Bloch-Sulzberger syndrome


in·con·ti·nen·ti·a pig·men·'ti

[MIM*146150, MIM*308300, and MIM*308310] a rare genodermatosis characterized by hyperpigmented lesions in linear, zebra stripe, and other bizarre configurations following the lines of Blaschko; occasionally accompanied by other developmental anomalies of the eyes, teeth, nails, skeleton, nails, heart. The dermatologic features involve four stages: stage I is characterized by erythema, vesicles, and pustules; stage II by papules, verrucous lesions, and hyperkeratosis; stage III by hyperpigmentation; and stage IV by pallor, atrophy, and scarring. Historically, there were thought to be two forms: the sporadic type of incontinentia pigmenti (IP1), which is now known to be hypomelanosis of Ito and the familial type (IP2), which is X-linked dominant and a genetic lethal in males.
See also: hypomelanosis of Ito. Synonym(s): Bloch-Sulzberger disease, Bloch-Sulzberger syndrome

Bloch-Sulz·ber·ger syn·drome

(blok-sults'ber-ger sin'drōm) Variously patterned hyperpigmented lesions after development of bullous verrucous skin lesions; associated with many conditions and has onset at birth or during early infancy. Death usually follows soon thereafter; X-linked inheritance.

Bloch,

Bruno, Swiss dermatologist, 1878-1933. Bloch-Sulzberger disease - genodermatosis that may also involve other structures. Synonym(s): Asboe-Hansen disease; Bloch-Sulzberger syndrome; incontinentia pigmentiBloch-Sulzberger syndrome - Synonym(s): Bloch-Sulzberger disease

Sulzberger,

Marion Baldur, U.S. dermatologist, 1895-1983. Bloch-Sulzberger disease - see under Bloch, BrunoBloch-Sulzberger syndrome - Synonym(s): Bloch-Sulzberger diseaseSulzberger-Garbe disease - resembles an exudative form of eczema described in Jewish males with oval lesions on the penis, trunk, and face. Synonym(s): Sulzberger-Garbe syndromeSulzberger-Garbe syndrome - Synonym(s): Sulzberger-Garbe disease

Bloch-Sulz·ber·ger syn·drome

(blok-sults'ber-ger sin'drōm) Finding of patterned hyperpigmented lesions after development of bullous verrucous skin lesions.