Wiscott-Aldritch, Acrodermatitis enteropathica, ataxia telangiectasia, atopic dermatitis, Langerhans cell histiocytosis (LCH)
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,
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,
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).
Hong R. Combined immunodeficiency diseases. In: McMillan JA,
DeAngelis CD, Feigin RD, Warshaw JB, eds. Oski’s Pediatrics:
Principles and Practice. 3rd ed. Philadelphia, Pa: Lippincott
Williams & Wilkins; 1999:2092-2099
Weston WL, Lane AT, Morelli JG. Color Textbook of Pediatric
Dermatology. 2nd ed. St Louis, Mo: Mosby, Inc; 1996:200-202, 313-314