Schwachman syndrome.. Neutropenia due to lack of external pancreatic secretions. Ischemic cartilage dysplasia. Ectopic spleen rash with thrombocytopenia

Schwachman syndrome:
This syndrome may be inherited as an autosomal recessive form characterized by:
- Sharing malabsorption and growth failure.
- Lack of neutrophils due to lack of external pancreatic secretions.
- Ischemic cartilage dysplasia, tends to cause special abnormalities in the head of the injured thigh.
Clinical manifestations:
Skin manifestations:
The presence of symmetric euchemia rash is sometimes accompanied by an eczema-like thrombocytopenia similar to that of the ischemic diuretic syndrome.
The cysts may be many and extended.
General features:
Neutropenia is an important component of the syndrome and may not appear during the first years of life.
- Thrombocytopenia may also occur and may lead to freckles or bleeding.
Frequent respiratory tract infections are sometimes serious.
Treatment:
- An effective pancreatic conclusion.
- Treatment of systemic skin infections.
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