Skin issues and infections in patients with CGD

Recently, we asked the chronic granulomatous disease (CGD) community to share their experiences with skin issues or infections, and we received many responses. Patients and caregivers described their battles managing mild to severe acne, boils, lupus-related rashes, and photosensitivity. Out of all the patients who responded, the most common response concerned acne. For those with severe, cystic acne, many found accutane to be helpful. Patients with milder forms of acne opted for antibiotics, such as oxytetracycline, metronidazole, and amoxicillin/clavulanate. Meanwhile, other patients said they found success with natural, over-the-counter treatments, such as tea tree oil and frankincense oil.

To find out more about skin infections and recommended treatments for patients with CGD, we asked an expert on CGD, Harry Malech, MD, Chief, Laboratory of Host Defenses, National Institutes of Health. Here’s what he said:

Acne

CGD does not cause acne, nor is it more common in CGD than in the general population. However, for those with a predisposition to have severe acne affecting face, chest, and back, it does seem that the acne is more likely to become infected and cause scarring. In addition, severe acne in CGD patients may, in a few patients, persist into adulthood (even into their early 30’s). Accutane is often recommended for treating severe acne in CGD, which for the most part is well tolerated by CGD patients. Antibiotics may be required as well for infected cysts, though the specific antibiotics should be guided by cultures and consultation with infectious disease experts in addition to the dermatologist.

Hidradenitis

 CGD does not cause hidradenitis (severe recurrent infections with abscess formation and scarring in the axilla [armpits]). As with acne, while CGD is not the cause, if one has the predisposition to get hidradenitis, it is made worse by CGD. Antibiotics and drainage are the mainstays, but severe hidradenitis may require surgical removal of the skin glands in the affected site and usually provides relief of the condition. Hidradenitis in non-CGD patients can be treated with anti-TNF agents, such as infliximab or Humira, but there are some concerns that these agents may increase infection risk in CGD.

Cutaneous lupus

CGD patients (and female carriers of the X-linked form of CGD) are at risk of cutaneous lupus. Many patients respond well to Plaquenil, but some may also need steroids for control of the rashes and other symptoms.

Photosensitivity from voriconazole

Voriconazole is a useful agent for the treatment of Aspergillus and other fungal infections. However, it is a very strong photosensitizing agent for the skin. CGD patients taking voriconazole must avoid direct sunshine on their skin. Even strong sunscreens do not protect from this photosensitivity reaction, which occurs from the visible spectrum of light and not just the ultraviolet portion of the sunlight. Avoiding much direct sunlight is best or switching to another antifungal that does not cause photosensitivity is another option.

Special thanks to Dr. Malech for giving us these helpful insights. Thank you to all who shared their experiences and questions.