3 109/l, lymphocytes 5 3.1 3 109/l), a normal hemoglobin, and thrombocytopenia (platelet count 5 114 3 109/ml). Complement studies were normal. Serum immunofixation showed an IgG-kappa monoclonal gammopathy. Flow cytometry on peripheral blood demonstrated a monoclonal B-lymphoid population, kappa immunophenotype, with CD20/CD5 co-expression, bright CD23 and 35% CD38 expression, consistent with CLL. CONCLUSIONS: We report a rare cutaneous manifestation of CLL, urticarial vasculitis. In this case, cutaneous lesions resulted in medical referral and evaluation, leading to the diagnosis of CLL. This case highlights the importance of prompt diagnosis of cutaneous vasculitis and the need for additional evaluation to exclude associated systemic conditions such as malignancy. Funding: University of Iowa Hospitals and Clinics
MONDAY
805
J ALLERGY CLIN IMMUNOL VOLUME 119, NUMBER 1
Tamoxifen, A Possible Novel Therapy For Systemic Mastocytosis R. Mian, P. E. Korenblat; Washington University School of Medicine, Barnes Jewish Hospital, St louis, MO. A 45 year old Caucasian female presented to the outpatient clinic with diffuse reddish-brown macular rash on her arms, legs, and chest with associated post-prandial cramping with diarrhea, hip pain and episodic hypotension. She had two ER visits for these symptoms. Physical exam was otherwise unremarkable. She had tried several different drug regimens including ranitidine, loratidine and cetirizine, with only intermittent prednisone bringing some relief. NSAIDs made her rash worse. Lab investigations for CBC, CMP, ESR, and DEXA Scan for hip and spine were all within normal range. UniCAP total tryptase was 65 nanogram per ml and mature tryptase was 1 nanogram per ml. Skin biopsy revealed abundant mast cells and small bowel mucosal biopsy revealed mast cells with CD117 expression. Patient was started on doxepin, cetirizine, fexofenadine, ranitidine, montelukast and oral cromolyn with partial relief to her symptoms. During her treatment she was diagnosed with stage 1A left breast intraductal carcinoma that was hormone receptor positive. She underwent lumpectomy and adjuvant chemo and radiation therapy. She was then prescribed Tamoxifen after which her difficult to treat symptoms of systemic mastocytosis improved. Tamoxifen is a nonsteroidal triphenylethylene antagonist of estrogen receptors. It is found to inhibit human mast cell proliferation possibly through ‘‘ion channel modulation.’’ Our patient has been taking Tamoxifen 20 mg per day for the past three years and her rash has improved significantly. This unique case highlights Tamoxifen as a possible novel treatment for systemic mastocytosis. This observation should be investigated further in a prospective clinical trial.
806
Urticaria: Characterization of 375 cases M. D. C. Petrola1, P. Adriana1, F. Maria1, T. Alcira2, Q. Velmar2; 1University of Carabobo, Valencia, VENEZUELA, 2Ciudad Hospitalaria Enrique Tejera, Valencia, VENEZUELA. RATIONALE: Descriptive study, series of cases. objective: characterization of urticarias. METHODS: Revision of medical histories (two authors, same method and approaches): Urticaria and/or Angioedema - unit of immunology (1996/ 06-2006). Classification: 2 groups: I) clinical presentation: a) Urticaria, b) Urticaria - Angioedema, c) Angioedema. II) time-appearance: a) acute recurrent urticaria (ARU): hives and/or edemas associated to unchain factors, repetitively. b) Chronic Urticaria (CU). more than 6 weeks periodically. RESULTS: 375 patients, 71.9 female%; under 12 years: equal prevalence in bouth sexes. Urticaria 1 angioedema: 63%, without difference between sexes. High serum IgE: 61%: without relationship with sex, age, characteristic, time and appearance; 51.42%: intestinal parasitosis. 13.85% (23) with higher IgE levels over 1000 U.I: 69.6% over 22 years old, 65.4%: ARU, 61%: urticaria-angioedema. 25.58%: allergy to drugs, 67.52% NSAIDs. CU: 34.3% (124), 71.3%: female; 29 (23.38%): well-known causes 44.8%: physical, 37.9% autoantibodies, 17.2%: deficit C1q inhibitor, 3.4%: low complement, 3.4%: HIV. 7.54% of the total group: ANA 1 5 92.9 feminine%, 71.2%: UrticariaAngioedema, 21.51%: ARU. 98% ARU and 99% CU: without collagen vascular illnesses. Positive dermografism: 6.9% total group, ARU prevail. 76.3%: foods as unchain factor, 80.8% with skin tests (ST) positive to foods. 45.9% ST positive to house dust, without concomitant allergic illness. Funding: Consejo de Desarrollo Cientı´fico y Humanı´stico U.C.
807
The use of Danazol in the Management of Refractory Chronic Urticaria M. Karakelides, C. R. Weiler; Mayo School of Graduate Medical Education, Rochester, MN. RATIONALE: Stanozolol has been shown to be an effective and safe adjuvant therapy for the treatment of refractory chronic urticaria in one study. There is only a single case report in the literature reporting the use of danazol in a patient with chronic urticaria. METHODS: The Mayo Clinic electronic database was reviewed to identify patients with the diagnosis of CU who were treated with danazol. The patients’ prior therapies, response to treatment and medication side effects were recorded and analyzed. RESULTS: Three patients were treated with danazol for refractory chronic urticaria. Two patients had resolution of their symptoms within two weeks of initiating therapy. The third patient did not have any symptomatic improvement after two weeks of treatment. This patient also started having episodes of dizziness after initiating treatment. CONCLUSIONS: Danazol may be of therapeutic benefit in the management of chronic urticaria refractory to mainstay therapy.
808
A Case of Urticarial Vasculitis Associated with Chronic Lymphocytic Leukemia J. C. Moore, M. B. Fasano; University of Iowa Hospitals and Clinics, Iowa City, IA. RATIONALE: Although cutaneous lesions are frequently seen in patients with hematological malignancies, cutaneous vasculitis is rarely seen in chronic lymphocytic leukemia (CLL). Here we report a case of CLL presenting as urticarial vasculitis. METHODS: Case report. RESULTS: An 83 year old male with long-standing thrombocytopenia presented to our clinic with a four month history of rash and intermittent lip edema. The rash was described as generalized, pruritic, raised, erythematous, persistent lesions with residual hyperpigmentation. Previous treatment with oral corticosteroids and antihistamines was not effective, nor did the rash resolve after stopping aspirin and hydrochlorothiazide. Skin biopsy showed superficial perivascular mixed inflammation, consistent with urticarial vasculitis. Laboratory tests revealed a normal white blood cell count (WBC 5 5.5 3 109/l, lymphocytes 5 3.1 3 109/l), a normal hemoglobin, and thrombocytopenia (platelet count 5 114 3 109/ml). Complement studies were normal. Serum immunofixation showed an IgG-kappa monoclonal gammopathy. Flow cytometry on peripheral blood demonstrated a monoclonal B-lymphoid population, kappa immunophenotype, with CD20/CD5 co-expression, bright CD23 and 35% CD38 expression, consistent with CLL. CONCLUSIONS: We report a rare cutaneous manifestation of CLL, urticarial vasculitis. In this case, cutaneous lesions resulted in medical referral and evaluation, leading to the diagnosis of CLL. This case highlights the importance of prompt diagnosis of cutaneous vasculitis and the need for additional evaluation to exclude associated systemic conditions such as malignancy. Funding: University of Iowa Hospitals and Clinics
MONDAY
805