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CASE REPORT
Year : 2012  |  Volume : 13  |  Issue : 1  |  Page : 38-40

Job syndrome


Department of Dermatology, Venereology and Leprosy, Fr. Muller Muller Medical College Hospital, Kankanady, Mangalore, Karnataka, India

Date of Web Publication23-Oct-2012

Correspondence Address:
Ramesh M Bhat
Department of Dermatology, Venereology and Leprosy, Fr. Muller Muller Medical College Hospital, Kankanady, Mangalore- 575 002, Karnataka
India
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Source of Support: None, Conflict of Interest: None


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  Abstract 

Job syndrome is a heterogeneous group of disorders characterised by recurrent staphylococcal infections, unusual eczema-like skin rashes, severe lung infections that result in pneumatoceles and very high concentrations of serum IgE. We present a 10-year-old boy with itchy pus-filled lesions all over the body since the third day of life. On examination, multiple excoriated papules and plaques with crusts distributed over the extremities, trunk, face, palms, scalp and genitalia were present. Staphylococcus aureus was grown on the pus culture. Serum IgE was significantly elevated. The patient showed remarkable improvement with topical and systemic antibiotics and vitamin C supplements. Early diagnosis of phagocytic disorders can be lifesaving and can lead to a significant reduction in morbidity.

Keywords: Serum IgE, staphylococcal infections, job syndrome


How to cite this article:
Shobha V, Bhat RM, Sukumar D, Srinath M K, Pinto A C. Job syndrome. Indian J Paediatr Dermatol 2012;13:38-40

How to cite this URL:
Shobha V, Bhat RM, Sukumar D, Srinath M K, Pinto A C. Job syndrome. Indian J Paediatr Dermatol [serial online] 2012 [cited 2019 May 20];13:38-40. Available from: http://www.ijpd.in/text.asp?2012/13/1/38/102811


  Introduction Top


Job syndrome is a heterogeneous group of disorders characterised by recurrent staphylococcal infections, unusual eczema-like skin rashes, severe lung infections that result in pneumatoceles and very high concentrations of serum IgE. Synonyms are hyperimmunoglobulin E recurrent infection syndrome, Buckley syndrome and hyper IgE syndrome (HIES).


  Case Report Top


A 10-year-old boy, born of consanguineous marriage and out of uneventful pregnancy, presented with itchy, pus-filled lesions all over the body and fever of 1 month duration. The lesions initially developed over his legs, which, over a period of 1 week, involved the upper limbs, trunk, face, scalp and palms. The lesions started with itching, becoming clear fluid filled, and later becoming pus filled, with a foul smell. There was history of sudden onset of several episodes of similar lesions since the third day of life, with symptom-free intervals of 1-2 months.

On examination, the child had multiple excoriated papules and plaques with crusts distributed over the extremities, trunk, face, palms, scalp and genitalia [Figure 1]. A few flaccid vesicles over the non-inflammed skin were present over the extremities [Figure 2]. Nikolskiy's and bulla spread signs were negative. Multiple pustules were seen over the trunk and extremities. There was generalised tender lymphadenopathy. Mucosae were normal. A differential diagnosis of atopic dermatitis, Job syndrome and pemphigus foliaceous was considered.
Figure 1: Multiple excoriated papules and plaques with crusts

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Figure 2: Flaccid vesicles over the non-inflammed base

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The investigations revealed a neutrophilic leukocytosis (18,600/cmm and neutrophils - 89%), normocytic normochromic to microcytic hypochromic peripheral smear. Tzanck smear showed neutrophils and absence of acantholytic cells. Pus for culture and sensitivity revealed a heavy growth of Staphylococcus aureus. Serum IgE was elevated (6816.00 IU/mL). Chest X-ray was normal. Skin biopsy showed intra-epidermal bulla [Figure 3] containing neutrophils, acantholytic cells and fibrin, while the dermis showed mixed inflammatory infiltrate composed of lymphocytes and eosinophils [Figure 4]. Direct immunofluorescence was negative.
Figure 3: Intraepidermal bulla containing neutrophils, acantholytic cells and fibrin and dermis showing mixed inflammatory infiltrate (hematoxylin and eosin stain, magnification ×10)

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Figure 4: Intraepidermal bulla containing neutrophils and fibrin (hematoxylin and eosin stain, magnification ×40)

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In view of the recurrent eczema with infections and elevated IgE levels, a diagnosis of Job syndrome was considered and treated with topical and systemic antibiotics and vitamin C supplements. The patient responded favorably to the treatment.


  Discussion Top


Job syndrome is a primary phagocytic disorder characterized by atopic-like dermatitis, "cold" soft tissue abscesses, recurrent pneumonias, pneumatoceles, craniofacial and skeletal abnormalities. [1]

In 1966, Davis, Schaller and Wedgwood first reported Job syndrome. The nomenclature is derived from the similarity of the condition to the biblical Prophet Job who was afflicted with sore boils on the sole of his feet. Pherwani et al, in 1994 reported the first case from India. [2],[3]

Clinical Features

The disease is characterised by papulopustular eruption with prominent crusting distributed over the scalp, face, neck, axillae and diaper area, with superinfection of the dermatitis with S. aureus. Impetigo, furunculosis, paronychia, cellulitis and characteristic "cold abscesses" are the staphylococcal infections seen. Pulmonary bacterial pneumonia, abscesses and empyema resulting in pneumatoceles are most frequent. Progressive coarsening of facial features reflect skeletal defects. Facial features like prominent forehead, broad nasal bridge and wide nasal tip are present by the age of 16 years. Dental anomalies include retention of primary teeth and lack of eruption of secondary teeth. Osteopenia, with at least three pathological fractures of the long bones, ribs and pelvis, is present in 57% of the patients, scoliosis in 63% of the adult patients and hyperextensibility of the joints in 68% of the patients. Usually, an elevated serum IgE level more than 2000 IU/mL and elevated eosinophil levels above 700 cells/μL with a normal white blood cell count will be seen. [4]

Autosomal-recessive HIES (AR-HIES) manifests as severe eczema, recurrent bacterial and viral skin infections, autoimmunity and devastating neurological complications that are often fatal in childhood. These patients have no tendency to form pneumatoceles and have no skeletal or dental abnormalities. Eosinophilia is more severe (17,500/μL). [4]

Atopic dermatitis patients usually have multiple breaks that occur in the peripheral retina and vitreous base, which may lead to rhegmatogenous retinal detachment and giant retinal breaks. [5]

Investigations recommended in these patients include complete blood count, blood differential count, absolute eosinophil count and serum globulin electrophoresis to look for high blood IgE levels. Chest X-ray may reveal lung abscesses. Other investigations include X-ray of the sinuses, X-ray of the bones, computed tomography scan of the chest and cultures of infected site. [4]

Prognosis in these patients is difficult to predict due to lack of correlation between serum levels of IgE, eosinophilia and susceptibility to infections. Patients with AR-HIES have a more severe course due to neurological complications. [4]

Job syndrome cannot be cured. Anti-staphylococcal antibiotics are effective for cutaneous infections. Prophylactic use of anti-staphylococcal antibiotics markedly reduces the incidence of skin abscesses and pneumonia. Incision and drainage for cutaneous and lung abscesses is required. IVIG therapy can be given. Ascorbic acid and cimetidine decrease the number of episodes of infections. Isotretinoin, cyclosporine-A and interferon-γ are also tried. [6]

Even though our patient did not have any skeletal and pulmonary abnormalities, dermatological findings like recurrent eczema with infections and elevated IgE levels prompted us to arrive at the diagnosis of Job syndrome. The skeletal abnormalities are usually seen during adolescence and the patient needs to be followed-up for these developments.


  Conclusion Top


Primary phagocytic disorders like HIES are rare and usually first manifest during childhood. A phagocytic disorder should be considered in patients with unusually severe and recurrent infections by common pathogens. Patients usually die prematurely due to pulmonary infections. Early diagnosis of phagocytic disorders can be lifesaving and can lead to a significant reduction in morbidity.

 
  References Top

1.Davis S, Schaller J, Wedgwood R. "Job's syndrome. Recurrent, "cold", staphylococcal abscesses". Lancet 1966;1:1013-5.  Back to cited text no. 1
    
2.Donabedian H, Gallin JI. The Hyper immunoglobulin E recurrent Infection (Job's) syndrome. A review of the NIH experience and literature. Medicine 1983;62:195-208.  Back to cited text no. 2
[PUBMED]    
3.Pherwani AV, Rodrigues C, Dasgupta A, Bavadekar M, Rao ND. "Hyper immunoglobulin E syndrome". Indian Pediatr 1994;31:328-30.  Back to cited text no. 3
    
4.Grimbacher B, Holland SM, Puck JM. "Hyper -IgE syndromes". Immunol Rev 2005;203:244-50.  Back to cited text no. 4
[PUBMED]    
5.Elizabeth HM, Hirose T. "Retinal detachment in pediatric population" AND Schepens' retinal detachment and allied diseases. 2nd ed. Boston: Butterworth-Heinemann publishers; 2000. p. 550610.  Back to cited text no. 5
    
6.Kimata H. "High-dose intravenous gamma-globulin treatment for hyperimmunoglobulinemia E syndrome". J Allergy Clin Immunol 1995;95:771-4.  Back to cited text no. 6
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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Abstract
Introduction
Case Report
Discussion
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