Incontinentia Pigmenti (Bloch-Sulzberger Syndrome)

Morgan, James D.

doi:10.1001/archpedi.1971.02110040078005

Cited by 28 publications

(3 citation statements)

References 20 publications

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“…Herpes simplex virus was demonstrated in the fluid from vesicles in another infant girl (Palmgren, 1962). However, in most reported cases--including ours--tests for serum viral antibodies have been negative, as have stool or CSF cultures for virus (O'Doherty and Norman, 1968;Morgan, 1971). Thus there is no conclusive evidence that I.p.…”

Section: Discussionmentioning

confidence: 74%

“…p. there is eosinophilia of the peripheral blood and vescicle fluid. Pruritic flare-ups and swelling of erythematous lesions can appear (Epstein et al, 1952;Morgan, 1971), and in one child bullous eruptions developed after a diphtheria-tetanus toxoid-pertussis vaccine injection (Morgan, 1971). According to Ubel and Korting (1950) and Epstein et al (1952), the perivascular foci of inflammation affecting especially the deeper arteries, and certain degenerative changes point to hypersensitivity reactions.…”

Section: Discussionmentioning

confidence: 99%

“…However, the various stages may coexist or may not always appear in each patient. It seems possible that the earlier inflammatory stages have appeared in utero and that after birth only the stage of pigmentation is visible (Morgan, 1971;Ebbing and Rook, 1972).…”

Section: Introductionmentioning

confidence: 99%

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Encephalitis in two members of a family with Incontinentia pigmenti (Bloch-Sulzberger syndrome)

et al. 1978

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Abstract. The Bloch-Sulzberger syndrome of Incontinentia pigmenti (I. p.) was observed in 3 generations of one family. The grandmother and mother of 3 sisters with I.p. showed solely the typical skin lesions and no associated ectodermal abnormalities.At the age of 2 years the eldest girl with I.p. suffered from encephalitis after vaccination against smallpox. Four months later, having fully recovered, the patient suddenly deteriorated and developed generalized seizures and signs of increased intracranial pressure. She died 12h after right temporal decompression. Neuropathological examination of the brain revealed acute hemorrhagic encephalopathy involving predominantly the white matter and leading to massive edema. In addition, there were residual signs of a perivenous encephalitis in the central and subcortical white matter of the forebrain. Signs of malformation or developmental abnormalities were absent.The second girl with I.p. experienced an afebrile convulsion 3 weeks after the first injection of diphtheria and tetanus toxoid. At the age of 2~4 years, one week after an upper respiratory tract infection, she developed an encephalitis which resulted in severe neurological sequelae.The third girl with I. p. suffered from an afebrile convulsion without proof of encephalitis at the age of 4 months.The pathogenesis of the CNS disorders which are frequently associated with I. p. is not yet known, but the history of neurologic lesions in this family suggests that inflammatory processes may play an important role in their evolution.

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Section: Discussionmentioning

confidence: 74%