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DermAtlas: LEG - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: Incontinentia_Pigmenti_3_080714   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: vesiculobullous eruptions /
genodermatosis/genetic disorder
Body Site: leg / buttock
thigh / abdomen
  Age: 13 days
Contributor: Charles Flores, MD    
Description: widespread linear and swirled red papules and vesicles
Comments: This 13-day-old girl was evaluated in the emergency room for a worsening rash. She was a full term baby delivered vaginally without complications. She appeared well, and vital signs were normal. She had widespread erythematous papules and vesicles distributed linearly on her legs and trunk as well as crusted vesicles on her scalp. There was no family medical history of skin disorders. Diagnosis was confirmed with a skin biopsy which revealed eosinophilic spongiosis.
Related Images: All related Images  Incontinentia_Pigmenti_1_080714  Incontinentia_Pigmenti_2_080714 

DermAtlas: SCALP - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: Incontinentia_Pigmenti_2_080714   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: vesiculobullous eruptions /
neonatal dermatology /
genodermatosis/genetic disorder
Body Site: scalp   Age: 13 days
Contributor: Charles Flores, MD    
Description: widespread linear and swirled red papules and vesicles
Comments: This 13-day-old girl was evaluated in the emergency room for a worsening rash. She was a full term baby delivered vaginally without complications. She appeared well, and vital signs were normal. She had widespread erythematous papules and vesicles distributed linearly on her legs and trunk as well as crusted vesicles on her scalp. There was no family medical history of skin disorders. Diagnosis was confirmed with a skin biopsy which revealed eosinophilic spongiosis.
Related Images: All related Images  Incontinentia_Pigmenti_1_080714  Incontinentia_Pigmenti_3_080714 

DermAtlas: LEG - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: Incontinentia_Pigmenti_1_080714   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: vesiculobullous eruptions /
Genetic Disorder /
genodermatosis/genetic disorder /
neonatal dermatology
Body Site: leg / thigh
abdomen / vulva (labia)
  Age: 13 days
Contributor: Charles Flores, MD    
Description: widespread linear and swirled red papules and vesicles
Comments: This 13-day-old girl was evaluated in the emergency room for a worsening rash. She was a full term baby delivered vaginally without complications. She appeared well, and vital signs were normal. She had widespread erythematous papules and vesicles distributed linearly on her legs and trunk as well as crusted vesicles on her scalp. There was no family medical history of skin disorders. Diagnosis was confirmed with a skin biopsy which revealed eosinophilic spongiosis.
Related Images: All related Images  Incontinentia_Pigmenti_2_080714  Incontinentia_Pigmenti_3_080714 

DermAtlas: LEG - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: Incontinentia_Pigmenti_2_070915   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: hyperpigmentation /
Genetic Disorder
Body Site: leg / total body
groin / diaper area
vulva (labia)
  Age: 2 months
Contributor: Oscar Lopez Flores, MD    
Description: asymmetric lines of Blaschko distributed hyperpigmented patches
Comments: This healthy 3-month-old girl had an urticarial and vesicular eruption shortly after birth before developing disseminated hyperpigmentation along lines of Blaschko. At 3 months of age her growth and development were normal.
Related Images: All related Images  Incontinentia_Pigmenti_1_070915  Incontinentia_Pigmenti_3_070915  Incontinentia_Pigmenti_4_070915 

DermAtlas: FACE - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: Incontinentia_Pigmenti_4_070915   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: hyperpigmentation /
Genetic Disorder
Body Site: face / total body
chest / axilla (armpit)
  Age: 2 months
Contributor: Oscar Lopez Flores, MD    
Description: asymmetric lines of Blaschko distributed hyperpigmented patches
Comments: This healthy 3-month-old girl had an urticarial and vesicular eruption shortly after birth before developing disseminated hyperpigmentation along lines of Blaschko. At 3 months of age her growth and development were normal.
Related Images: All related Images  Incontinentia_Pigmenti_1_070915  Incontinentia_Pigmenti_3_070915  Incontinentia_Pigmenti_2_070915 

DermAtlas: LEG - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: Incontinentia_Pigmenti_3_070915   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: hyperpigmentation /
Genetic Disorder
Body Site: leg / total body   Age: 2 months
Contributor: Oscar Lopez Flores, MD    
Description: asymmetric lines of Blaschko distributed hyperpigmented patches
Comments: This healthy 3-month-old girl had an urticarial and vesicular eruption shortly after birth before developing disseminated hyperpigmentation along lines of Blaschko. At 3 months of age her growth and development were normal.
Related Images: All related Images  Incontinentia_Pigmenti_1_070915  Incontinentia_Pigmenti_4_070915  Incontinentia_Pigmenti_2_070915 

DermAtlas: LEG - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: Incontinentia_Pigmenti_1_070915   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: hyperpigmentation /
Genetic Disorder
Body Site: leg / total body
diaper area / groin
vulva (labia)
  Age: 2 months
Contributor: Oscar Lopez Flores, MD    
Description: asymmetric lines of Blaschko distributed hyperpigmented patches
Comments: This healthy 3-month-old girl had an urticarial and vesicular eruption shortly after birth before developing disseminated hyperpigmentation along lines of Blaschko. At 3 months of age her growth and development were normal.
Related Images: All related Images  Incontinentia_Pigmenti_3_070915  Incontinentia_Pigmenti_4_070915  Incontinentia_Pigmenti_2_070915 

DermAtlas: ABDOMEN - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: incontinentia_pigmenti_1_061231   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: genodermatosis/genetic disorder /
linear eruptions /
Blaschko lines
Body Site: abdomen / chest
arm / leg
genital / vulva (labia)
  Age: 3 years
Contributor: Bernard Cohen, MD    
Description: asymmetric linear grayish brown slightly atrophic patches following the lines of Blaschko
Comments: This healthy 3-year-old girl developed urticarial vesicular papules and vesicles during the first 2 weeks of life. As the lesions evolved crusted papules followed and eventually resolved leaving atrophic linear hyperpigmented patches. Note her hypoplastic and malformed teeth.
Related Images: incontinentia_pigmenti_2_061231 

DermAtlas: ABDOMEN - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: incontinentia_pigmenti_2_061231   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: genodermatosis/genetic disorder /
linear eruptions /
Blaschko lines
Body Site: abdomen / chest
arm / leg
genital / vulva (labia)
  Age: 3 years
Contributor: Bernard Cohen, MD    
Description: asymmetric linear grayish brown slightly atrophic patches following the lines of Blaschko
Comments: This healthy 3-year-old girl developed urticarial vesicular papules and vesicles during the first 2 weeks of life. As the lesions evolved crusted papules followed and eventually resolved leaving atrophic linear hyperpigmented patches. Note her hypoplastic and malformed teeth.
Related Images: incontinentia_pigmenti_1_061231 

DermAtlas: Histology - incontinentia pigmenti
© 2001-2009, DermAtlas
Image Name: incontinentia_pigmenti_1_070501   File Type: jpg
Diagnosis: INCONTINENTIA PIGMENTI   Category: genodermatosis/genetic disorder /
vesiculobullous eruptions
Body Site: axilla (armpit) / groin
vulva (labia) / leg
  Age: 4 months
Contributor: Greg Hosler, MD, PhD    
Description: There is marked spongiosis with exocytosis of numerous cells. There are minimal changes to the stratum corneum.
Comments: This is a great case, a rare dermpath find outside of courses and study sets. This 4-month-old girl developed tiny vesicles over her axillae, inguinal region, vulva and legs, present since birth. Some had mild thickening. The biopsy was submitted with a differential diagnosis of incontinentia pigmenti (IP), dermatitis herpetiformis, or bullous impetigo. IP is X-linked with a defect in NEMO. The inheritence pattern explains why this is almost exclusively found in females. Of the classic 3 stages (spongiosis, verrucous, pigmentary), this biopsy was taken from the first. The histologic differential diagnosis would include allergic contact dermatitis, arthropod bite, and bullous pemphigoid, but the clinical history seals the diagnosis..great case!
Related Images: incontinentia_pigmenti_2_070501 

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© DermAtlas, Johns Hopkins University; 2000-2009
Bernard A. Cohen, MD, Christoph U. Lehmann, MD

DermAtlas was last updated: Oct-28-2009
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