Human immunodeficiency virus disease and immunodeficiency syndromes
Immunodeficiency results from absence or failure of one or more elements of the immune system. It may be acquired, e.g. acquired immune deficiency syndrome (AIDS), or inherited, e.g. chronic mucocutaneous candidiasis.
Human immunodeficiency virus (HIV) disease
Infection with HIV is a progressive process that mostly leads to the development of AIDS.
Clinical presentation
As the disease progresses, the number of CD4+ lymphocytes falls and, when the blood count is below 50 cells/mL in the late phase of HIV infection (AIDS), M. avium complex infection, lymphoma and encephalopathy may develop. The mean latent period between infection and the development of AIDS is 10 years. Skin signs include the following (Table 1):
Dry skin. Skin dryness, often with asteatotic eczema, and seborrhoeic dermatitis (Fig. 1) are common and early findings. Their severity increases as the disease advances.
Fungal and papillomavirus infections. Tinea infections and perianal and common viral warts are seen in early disease.
Acne and folliculitis. These worsen in early and mid-stage disease.
Other infections. Oral candidiasis, oral hairy leukoplakia (thought to be associated with Epstein–Barr virus; Fig. 2) and infection with herpes simplex, herpes zoster, molluscum contagiosum and Staphylococcus aureus are increased in advanced disease.
Other dermatoses. Drug eruptions, hyperpigmentation and basal cell carcinomas are more common; psoriasis can get worse and syphilis may coexist.
Kaposi’s sarcoma. Kaposi’s sarcoma is a multicentric tumour of vascular endothelium seen in a third of patients with AIDS or AIDS-related complex, particularly in male homosexuals. It presents as purplish nodules or macules on the face, limbs, trunk or in the mouth (Fig. 3), but often also involves the internal organs and lymph nodes. Kaposi’s sarcoma is due to co-infection with herpes virus 8. A more benign sporadic form, seen in elderly East European Jewish men, is not associated with HIV.
Lymphoma. Lymphomas seen with late-phase HIV infection are often extranodal and sometimes cutaneous.