Immunodeficiency

What is immunodeficiency?

Immunodeficiency can be caused by inherited syndromes, infections, medications, medical conditions, pregnancy, aging, and many other factors. Immunodeficiency is defined as the improper functioning of the immune system. Gravity varies and leads to different types of infection. It can occur at multiple points through the immune response. So it can lead to:

• Serious bacterial infections (eg. Staphylococcus aureus)
• Severe fungal infection (eg, oral yeast infection)
• Persistent viral infections (eg, extensive molluscum contagiosum)
• Do not thrive in babies and children.
• Infections due to unusual or opportunistic. organisms
• Delayed recovery from illness.
• Non infectious granulomatous skin disorders
• Certain types of cancer such as Epstein Barr virus associated B cells lymphoma, Merkel cell associated with polyomavirus carcinoma and Kaposi associated with the human herpes virus 8 sarcoma.

Immunodeficient people who feel bad should be carefully evaluated, and the infection should be treated aggressively.

Primary immunodeficiency diseases

Primary immunodeficiency diseases are present from birth and persist throughout life. They are due to infrequent or rare genetic defects, and there is often a family history of the condition. The World Health Organization lists 185 different primary immunodeficiency diseases. They include:

• Common variable immune deficiency
• X-linked agammaglobulinemia
• Selective IgA deficiency
• Severe combined immune deficiency
• Chronic granulomatous disease
• Wiskott-Aldrich syndrome
• Hyper-IgM syndrome
• DiGeorge syndrome
• IgG subclass and specific deficiency antibody deficiency
• Ataxia telangiectasia
• Hyper-IgE syndrome
• Complement deficiencies
• Interleukin 7 deficiency (predisposes to viral warts)

Acquired immunodeficiency

The most well-known secondary or acquired immunodeficiency disease is due to infection with the human immunodeficiency virus (HIV) that leads to AIDS (acquired immunodeficiency syndrome).

Human T cell Lymphotropic virus type 1 (HTLV-1) also leads to immunodeficiency.

Cancers, particularly blood disorders that affect white blood cells such as leukemia and lymphoma, can lead to severe immune deficiency.

Drug immunosuppression

Immunosuppression is a general buffering of the immune response, generally in response to immunosuppressive drugs. The immune response is still mounted, but it is not as efficient or effective.

Immunosuppressive medications include:

• Systemic corticosteroids (for children, prednisone> 2 mg / kg / day for two weeks or> 1 mg / kg / day for> four weeks), which suppress T lymphocytes via TH1, TH2 and TH17
• High doses of methotrexate
• Cyclosporine
• Azathioprine
• Mycophenolate
• Cyclophosphamide
• Biological agents

Lower doses or shorter cycles of systemic steroids are not considered to be significantly immunosuppressive. The low dose of methotrexate used for skin conditions mainly acts as an antiinflammatory drug and is only weakly immunosuppressive.

How are skin infections related to immunodeficiency?

Skin infections become more common as the amount of CD4 + increases. T lymphocytes reduces.

Slight reduction in CD4 + T-lymphocyte The count (> 200) is part of normal aging. This leads to an increase incidence of Herpes zoster (shingles) and in countries where it is predominant, classic or old world Kaposi's sarcoma.

Moderate reduction on CD4 + T lymphocytes The count (200-50) occurs in some elderly patients, newborns, and in primary immunodeficiency diseases. Deficient T lymphocytes tend to produce the TH2 class of cytokines, which lead to improvement allergic reactions characterized by eosinophilic Infiltrations These include:

• Atopic eczema with staphylococcal infection
• Improved and persistent insect bite reactions, often with blisters
• Eosinophilic folliculitis
• Transient acantholytic skin disease (Grover's disease)
• Prurigo (itchy red bumps)
• Eosinophilic cellulitis
• Hypereosinophilic syndrome
• Eosinophilic disorder of myeloproliferative disease.

Infections can include extensive viral warts and crusted scabies.

Severe reduction in CD4 + T lymphocyte count (<50) generalmente indica infección con VIH. Conduce a manifestaciones inusuales de infección e infección con organismos que normalmente son inofensivos en individuos sanos. Parece que el sistema inmune tolera la presencia de organismos que normalmente lucha para erradicar la piel. Ejemplos incluyen:

• Warty or ectimatous forms of herpes zoster (CD4 <25),
• Bacillary angiomatosis
• Cryptococcosis
• Kaposi's sarcoma associated with HIV
• Hairy leukoplakia

What tests should be done for immunodeficiency or immunosuppression?

Because the onset of skin conditions is often different in immunosuppressed individuals compared to normal conditions, unusual conditions and infections should be considered. The tests include repeated:

• Swabs for viruses and bacteria culture
• Scrapings for mycology
• Biopsies for histopathology and microbiological cultures.