Molluscum contagiosum and Job's syndrome.
MN Martins, MS Tullu, SA Mahajan
Department of Paediatrics, Seth G. S. Medical College and K. E. M. Hospital, Parel, Mumbai - 400 012, India., India
M N Martins
Department of Paediatrics, Seth G. S. Medical College and K. E. M. Hospital, Parel, Mumbai - 400 012, India.
|How to cite this article:|
Martins M N, Tullu M S, Mahajan S A. Molluscum contagiosum and Job's syndrome. J Postgrad Med 2001;47:268-9
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Martins M N, Tullu M S, Mahajan S A. Molluscum contagiosum and Job's syndrome. J Postgrad Med [serial online] 2001 [cited 2020 Jul 15 ];47:268-9
Available from: http://www.jpgmonline.com/text.asp?2001/47/4/268/172
A three-and-half-year-old male child presented with fever, cough and breathlessness of four day’s duration. The child was diagnosed as a case of Job’s Syndrome (Hyper-IgE syndrome) at the age of two years (IgE level: 15062 IU/ml; reference range: 10-180 IU/ml) and had previous three admissions for lower respiratory tract infections. The child had respiratory distress and crepitations were heard bilaterally. He also had multiple papular lesions scattered on the face and the neck [Figure:1]. These were diagnosed as molluscum contagiosum on a skin biopsy [Figure:2]. The chest radiograph revealed bronchopneumonia with multiple pneumatocoeles in both the lungs. Investigations revealed anaemia (haemoglobin: 7.3 g/dl; PCV: 25%), leukocytosis (count of 25,900 cells/cumm), eosinophilia (absolute eosinophil count of 518 cells/cumm), high ESR (55 mm at 1 hr), hypoxia on arterial blood gas analysis and normal liver and renal function tests. Coagulase negative staphylococcus was isolated from the skin swab. The patient was treated with oxygen, intravenous fluids and intravenous vancomycin and amikacin (for 14 days). The extraction of some of the molluscum contagiosum lesions was performed. For the remaining lesions, the child is on treatment with topical Tretinoin cream (0.05% locally).
Job’s syndrome is a rare primary immunodeficiency disorder characterised by extremely high levels of serum immunoglobulin E (IgE) and recurrent serious staphylococcal infections of skin, lungs, joints, etc., Variable associated features include coarse facies, cold cutaneous abscesses, eczematous rashes and osteopenia. Apart from high serum IgE levels, blood and sputum eosinophilia and poor cell-mediated and antibody responses to neoantigens may be present., Also, variable defects of mononuclear and/or polymorphonuclear chemotaxis is present in some cases., Treatment necessitates the use of antibiotics for the infections and surgical drainage of the abscesses. Long-term treatment with penicillinase-resistant penicillin is recommended to prevent recurrent infections., Intravenous immunoglobulin may be administered to those with antibody-deficiency. Surgery may be required for superinfected pneumatoceles or pneumatoceles persisting beyond six months.,
Molluscum contagiosum lesions are characterized by single or multiple, rounded, dome shaped, pink waxy papules ranging from 1 to 5 mm (rarely up to 1 cm) in diameter. The causative agent is a DNA poxvirus. The lesions are umbilicated and contain a caseous plug. These lesions are commonly seen on the face, eyelids, neck, axillae and thighs. The mode of acquisition is via direct contact with an infected person or via fomites and spread occurs by autoinoculation. Histologically, there are cells with intracytoplasmic inclusion bodies, also called molluscum bodies or Henderson Paterson bodies [Figure:2]. These first appear as single, minute, ovoid eosinophilic structures in the stratum malphigium and become increasingly prominent as the cells move upwards from the basal layer to the stratum corneum. There may be spontaneous resolution of the lesions over few months. In cases with widespread lesions and in those with immunosuppression, thorough treatment is necessary. Treatment of the patient also prevents spread to other family members. The management entails extraction of the lesions with a curette. Other modalities of management include - use of local Tretinoin, liquid nitrogen, cantharidin (0.9%), benzoyl peroxide, trichloroacetic acid, cryotherapy, etc., A lesion-free period of 4 months can be considered as cure. Molluscum contagiosum is an epidermal disorder and over-treatment should be avoided to prevent scarring.
This case is being presented to highlight the occurrence of multiple lesions of molluscum contagiosum in a child with Job’s syndrome. Extensive lesions of molluscum contagiosum can occur in immunodeficient children and in those with atopic dermatitis. It is unclear whether patients with Job’s syndrome are at an increased risk for developing extensive or severe viral infections. Viral infections like molluscum contagiosum need intact cell- mediated immunity for combating the infection. Job’s syndrome is known to be associated with poor antibody and cell- mediated responses to neo-antigens. This may be a possible reason for extensive molluscum contagiosum lesions in our case.
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