Skin Immunity Sample Collection Involving Blisters and Biopsies

Background: The way the body heals and protects itself from getting sick is called the immune response. Some people with weak immune systems get sick often or get rashes and skin infections. Researchers want to find out how the immune system and skin problems are related so they can help these people. Objective: To learn about how immune response and skin healing are related to each other. Eligibility: People ages 18-65 with hyper IgE syndrome or Job syndrome or people ages 7-65 with chronic granulomatous disease. Healthy volunteers ages 18 65 are also needed. Design: Participants will be screened with: Medical history Physical exam Possible urine tests Participants will have 1 to 3 visits within about a week. Visits will include the following: Participants will have a wells device strapped to the inside of the forearm. It will suction the skin and pull the top layer away to form 8 blisters. The skin over the blisters and the liquid inside will be collected. Participants will have up to 4 skin biopsies. A sharp tool will remove a small plug of skin from the forearm. Participants may have blood and urine tests. The skin on participants skin will be rubbed with a cotton swab. Some participants will have an overnight visit. They will have the blister device placed back on the arm. The wells will be lined up over the blister wounds. The wells will be filled with either saline or the participant s blood serum. The device will be covered and left on the arm for up to 24 hours. Doctors will periodically remove some liquid from the wells.

Sex

All

Minimum age

7 Years

Maximum age

65 Years

* INCLUSION CRITERIA:

  1. Meets one of the following:

     1. has documentation of HIES or CGD (patient populations); or
     2. does not have clinically apparent evidence of an immune defect or history of invasive or recurrent S. aureus infections (healthy volunteers).
  2. Between the following age limits (inclusive):

     1. 18 and 65 years old for healthy volunteers;
     2. 7 and 65 years old for patients with CGD;
     3. 18 and 65 years old for patients with HIES.
  3. Willing to allow storage of blood, RNA, bacterial and fungal cultures, and other tissue samples for future research.
  4. Able to provide informed consent.

EXCLUSION CRITERIA:

The following exclusion criteria apply to all subjects:

1. For individuals undergoing blister or skin biopsy procedures, history of keloid formation.
2. Current or prior (within 3 months) anticoagulant or anti-platelet therapy (other than aspirin or non-steroidal anti-inflammatory drugs \[NSAIDs\]).
3. Current or prior (within 3 months) use of immunomodulatory drugs (e.g., chemotherapy, steroids), except if approved by the principal investigator.
4. Pregnancy.
5. Any condition that, in the opinion of the investigator, contraindicates participation in the study.

• BACKGROUNDKlevens RM, Morrison MA, Nadle J, Petit S, Gershman K, Ray S, Harrison LH, Lynfield R, Dumyati G, Townes JM, Craig AS, Zell ER, Fosheim GE, McDougal LK, Carey RB, Fridkin SK; Active Bacterial Core surveillance (ABCs) MRSA Investigators. Invasive methicillin-resistant Staphylococcus aureus infections in the United States. JAMA. 2007 Oct 17;298(15):1763-71. doi: 10.1001/jama.298.15.1763. PMID 17940231
• BACKGROUNDNaimi TS, LeDell KH, Como-Sabetti K, Borchardt SM, Boxrud DJ, Etienne J, Johnson SK, Vandenesch F, Fridkin S, O'Boyle C, Danila RN, Lynfield R. Comparison of community- and health care-associated methicillin-resistant Staphylococcus aureus infection. JAMA. 2003 Dec 10;290(22):2976-84. doi: 10.1001/jama.290.22.2976. PMID 14665659
• BACKGROUNDMyles IA, Datta SK. Staphylococcus aureus: an introduction. Semin Immunopathol. 2012 Mar;34(2):181-4. doi: 10.1007/s00281-011-0301-9. Epub 2012 Jan 27. No abstract available. PMID 22282052