Bloch-Sulzberg syndrome

Synonyms: Incontinentia pigmenti, melanoblastosis Bloch-Sulzberg, Siemens-Bloch-Sulzberg syndrome, melanoblastosis cutis linearis sive systematica, naevus pigmentosus systematicus.

Named after Swiss dermatologist Bruno Bloch and American dermatologist Marion Baldur Sulzberg.

Genetics:
 * OMIM: 308300
 * The disease is caused by a mutation in the gene of IKBKG (NEMO) in the Xq28 region.
 * Inheritance: X-linked dominant, male lethal, variable expression in females (due to random X-chromosome inactivation).

Characteristics: preview| 200 px|right| Deformation of teeth in a 3-year-old girl
 * cinnamon dirty symmetrical skin spots on the sides,
 * alopecia,
 * dystrophy nails,
 * defects of teeths,
 * corneal opacity,
 * microcephaly.