Abstract
Hyperimmunoglobulin E syndrome is a rare disorder characterized by pruritic dermatitis, recurrent staphylococcal skin abscesses and extremely elevated concentrations of immunoglobulin E in serum.
We report a patient who has pruritic vesiculopapules, recurrent episodes of skin abscesses on the scalp and face, and respiratory problems from the age of ten days (severe cough, wheezing, acute otitis media, pneumonia). She was diagnosed as Hyper-lgE syndrome and treated with IVIG along with antibiotics.
Key Words: Hyperimmunoglobulin E
syndrome, Job’s syndrome
References
- Chamlin SL, McCalmont TH, Cunningham BB, et al. Cutaneous manifestations of hyper-lgE syndrome in infants and children. J Fediatr 2002;141:572-575.
- Davis SD, Schaller S, Wedgewood FU. Job's syndrome: Recurrent 'Cold staphylococcal abscesses. Lancet 1996;i: 1013-1015.
- Buckley RM, Wray BB, Belmaker E. Extreme hyperimmunoglobulin E and undue susceptibility to infection. Pediatrics 1972;49:59-70.
- Donabedian H, Oallin Jl. The hyperimmunoglobulin E syndrome, recurrent infection (Job's) syndrome: A review of the HIM experience and the literature. Medicine 1983;62:195-208.
- Moneret-Vautrin DA, Kanny G, Thinus G. Myperimmunoglobulin E syndrome with recurrent infections (Job's syndrome). Rev Med Intern 1999;20:133-140.
- Shirafuji Y, Matsuura H, Sato A, et al. Myperimmunoglobulin E syndrome: a sign of TM1/TM2 imbalance? Eur J Dermatol 1999;9: 129-131.
- Grimbacher B, Holland SM, Gallin Jl, et al. Hyper-lg E syndrome with recurrent infections—an autosomal dominant multisystem disorder. M Engl J Med 1999; 340:692-702.
- Pherwani AV, Madnani MA.
- Hyperimmunoglobulin E syndrome. Indian Pediatrics 2001 ;38:1029-1034.
- Buckley RH. Immunodeficiency with
- thrombocytopenia and eczama (Wiskott- Aldrich Syndrome). In: Behrman RE, Kliegman RM, Arvin AM, eds. Melson Textbook of Pediatrics, 15th. ed. USA: W.B. Saunders
- Company, 1996:576.
- Waldfahrer F, Pah! S, Federspil PA, Iro H. Hyper-lg E syndrome with EMT manifestations. Overview and case report of successful therapy with high dosage i.v. immunoglobulin. HMO 1999;47:1063-1068.
- Bilora E, Petrobelli F, Boccioletti V, Pomerri E. Moderate-dose intravenous immunoglobulin treatment of Job's syndrome. A case report. Minerva Med 2000;91:113-1 16.
- Gennery AR, Flood TJ, Abinun M, Cant AJ. Bone marrow transplantation does not correct the hyper-lg E syndrome. Bone
Abstract
Hyperimmunoglobulin E syndrome is a rare disorder characterized by pruritic dermatitis, recurrent staphylococcal skin abscesses and extremely elevated concentrations of immunoglobulin E in serum.
We report a patient who has pruritic vesiculopapules, recurrent episodes of skin abscesses on the scalp and face, and respiratory problems from the age of ten days (severe cough, wheezing, acute otitis media, pneumonia). She was diagnosed as Hyper-lgE syndrome and treated with IVIG along with antibiotics.
Key Words: Hyperimmunoglobulin E
syndrome, Job’s syndrome
References
- Chamlin SL, McCalmont TH, Cunningham BB, et al. Cutaneous manifestations of hyper-lgE syndrome in infants and children. J Fediatr 2002;141:572-575.
- Davis SD, Schaller S, Wedgewood FU. Job's syndrome: Recurrent 'Cold staphylococcal abscesses. Lancet 1996;i: 1013-1015.
- Buckley RM, Wray BB, Belmaker E. Extreme hyperimmunoglobulin E and undue susceptibility to infection. Pediatrics 1972;49:59-70.
- Donabedian H, Oallin Jl. The hyperimmunoglobulin E syndrome, recurrent infection (Job's) syndrome: A review of the HIM experience and the literature. Medicine 1983;62:195-208.
- Moneret-Vautrin DA, Kanny G, Thinus G. Myperimmunoglobulin E syndrome with recurrent infections (Job's syndrome). Rev Med Intern 1999;20:133-140.
- Shirafuji Y, Matsuura H, Sato A, et al. Myperimmunoglobulin E syndrome: a sign of TM1/TM2 imbalance? Eur J Dermatol 1999;9: 129-131.
- Grimbacher B, Holland SM, Gallin Jl, et al. Hyper-lg E syndrome with recurrent infections—an autosomal dominant multisystem disorder. M Engl J Med 1999; 340:692-702.
- Pherwani AV, Madnani MA.
- Hyperimmunoglobulin E syndrome. Indian Pediatrics 2001 ;38:1029-1034.
- Buckley RH. Immunodeficiency with
- thrombocytopenia and eczama (Wiskott- Aldrich Syndrome). In: Behrman RE, Kliegman RM, Arvin AM, eds. Melson Textbook of Pediatrics, 15th. ed. USA: W.B. Saunders
- Company, 1996:576.
- Waldfahrer F, Pah! S, Federspil PA, Iro H. Hyper-lg E syndrome with EMT manifestations. Overview and case report of successful therapy with high dosage i.v. immunoglobulin. HMO 1999;47:1063-1068.
- Bilora E, Petrobelli F, Boccioletti V, Pomerri E. Moderate-dose intravenous immunoglobulin treatment of Job's syndrome. A case report. Minerva Med 2000;91:113-1 16.
- Gennery AR, Flood TJ, Abinun M, Cant AJ. Bone marrow transplantation does not correct the hyper-lg E syndrome. Bone
Details
| Journal Section | Case Reports |
|---|---|
| Authors | |
| Publication Date | December 3, 2016 |
| Published in Issue | Year 2003 Volume: 16 Issue: 3 |