Understanding Skin Conditions Among Patients with Primary Immunodeficiencies

Patients with primary immunodeficiencies (PID) are more likely to get recurrent, unusual, prolonged, or severe skin infections, as well as noncutaneous signs such as eczema, erythroderma, granulomas, urticaria, or vasculitis. Knowledge of these common cutaneous manifestations among patients with PID may provide a better outline to health care practitioners to provide timely diagnosis and treatment options, according to Fibin Thanveer, MBBS, DDVL, MD, writing in the Journal of Skin and Sexually Transmitted Diseases.

Although individual immunodeficiency syndromes are rare, PIDs as a whole are not uncommon.

“A health survey in people of all ages in the United States reported a population prevalence of 1 in 1200 of diagnosed PID,” Thanveer stated. “Abnormalities in humoral immunity account for more than 50% of PIDs.” When the immune system is compromised, patients become more prone to infections involving the skin.

Shedding light on the study headed by Berron-Ruiz et al., out of the 90 patients with PID, 69% had skin infections, 29% had eczema-dermatitis, and 44% had other associated cutaneous conditions. Another study by Al-Herz et al. showed evidence that skin infections (30%) were the most prevalent cutaneous manifestation, followed by eczema (19%), erythroderma (7%), diffuse alopecia (7%), telangiectasia (6%), and autoimmune skin diseases (6%).

Fungal infections are to watch out for, Dr Thanveer said. Although it is more common to see acquired immunodeficiencies manifesting in unusual fungal infections, recent times have shown that an increasing number of PIDs are being reported as the underlying cause for fungal infections.

Compared to bacterial and fungal skin infections, viral skin infections are considered less common manifestations among patients with PID. “Recurrent, severe herpes simplex or herpes zoster infections; extensive and persistent infections with molluscum contagiosum; and human papillomavirus infections along with other features have been described in DOCK8 deficiency but not commonly in AD-HIES,” Dr Thanveer said.

Compared to bacterial and fungal skin infections, viral skin infections are among the less common manifestations in PIDs.

A single cutaneous manifestation is not a sole indicator of a primary immune deficiency disease. However, a series of events over time is a fairly good diagnostic of an underlying PID. Acting upon such suspicions may help make better and timely decisions regarding diagnosis and treatment plans among patients with primary immune deficiency disease.

—Priyam Vora

Reference:
Thanveer F. Cutaneous manifestations in primary immunodeficiency diseases. J Skin Sex Transm Dis. 2021;3(2):143-150. DOI: 10.25259/JSSTD_48_2020