Wiscott-Aldritch, Acrodermatitis enteropathica, ataxia telangiectasia, atopic dermatitis, Langerhans cell histiocytosis (LCH)



Ataxia Telangiectasia

Patients who have Ataxia telangiectasia (an autosomal recessive disorder) may be distinguished from those who have WAS by the presence of severe cerebellar ataxia and telangiectasias involving the conjunctivae and skin. Other cutaneous manifestations include vitiligo, café au lait spots, and premature graying of the hair.

Although the lesions of atopic dermatitis are analogous to those seen in WAS, patients who have the latter disorder often exhibit petechiae and the associated abnormalities discussed previously.

Langerhans cell histiocytosis (LCH, see Histiocytosis) is the term applied to a group of disorders characterized by the accumulation of histiocytes in the skin or other organs. Unlike the lesions of WAS, those of LCH are erythematous, orange or yellow-brown papules or nodules. Individual lesions may exhibit scale or petechiae or may become purpuric. Affected individuals may present with skin disease alone or systemic complications (eg, bone pain or swelling, diabetes insipidus).

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