Progressive, persistent, hyperkeratotic lesions in incontinentia pigmenti

Bessems, P. J.

doi:10.1001/archderm.124.1.29

Cited by 10 publications

(5 citation statements)

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“…In only few cases has an appearance after the first 2 months been noted, whereas the inflammatory stages can already develop in utero so that at birth only whirled hyper pigmentation is seen [3][4][5][6][7][8], The persistence of inflamma tory activity into adult life has been described as well as progressive, persistent hyperkeratotic lesions in inconti nentia pigmenti [9,10], Also the appearance of warty sub ungual lesions in combination with bone lesions has been documented in a young man 111],…”

Section: Discussionmentioning

confidence: 99%

Recurrent Inflammation in Incontinentia pigmenti of a Seven-Year-Old Child

Pfau¹,

Landthaler²

1995

Dermatology

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A young girl has undergone the typical skin manifestations of incontinentia pigmenti, leaving now only discrete hyperpigmentation on the flexor sides of the lower extremities. Each time when she has a feverish infection, a recurrence of inflammation can be seen within the hypeφigmented areas. At the same time when the fever subsides, the acute skin manifestations disappear as well. The combination of recurrent inflammatory skin manifestations within the hypepigmented skin areas of incontinentia pigmenti and fever is unique. The reasons for this phenomenon and for its strict limitation to the areas of incontinentia pigmenti remain unclear.

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Section: Discussionmentioning

confidence: 99%

Recurrent Inflammation in Incontinentia pigmenti of a Seven-Year-Old Child

Pfau¹,

Landthaler²

1995

Dermatology

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“…15 The frequency of such late recurrences, sometimes several years after the neonatal period, remains unknown. [16][17][18][19] Thus, a diagnosis of IP could be considered in the case of a child presenting with recurrent inflammatory lesions of unknown origin along the Blaschko lines. The frequency of stage 2 verrucous and keratotic lesions in our series was consistent with published data (80% vs 70%, Figure 1B), as well as the lesions' time of appearance (within the first 2 months of life) and duration (they cleared within 6 months).…”

Section: Commentmentioning

confidence: 99%

Clinical Study of 40 Cases of Incontinentia Pigmenti

Hadj‐Rabia

Froidevaux²,

Bodak³

et al. 2003

Arch Dermatol

167

173

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“…More recently, the case of an 8-month-old child with an extensive vesiculobullous rash was reported by Van Leeuwen et al, 7 but the existence of a fever in the preceding days was not mentioned. Bessems et al 8 described a 20-year-old patient who developed, from the age of 11 years, exacerbations of inflammatory lesions accompanied by persistent hyperkeratotic and verrucous lesions restricted to the hyperpigmented linear areas. No triggering factor was noted.…”

Section: Commentmentioning

confidence: 99%

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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Progressive, persistent, hyperkeratotic lesions in incontinentia pigmenti

Cited by 10 publications

References 0 publications

Recurrent Inflammation in Incontinentia pigmenti of a Seven-Year-Old Child

Recurrent Inflammation in Incontinentia pigmenti of a Seven-Year-Old Child

Clinical Study of 40 Cases of Incontinentia Pigmenti

Late Recurrence of Inflammatory First-Stage Lesions in Incontinentia Pigmenti

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