Alibert-Bazin Syndrome

A type of non-Hodgkin lymphoma, known more commonly as "Mycosis Fungoides" was formerly known as "Alibert-Bazin syndrome" from the French physician, Jean Lousi Alibert, who in 1806 was the first to describe a patient with mycosis fungoides. Mycosis fungoides (also known as Alibert-Bazin syndrome or granuloma fungoides), is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time.  The name mycosis fungoides is somewhat misleading–it loosely means "mushroom-like fungal disease". The disease, however, is not a fungal infection but rather a type of non-Hodgkin's lymphoma. It was so named because Alibert described the skin tumors of a severe case as having a mushroom-like appearance.

Origins and causes

The cause of mycosis fungoides is unknown, but it is not believed to be hereditary or genetic in the vast majority of cases. One incident has been reported of a possible genetic link. It is not contagious. It is rare for the disease to appear before age 20, and it appears to be noticeably more common in males than females, especially over the age of 50, where the incidence of the disease (the risk per person in the population) does increase. The average age of onset is between 45 and 55 years of age for patients with patch and plaque disease only, but is over 60 for patients who present with tumours, erythroderma (red skin) or a leukemic form (the Sézary syndrome). The disease is an unusual expression of CD4 T cells, a part of the immune system. These T cells are skin-associated, meaning that they biochemically and biologically are most related to the skin, in a dynamic manner. Mycosis fungoides is the most common type of 'Cutaneous T-cell Lymphoma' (CTCL), but there are many other types of CTCL that have nothing to do with mycosis fungoides and these disorders are treated differently.

Symptoms, diagnosis, and stages

Typical visible symptoms include rashlike patches, tumors, or lesions. Itching (pruritus) is common, perhaps in 20% of patients, and is not universal. Diagnosis is sometimes difficult because the early phases of the disease often resemble eczema or even psoriasis. As with any serious disease, it is advisable to pursue the opinion of a medical professional if a case is suspected. Diagnosis is generally accomplished through a skin biopsy. Several biopsies are recommended, to be more certain of the diagnosis. The diagnosis is made through a combination of the clinical picture and examination, and is confirmed by biopsy. To stage the disease, various tests may be ordered, to assess nodes, blood and internal organs, but most patients present with disease apparently confined to the skin, as patches (flat spots) and plaques (slightly raised or 'wrinkled' spots).
 

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