Incontinentia Pigmenti: An Extensive Second Episode of a “First‐Stage” Vesicobullous Eruption

Leeuwen, Robert L. van; Wintzen, Marjolein; Praag, M. C. G. Van

doi:10.1046/j.1525-1470.2000.017001070.x

Cited by 28 publications

(28 citation statements)

References 2 publications

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“…7,8,16,17 In our particular patient, lesions were always triggered by an infectious episode, sun exposure, or emotional stress. The frequency and significance of such late recurrences remain unknown.…”

Section: Discussionmentioning

confidence: 78%

“…Stage 1 and 2 lesions are usually absent, and persistent stage 3 lesions are often inconstant and discrete. [7][8][9][10][11][12] In adults, the clinical diagnosis of IP is principally based on the late onset of stage 4 lesions and their association with dental, nail, ocular, or central nervous system anomalies. 13,14 Even so, these lesions are often unrecognized.…”

mentioning

confidence: 99%

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Clinical and histologic features of incontinentia pigmenti in adults with nuclear factor-κB essential modulator gene mutations

Hadj‐Rabia

Rimella²,

Smahi

et al. 2011

Journal of the American Academy of Dermatology

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Section: Discussionmentioning

confidence: 78%

mentioning

confidence: 99%

Clinical and histologic features of incontinentia pigmenti in adults with nuclear factor-κB essential modulator gene mutations

Hadj‐Rabia

Rimella²,

Smahi

et al. 2011

Journal of the American Academy of Dermatology

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“…In the observation reported by Van Leeuwen et al 7 most of the recurrent inflammatory lesions occurred on the trunk, a site that was relatively spared during the first episode. Our first patient presented with lesions on the trunk.…”

Section: Commentmentioning

confidence: 87%

Late Recurrence of Inflammatory First-Stage Lesions in Incontinentia Pigmenti

Bodak¹,

Hadj‐Rabia²,

Hamel‐Teillac³

et al. 2003

Arch Dermatol

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Background: Incontinentia pigmenti (IP) is an Xlinked genodermatosis that is manifested by neonatal inflammatory vesicles localized along the lines of Blaschko. These lesions usually clear spontaneously within a few months, leaving hyperpigmentation. Ophthalmologic and neurologic symptoms can be associated with IP. Late recurrences of the first-stage inflammatory lesions after the initial rash are uncommon and have been reported infrequently. The mechanism involved in this phenomenon is unclear. However, the recent identification of NEMO/IKKγ as the gene responsible for IP sheds new light on its pathophysiologic origins.Observations: We report 5 cases of children who experienced episodes of late reactivation of IP. In all cases, the recurrences occurred on the previously hyperpigmented streaks several months or years after resolution of the initial eruptions. In most cases, the recurrences were preceded by an infectious episode.Conclusions: These IP recurrences suggest that mutated cells can persist a long time in the epidermis. We theorize that infections trigger the reactivations. The NEMO/IKKγ gene encodes a protein essential in nuclear factor B activation, which is required for resistance to tumor necrosis factor ␣-induced apoptosis. We discuss the role of a proinflammatory cytokine such as tumor necrosis factor ␣ as a triggering factor for the reactivation.

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“…This is something that has been indeed observed in few IP patients. 24,25 Anhidrotic ectodermal dysplasia with immunodeficiency (X-linked form)…”

Section: Incontinentia Pigmentimentioning

confidence: 99%

NF-κB-related genetic diseases

Courtois

Smahi

2006

Cell Death Differ

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The recent identification of genetic diseases (incontinentia pigmenti, anhidrotic ectodermal dysplasia with immunodeficiency and cylindromatosis) resulting from mutations affecting components of the nuclear factor-jB (NF-jB) signaling pathway provides a unique opportunity to understand the function of NF-jB in vivo. Besides confirming the importance of NF-jB in innate and acquired immunity or bone mass control, analysis of these diseases has uncovered new critical roles played by this transcription factor in the development and homeostasis of the epidermis and the proper function of lymphatic vessels. In addition, the identified mutations will help understanding at the molecular level how NF-jB is activated in response to cell stimulation.

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Incontinentia Pigmenti: An Extensive Second Episode of a “First‐Stage” Vesicobullous Eruption

Cited by 28 publications

References 2 publications

Clinical and histologic features of incontinentia pigmenti in adults with nuclear factor-κB essential modulator gene mutations

Clinical and histologic features of incontinentia pigmenti in adults with nuclear factor-κB essential modulator gene mutations

Late Recurrence of Inflammatory First-Stage Lesions in Incontinentia Pigmenti

NF-κB-related genetic diseases

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