Dermatologic Manifestations of Job Syndrome

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Dermatologic Manifestations of Job Syndrome

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First described in 1966, the hyperimmunoglobulin E (hyper-IgE or HIE) syndrome is a rare immunodeficiency disorder that has an autosomal dominant inheritance pattern. HIE syndrome has variable expressivity and is associated with multiple abnormalities. The most common findings are recurrent skin abscesses (hence, the name Job syndrome), pneumonia with pneumatocele development, and high serum levels of IgE. Facial, dental, and skeletal features are also associated with this syndrome. A few phenotypic descriptions in the Bible from the Book of Job are said to resemble some of these patients, beyond the boils.^[1]See the image below.

Although most cases are sporadic, multiplex families displaying autosomal dominant and autosomal recessive inheritance have been described.^[2]Autosomal recessive patients tend to have severe molluscum contagiosum and other viral infections and may develop severe neurological complications. These patients also lack skeletal or dental involvement and do not develop lung cysts. Some authorities believe 2 separate syndromes exist, not one.

Type 1 HIE syndrome displays abnormalities in multiple systems, including the skeletal, dental, and immune systems, whereas type 2 HIE syndrome shows abnormalities confined to the immune system.^[3]Hypomorphic mutations have been found in the signal transducer and activator of transcription 3 (STAT3) gene in type 1 HIE syndrome and a null mutation in the tyrosine kinase 2 (Tyk2) gene. Cytokine responses in both types of HIE syndrome revealed severe defects leading to impaired T-helper type 17 function. Another study credited deficiency of Th17 cells in HIE syndrome to mutations in STAT3 in a majority of evaluated patients.^[4]However, defective Th17 responses may be seen in classic disease without STAT3 mutations, with the extent of the defective Th17 response postulated to determine the clinical phenotype.^[5]

Also see the pediatrics article Hyperimmunoglobulinemia E (Job) Syndrome.

The pathophysiology of Job syndrome (HIE syndrome, or hyper-IgE syndrome) is not completely understood.^[6]Patients consistently have a poor, delayed hypersensitivity response to antigens. This delayed response is associated with alterations in T-lymphocyte populations and various interleukin and cytokine abnormalities.^[7]One of the earliest reports on the pathophysiology of Job syndrome described a chemotactic defect in neutrophils.^[8]This defect has since been attributed to defective production of interferon-gamma, a major activator of neutrophils when stimulated by interleukin (IL)–12. The poor production of interferon-gamma in response to IL-12 results in the marked elevation of IgE levels (by means of unopposed IL-4 action).^[9]

Other factors in the abnormal immunologic response are described. Patients with HIE syndrome have elevated levels of granulocyte-macrophage colony-stimulating factor, which may also explain the decreased chemotaxis and increased oxygen radical production and tissue damage.^[10]Deficient suppressor T-cell numbers and activity and an imbalance in helper T cell type 1 (TH1) and helper T cell type 2 (TH2) also may play a role in an abnormal response.^[11]

Although the cytokine dysregulation seems to play a role in its pathophysiology, the causative gene has not yet been identified.^[12]In one study, no unique polymorphisms or mutations were found in candidate genes from the toll-like receptor pathway.^[13]A significantly large number of immunoglobulin-related genes were found to be up-regulated in this syndrome. Perhaps the distinct patterns may facilitate understanding of its pathophysiology and, possibly, its diagnosis.

The hyper-IgE syndromes have multiple genetic bases. The majority of patients have dominant mutations in the signal transducer and activator of transcription 3 (STAT3) gene STAT3.^[14]They do not seem to have a propensity to develop food allergies and anaphylaxis.^[15]Autosomal recessive mutations in DOCK8 are linked with the autosomal recessive hyper-IgE syndrome in combination with severe atopic dermatitis, food allergies, and recurrent pneumonias.^{[16, 17, 18]}Dominant-negative mutations in the STAT3 gene have been associated with the classic multisystem form of hyper-IgE syndrome.^[19]A novel STK4 mutation has been described in patients with autoimmune cytopenias having features overlapping with those of DOCK-8 deficiency hyper-IgE syndrome.^[20]

Hyper-IgE syndrome may be associated with defective salivary activity, which accounts for the enhanced susceptibility of these patients to oral candidiasis.^[21]

Although the described defects in immune response may explain the recurrent infections and chronic dermatitis associated with Job syndrome (HIE syndrome, or hyper-IgE syndrome), the many other congenital abnormalities are not readily explained. A single-locus autosomal dominant model of inheritance with varying expressivity is described,^[22]; the greater severity of cases in younger generations of patients may suggest genetic anticipation. Findings from a multipoint analysis confirm that the proximal 4q region contains the disease locus for Job syndrome.^[23]

Most patients have HIE syndrome caused by dominant negative mutations in the STAT3 gene.^[24]Impaired Th17 cell development may account for the cutaneous pathology.

STAT3 mosaicism may produce a milder phenotype.^[25]

Job syndrome (HIE syndrome, or hyper-IgE syndrome) is a rare disorder; about 250 cases have been published.

Job syndrome (HIE syndrome, or hyper-IgE syndrome) occurs in people of diverse ethnic backgrounds and does not seem to be more common in any specific population.

No sex predilection is reported for Job syndrome (HIE syndrome, or hyper-IgE syndrome).

Job syndrome (HIE syndrome, or hyper-IgE syndrome) usually commences in infancy, but diagnosis is often delayed until childhood or even adulthood.

Few data are available on the prognosis of patients with Job syndrome (HIE syndrome, or hyper-IgE syndrome). Many Job syndrome patients who are undergoing regular monitoring and receiving appropriate treatment will live beyond the age of 50 years. Death is often due to infectious complications. The mortality rate is elevated because of systemic infections.

Significant morbidity is associated with Job syndrome (HIE syndrome, or hyper-IgE syndrome). The vast majority of patients have severe cutaneous and pulmonary disease, and most patients have multiple bone fractures and scoliosis.

Educate patients with Job syndrome (HIE syndrome, or hyper-IgE syndrome) about the importance of recognizing the early signs of infection so that treatment can be initiated as soon as possible.

Mild local pain should be considered a sign of possible infection, and Job syndrome patients should be taught that the typical inflammatory response does not necessarily occur.

Milner JD. Learning from Job: A Rare Genetic Disease and Lessons of Biblical Proportions. Rambam Maimonides Med J. 2018 Jan 29. 9 (1):[Medline].

Grimbacher B, Holland SM, Puck JM. Hyper-IgE syndromes. Immunol Rev. 2005 Feb. 203:244-50. [Medline].

Minegishi Y, Karasuyama H. Genetic origins of hyper-IgE syndrome. Curr Allergy Asthma Rep. 2008 Sep. 8(5):386-91. [Medline].

Ma CS, Chew GY, Simpson N, et al. Deficiency of Th17 cells in hyper IgE syndrome due to mutations in STAT3. J Exp Med. 2008 Jul 7. 205(7):1551-7. [Medline]. [Full Text].

van de Veerdonk FL, Marijnissen R, Joosten LA, et al. Milder clinical hyperimmunoglobulin E syndrome phenotype is associated with partial interleukin-17 deficiency. Clin Exp Immunol. 2010 Jan. 159(1):57-64. [Medline]. [Full Text].

Sowerwine KJ, Holland SM, Freeman AF. Hyper-IgE syndrome update. Ann N Y Acad Sci. 2012 Feb. 1250:25-32. [Medline].

Stiehm ER. Cytokine dysregulation in the hyperimmunoglobulinemia E syndrome. J Pediatr. 2000 Feb. 136(2):141-3. [Medline].

Paslin D, Norman ME. Atopic dermatitis and impaired neutrophil chemotaxis in Job’s syndrome. Arch Dermatol. 1977 Jun. 113(6):801-5. [Medline].

Simon HU, Seger R. Hyper-IgE syndrome associated with an IL-4-producing gamma/delta(+) T-cell clone. J Allergy Clin Immunol. 2007 Jan. 119(1):246-8. [Medline].

Vargas L, Patino PJ, Rodriguez MF, et al. Increase in granulocyte-macrophage-colony-stimulating factor secretion and the respiratory burst with decreased L-selectin expression in hyper-IgE syndrome patients. Ann Allergy Asthma Immunol. 1999 Sep. 83(3):245-51. [Medline].

Shirafuji Y, Matsuura H, Sato A, Kanzaki H, Katayama H, Arata J. Hyperimmunoglobin E syndrome: a sign of TH1/TH2 imbalance?. Eur J Dermatol. 1999 Mar. 9(2):129-31. [Medline].

Tanaka T, Takada H, Nomura A, Ohga S, Shibata R, Hara T. Distinct gene expression patterns of peripheral blood cells in hyper-IgE syndrome. Clin Exp Immunol. 2005 Jun. 140(3):524-31. [Medline].

Hawn TR, Ozinsky A, Williams LM, et al. Hyper-IgE syndrome is not associated with defects in several candidate toll-like receptor pathway genes. Hum Immunol. 2005 Jul. 66(7):842-7. [Medline].

Engelhardt KR, McGhee S, Winkler S, et al. Large deletions and point mutations involving the dedicator of cytokinesis 8 (DOCK8) in the autosomal-recessive form of hyper-IgE syndrome. J Allergy Clin Immunol. 2009 Dec. 124(6):1289-302.e4. [Medline]. [Full Text].

Dosanjh A. Pediatric anaphylaxis and hyper IgE syndrome. J Asthma Allergy. 2017. 10:31-33. [Medline].

Koskenvuo M, Kainulainen L, Vanto T, Lukkarinen H, Lähteenmäki P, Ruuskanen O. [Severe atopy and allergy–rare hyper-IgE syndrome caused by the DOCK8 mutation as underlying condition]. Duodecim. 2015. 131 (6):541-4. [Medline].

Hagl B, Heinz V, Schlesinger A, et al. Key findings to expedite the diagnosis of hyper-IgE syndromes in infants and young children. Pediatr Allergy Immunol. 2016 Mar. 27 (2):177-84. [Medline].

Wang S, Mou W, Xu Z, Gui J, Ma L. Autosomal recessive hyper-IgE syndrome in two brothers of a Chinese family with a novel mutation in DOCK8 gene. J Eur Acad Dermatol Venereol. 2018 Feb 8. [Medline].

Minegishi Y. Hyper-IgE syndrome. Curr Opin Immunol. 2009 Oct. 21(5):487-92. [Medline].

Halacli SO, Ayvaz DC, Sun-Tan C, Erman B, Uz E, Yilmaz DY, et al. STK4 (MST1) deficiency in two siblings with autoimmune cytopenias: A novel mutation. Clin Immunol. 2015 Jun 25. [Medline].

Conti HR, Baker O, Freeman AF, Jang WS, Holland SM, Li RA, et al. New mechanism of oral immunity to mucosal candidiasis in hyper-IgE syndrome. Mucosal Immunol. 2011 Feb 23. [Medline].

Grimbacher B, Holland SM, Gallin JI, et al. Hyper-IgE syndrome with recurrent infections–an autosomal dominant multisystem disorder. N Engl J Med. 1999 Mar 4. 340(9):692-702. [Medline].

Grimbacher B, Schaffer AA, Holland SM, et al. Genetic linkage of hyper-IgE syndrome to chromosome 4. Am J Hum Genet. 1999 Sep. 65(3):735-44. [Medline].

Minegishi Y, Saito M. Cutaneous Manifestations of Hyper IgE Syndrome. Allergol Int. 2012 Mar 25. 0(0):[Medline].

Hsu AP, Sowerwine KJ, Lawrence MG, Davis J, Henderson CJ, Zarember KA, et al. Intermediate phenotypes in patients with autosomal dominant hyper-IgE syndrome caused by somatic mosaicism. J Allergy Clin Immunol. 2013 Jun. 131(6):1586-93. [Medline].

Vigliante CE, Costello BJ, Quinn PD. Life-threatening cervicofacial infection in a child with hyperimmunoglobulin-E syndrome. J Oral Maxillofac Surg. 2001 May. 59(5):561-5. [Medline].

O’Connell AC, Puck JM, Grimbacher B, et al. Delayed eruption of permanent teeth in hyperimmunoglobulinemia E recurrent infection syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000 Feb. 89(2):177-85. [Medline].

Onal IK, Kurt M, Altundag K, Aksoy S, Dincer M, Gullu I. Peripheral T-cell lymphoma and Job’s syndrome: a rare association. Med Oncol. 2006. 23(1):141-4. [Medline].

Ling JC, Freeman AF, Gharib AM, et al. Coronary artery aneurysms in patients with hyper IgE recurrent infection syndrome. Clin Immunol. 2007 Mar. 122(3):255-8. [Medline].

Sarmento KM Jr, Tomita S, Caliman e Gurgel JD. Association between nasal polyposis, Dubowitz syndrome and hyper-IgE syndrome. Int J Pediatr Otorhinolaryngol. 2008 May. 72(5):711-4. [Medline].

Kharkar V, Kardekar S, Gutte R, Mahajan S, Thakkar V, Khopkar U. Disseminated molluscum contagiosum infection in a hyper IgE syndrome. Indian J Dermatol Venereol Leprol. 2012 May. 78(3):371-4. [Medline].

Siah TW, Gennery A, Leech S, Taylor A. Gross generalized molluscum contagiosum in a patient with autosomal recessive hyper-IgE syndrome, which resolved spontaneously after haematopoietic stem-cell transplantation. Clin Exp Dermatol. 2013 Mar. 38(2):196-7. [Medline].

Borges WG, Hensley T, Carey JC, Petrak BA, Hill HR. The face of Job. J Pediatr. 1998 Aug. 133(2):303-5. [Medline].

Freeman AF, Domingo DL, Holland SM. Hyper IgE (Job’s) syndrome: a primary immune deficiency with oral manifestations. Oral Dis. 2009 Jan. 15(1):2-7. [Medline].

James J, Thekkeveetil AK, Vadakkepurayil K. Oral Manifestations of Job’s Syndrome in a Paediatric Dental Patient – A Case Report. J Clin Diagn Res. 2016 Nov. 10 (11):ZD04-ZD05. [Medline].

Rana C, Krishnani N, Kumari N, Shastri C, Poddar U. Rectal histoplasmosis in Job’s syndrome. Indian J Gastroenterol. 2013 Jan. 32(1):64-5. [Medline].

Hori J, Yamaguchi S, Watanabe M, Osanai H, Hori M. Fournier gangrene associated with hyper IgE syndrome (Job syndrome). Int J Urol. 2008 Apr. 15(4):372-3. [Medline].

Kumanovics A, Perkins SL, Gilbert H, Cessna MH, Augustine NH, Hill HR. Diffuse large B cell lymphoma in hyper-IgE syndrome due to STAT3 mutation. J Clin Immunol. 2010 Nov. 30(6):886-93. [Medline].

Schimke LF, Sawalle-Belohradsky J, Roesler J, et al. Diagnostic approach to the hyper-IgE syndromes: immunologic and clinical key findings to differentiate hyper-IgE syndromes from atopic dermatitis. J Allergy Clin Immunol. 2010 Sep. 126(3):611-7.e1. [Medline].

Khan K, Wozniak SE, Giannone AL, Abdulmassih ME. A Boy with Relentless Pruritus: Job’s Syndrome. Am J Case Rep. 2016 Feb 21. 17:104-10. [Medline].

Patiroglu T, Gungor HE, Lazaroski S, Unal E. Chronic granulomatous disease with markedly elevated IgE levels mimicking hyperimmunoglobulin E syndrome. Acta Microbiol Immunol Hung. 2013 Jun. 60(2):155-62. [Medline].

Gamberale A, Moreira I, Bartoletti B, Cruz V, Bezrodnik L, Alberti F, et al. [Job’s syndrome and miliary tuberculosis]. Medicina (B Aires). 2014. 74(4):311-4. [Medline].

Belhassen-García M, Pardo-Lledías J, Pérez del Villar L, Muro A, Velasco-Tirado V, Blázquez de Castro A, et al. Relevance of eosinophilia and hyper-IgE in immigrant children. Medicine (Baltimore). 2014 Jul. 93(6):e43. [Medline].

Kumanovics A, Wittwer CT, Pryor RJ, et al. Rapid Molecular Analysis of the STAT3 Gene in Job Syndrome of Hyper-IgE and Recurrent Infectious Diseases. J Mol Diagn. 2010 Jan 21. [Medline].

Ge AX, Ryan ME, Holland SM, et al. Acupuncture for symptom management in patients with hyper-IgE (Job’s) syndrome. J Altern Complement Med. 2011 Jan. 17(1):71-6. [Medline]. [Full Text].

Tanaka H, Ito R, Onodera N, Waga S. Efficacy of long-term sulfamethoxazole-trimethoprim therapy in a boy with hyperimmunoglobulin E syndrome. Tohoku J Exp Med. 1998 Sep. 186(1):61-6. [Medline].

Kojima K, Inoue Y, Katayama Y, et al. Improvement with disodium cromoglycate of neutrophil phagocytosis and respiratory burst activity in a patient with hyperimmunoglobulin E syndrome. Allergy. 1998 Nov. 53(11):1101-3. [Medline].

Wakim M, Alazard M, Yajima A, Speights D, Saxon A, Stiehm ER. High dose intravenous immunoglobulin in atopic dermatitis and hyper-IgE syndrome. Ann Allergy Asthma Immunol. 1998 Aug. 81(2):153-8. [Medline].

Bittner TC, Pannicke U, Renner ED, et al. Successful long-term correction of autosomal recessive hyper-IgE syndrome due to DOCK8 deficiency by hematopoietic stem cell transplantation. Klin Padiatr. 2010 Nov. 222(6):351-5. [Medline].

Metin A, Tavil B, Azik F, Azkur D, Ok-Bozkaya I, Kocabas C, et al. Successful bone marrow transplantation for DOCK8 deficient hyper IgE syndrome. Pediatr Transplant. 2012 Jan 17. [Medline].

Ge AX, Ryan ME, Holland SM, et al. Acupuncture for symptom management in patients with hyper-IgE (Job’s) syndrome. J Altern Complement Med. 2011 Jan. 17(1):71-6. [Medline]. [Full Text].

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi