It was found that 75% of patients stabilize or improve over several months as opposed to 25% of patients who do not respond and have progressive disease. But despite initial improvement, most patients survive only a few months. Breast cancer and small cell lung cancer are the two solid tumors that respond best to treatment Some patients do better than others, particularly those whose primary cancer is hematologic, bone marrow and lymph nodes. Factors that lower survival
Much of prognosis can be determined from the damage due to primary cancer. Negative hormone receptor status, poor performance status, more than 3 chemotherapy regimes, and high Cyfra 21-1 level at diagnosis, all indicates lower survival period of patients with NM. Cyfra 21-1 is a fragment of the cytokeratin 19 and may reflect the tumor burden within the CSF. Epidemiology
In the United States, 1–8% of cancer patients are diagnosed with leptomeningeal disease, with approximately 110,000 cases per year. The exact incidence of leptomeningeal disease is difficult to determine, since gross examination at autopsy may overlook signs of leptomeningeal disease, and microscopic pathological inspection may be normal if the seeding is multifocal or if an unaffected area of the central nervous system (CNS) is examined. Current research
New treatments and clinical trial for breast cancer patients and non-small cell lung cancer patients with leptomeningeal disease are currently being explored.People with leptomeningeal metastasis are generally excluded from clinical trials, thereby limiting the systematic assessment of novel therapies in this subgroup of patients with poor prognosis.
IgA pemphigus is a subtype of pemphigus with two distinct forms:

Subcorneal pustular dermatosis (also known as Sneddon–Wilkinson disease and pustulosis subcornealis) is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils. : 203  This is distinct from and not to be confused with subcorneal pustular dermatosis type of IgA pemphigus. Sneddons syndrome, also known as Ehrmann-Sneddon syndrome, is also a different syndrome. Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis. : 465

History
Early descriptions were made by Darrell Wilkinson, a British dermatologist. See also
Pemphigus
List of cutaneous conditions
List of conditions caused by problems with junctional proteins
List of immunofluorescence findings for autoimmune bullous conditions

References


== External links ==