Aplasia cutis congenital is a rare congenital absence of skin, exceptionally involves extremities. Most cases are sporadic but familial autosomal dominant pattern can be found. The etiology remains unclear, both genetic and environmental causes have been implicated. This lesion has primarily a clinical diagnosis. Imaging studies may be used to evaluate underlying tissues and investigate other comorbid abnormalities. Treatment modalities depend upon the extent defect and involvement of underlying tissues. Genetic counseling is useful in cases of hereditary form. We report a new case of extensive bilateral aplasia cutis congenital of extremities in a new born, occurred as an isolated sporadic case without family history, no teratogenic medication and no evidence for an amniotic band or infection in pregnancy.
Kasabach Merritt syndrome is defined by clinical and biological association of a purplish-red vascular tumor rapidly extensive, thrombocytopenia, a variable degree of disseminated intravascular coagulation and sometimes anemia. It usually occurs in neonates and infants less than 6 months, sometimes in utero. The imaging can confirm the diagnosis and search for deep locations. This syndrome can be life-threatening by the risk of bleeding, coagulopathy, thrombosis, compression and heart failure. The therapeutic management should be early, multidisciplinary adapting therapeutic benefits to risks in order to improve the prognosis. We report a case of Kasabach Merritt syndrome, we insist through a literature review of diagnostic, therapeutic and evolutionary difficulties.
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