Bloch Sulzberger

Bloch Sulzberger Syndroom

Figure 1.183. Incontinentia pigmenti (Bloch-Sulzberger syndrome) is considered to be a neurocutaneous syndrome seen only in female infants (X-linked dominant, generally lethal prenatally to the male). It has four cutaneous phases. In this infant note the early maculopapular appearance without vesicles or pigmentation. The lesions contain eosinophils, and blood eosinophilia is frequently present. In the next phase there are many vesicular lesions. These then become bullous and pustular. The bullae may be superseded by verrucous lesions in the same distribution, and after these disappear, whorls of hyperpigmentation appear. In the final phase, hypopigmented patches may occur. The patterning of the skin abnormalities follows the path of Blaschko's lines, which represent the course of early migration of primordial skin cells progressing from the dorsal to the ventral midline.

Figure 1.184. Within the first month of life in incontinentia pigmenti, the vesicular phase develops as noted in this infant. Note the linearly arranged vesicles and red nodules on the flexor surface of the upper and lower extremities. The blisters may cluster in a bizarre arrangement if they are numerous.

Figure 1.184. Within the first month of life in incontinentia pigmenti, the vesicular phase develops as noted in this infant. Note the linearly arranged vesicles and red nodules on the flexor surface of the upper and lower extremities. The blisters may cluster in a bizarre arrangement if they are numerous.

Incontinentia Pigmenti

Figure 1.185. Another infant with incontinentia pigmenti. Note the maculovesicular pigmented appearance of the skin in a swirling pattern. The infant developed seizures on the 2nd day of life. Systemic manifestations occur in about 70% of infants (20% at birth). These include central nervous system (microcephaly, seizures, and retardation), ocular (cataracts, retinal dysplasia, etc.), osseous (hemi vertebrae, extra ribs, hemiatrophy, etc.), dental (hypodontia, etc.), and occasionally cardiac abnormalities.

Maculovesicular
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Responses

  • Denita Walker
    My grand daughter was recently born with IP and has already suffered a stroke. I am trying to find any information to educate myself and her MD's. Has there been any determination as to what causes the strokes? I have read small vessels on one website and neuroectodermal tissue in the vessels of the brain in another. One said treat with ASA very tiny doses and the other with steriods....Please send any information you have on the disease process.<br />Thanks for your help!<br />Denita Walker
    7 years ago

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