J Korean Rheum Assoc.  2007 Jun;14(2):149-153. 10.4078/jkra.2007.14.2.149.

A Case of Septic Arthritis in Patient with Wiskott-Aldrich Syndrome

• Do JH

• KMID: 1526432
• DOI: http://doi.org/10.4078/jkra.2007.14.2.149

Abstract

The Wiskott-Aldrich syndrome is a rare disease characterized by thrombocytopenia, recurrent eczema and a marked vulnerability to recurrent infection. Patients with Wiskott-Aldrich syndrome have frequent infections by bacteria which have polysaccharide capsules such as Pneumococcus, Hemophilus influenzae and Neisseria meningitidis due to poor antibody response to polysaccharide antigens. We experienced a 21 years old man with septic arthritis combined with Pneumococcal pneumonia, multifocal abscesses in back and iliacus muscle. After treatment with antibiotics for one month, he was treated successfully.

Keyword

Wiskott-Aldrich syndrome; Infection; Septic arthritis

MeSH Terms

Abscess
Anti-Bacterial Agents
Antibody Formation
Arthritis, Infectious*
Bacteria
Capsules
Eczema
Haemophilus influenzae
Humans
Neisseria meningitidis
Pneumonia, Pneumococcal
Rare Diseases
Streptococcus pneumoniae
Thrombocytopenia
Wiskott-Aldrich Syndrome*
Young Adult
Anti-Bacterial Agents
Capsules

Figure

• 

  Fig. 1. (A) Chest radiograph shows increased infiltration of right lower lobe. (B) Infiltration was disappeared after antibiotic treatment.

• 

  Fig. 2. (A) CT scan of right shoulder joint shows joint effusion with synovial enhancement (arrow). (B) Joint effusion was disappeared after antibiotic treatment.

• 

  Fig. 3. (A) CT scan of pelvis shows multifocal abscesses of right back muscle and iliacus muscle (arrows). (B) Abscesses were disappeared after antibiotic treatment.

Reference

1). Blaese RM., Strober W., Brown RS., Waldmann TA. The Wiskott-Aldrich syndrome. A disorder with a possible defect in antigen processing or recognition. Lancet. 1968. 1:1056–61.

2). Kildeberg P. The Aldrich syndrome. Report of a case and discussion of pathogenesis. Pediatrics. 1961. 27:362–9.

3). Meuwissen HJ., Bortin MM., Bach FH., Porter IH., Schreinmachers D., Harrison BA, et al. Long-term survival after bone marrow transplantation: a 15-year follow-up report of a patient with Wiskott-Aldrich syndrome. J Pediatr. 1984. 105:365–9.

4). Sordet C., Cantagrel A., Schaeverbeke T., Sibilia J. Bone and joint disease associated with primary immune deficiencies. Joint Bone Spine. 2005. 72:503–14.

5). Wade NA., Lepow ML., Veazey J., Meuwissen HJ. Progressive varicella in three patients with Wiskott-Aldrich syndrome: treatment with adenine arabinoside. Pediatrics. 1985. 75:672–5.

6). Saurat JH. Eczema in primary immune-deficiencies. Clues to the pathogenesis of atopic dermatitis with special reference to the Wiskott-Aldrich syndrome. Acta Derm Venereol Suppl. 1985. 114:125–8.

7). ᄋppenheim JJ., Blaese RM., Waldmann TA. Defective lymphocyte transformation and delayed hypersensitivity in Wiskott-Aldrich syndrome. J Immunol. 1970. 104:835–44.

8). Blaese RM., Strober W., Levy AL., Waldmann TA. Hypercatabolism of IgG, IgA, IgM, and albumin in the Wiskott-Aldrich syndrome. A unique disorder of serum protein metabolism. J Clin Invest. 1971. 50:2331–8.

9). Ayoub EM., Dudding BA., Cooper MD. Dichotomy of antibody response to group A streptococcal antigens in Wiskott-Aldrich syndrome. J Lab Clin Med. 1968. 72:971–9.

10). Mentzer SJ., Remold-ᄋ: Donell E., Crimmins MA., Bierer BE., Rosen FS., Burakoff SJ. Sialophorin, a surface sialoglycoprotein defective in the Wiskott-Aldrich syndrome, is involved in human T lymphocyte proliferation. J Exp Med. 1987. 165:1383–92.

11). Remold-ᄋ: Donnell E., Kenney DM., Parkman R., Cairns L., Savage B., Rosen FS. Characterization of a human lymphocyte surface sialoglycoprotein that is defective in Wiskott-Aldrich syndrome. J Exp Med. 1984. 159:1705–23.

12). Dubiel A., Szczerbinski T., Kurtyka Z., Kowalczyk D. Wiskott-Aldrich syndrome, a case report. Clinical Pediatrics. 1990. 29:434–7.