Questions about XP patient care? XP Expert Resource Group offers answers

Expert Resource Groups (ERGs) are ancillary Academy groups comprised of individuals with specific expertise who come together to share information and knowledge about a particular topic. The ERGs share a passion for the advancement of patient care and are valuable contributors to an array of Academy initiatives and efforts. The Academy presently has 10 approved ERGs: Atopic Dermatitis; Dermatologists in Industry; Epidemiology; Lesbian, Gay, Bisexual, and Transgender and Sexual Minority Health; Quality and Patient Safety Officers; Psoriasis; Sexually Transmitted Diseases; Society of Dermatology Hospitalists; Women’s Health; and Xeroderma Pigmentosum (XP).

Member to Member recently interviewed John DiGiovanna, MD and Kenneth Kraemer, MD, co-directors of the XP-ERG, about the group’s mission.

Member to Member: Why was the XP-ERG formed?

digiovanna_john.jpgDrs. DiGiovanna and Kraemer: Xeroderma pigmentosum (XP) and related disorders are very rare and have high morbidity and mortality. XP presents great difficulties for patients, their families and their treating dermatologists. These disorders may be difficult to diagnose.

Skin cancer occurs in these patients at a frequency 10,000-fold higher than the general population, and typically affects young children, with an average age of less than 10 years.

kraemer-kenneth.jpgInfants with XP can develop severe blistering sunburns on minimal sun exposure. Sadly, distressed parents trying to address a severe, unexpected blistering sunburn can be wrongly accused of child abuse.

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Skin cancer occurs in XP patients at a frequency 10,000-fold higher than the general population, and typically affects young children. Patients often are clocked in protective wear to protect against the sun.

The large numbers of skin cancers at a very early age pose many logistic and management problems. The extreme degree of sun protection required is difficult to understand and to achieve. With many subtle differences in subtypes, understanding which problems need surveillance, and how to achieve that can be complex.

Organized before 2000 as the XP Task Force, the AAD recognized that they could make a substantial impact on many of these problems and provide a huge service to dermatologists and patients by providing a venue for bringing dermatologists caring for these patients, together with experts having experience in XP management along with resources, such as the patient advocates and researchers. This group could facilitate development of practical solutions to difficult management problems.

Since these stakeholders, including many international participants, typically attend the annual meeting of the AAD, the XP ERG provides a convenient opportunity to discuss difficult clinical problems and share the successes, hurdles and failures of their patients. By providing an accessible venue, dermatologists caring for patients can bring their challenging clinical problems to share with experts familiar with XP.

Knowledge that can be gained from understanding XP and studying its underlying mechanisms gives us powerful insights into knowledge of how skin cancer develops and can be prevented in the skin of individuals in the general population. The DNA repair mechanisms which are defective in these patients protect most of the general population from skin cancer. Understanding, managing, and controlling the skin cancer epidemic is an important AAD goal.

MTM: Who is involved in the XP-ERG?

Drs. DiGiovanna and Kraemer: We are the co-directors, and participants include dermatologists, trainees, researchers and patient advocates with an interest in XP and related disorders. Approximately 35 participants meet at the AAD Annual Meeting to discuss patient care and related issues. We continually seek additional participants with expertise, interest and issues related to these disorders.

MTM: What have been some of the group's accomplishments to date?

Drs. DiGiovanna and Kraemer: We provide information and avenues for patient diagnosis, cancer management, surveillance for neurological and other complications. We have brought together researchers with dermatologists who have patients that are difficult to manage and provide guidance and support. Participation by patient advocacy groups provides information about substantial resources available to leverage the expertise of the dermatologists managing these patients.

MTM: How do you share information with dermatologists and other healthcare professionals?

Drs. DiGiovanna and Kraemer: Throughout the year we take email or phone questions from, and network with, dermatologists, patients and other physicians to address relevant questions and discuss management issues.

MTM: How can Academy members get involved in the XP-ERG?

Drs. DiGiovanna and Kraemer: Academy members following patients or with an interest are encouraged to contact Dr. DiGiovanna (jdg@nih.gov) or Dr. Kraemer (kraemerk@nih.gov) to be added to the mailing list. We would welcome their participation in next year’s XP-ERG meeting at the Annual Meeting of the AAD.