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Presented by Dept of Dermatopathology and prepared by Marc Lewin, M.D.
Case 1: Erythematous papules and vesicles on the leg of a 6week old female.
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Question 1 of 1
1. Question
Week 307: Case 1
Erythematous papules and vesicles on the leg of a 6week old femaleimages/4-10-07case01a.jpg
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images/4-10-07case01d.jpgCorrect
Answer: Incontinentia Pigmenti
Histology: none provided
Discussion: Incontinentia pigmenti is an uncommon genodermatosis. It is a chromosomal instability disorder which is inherited in an X-linked dominant fashion and is linked to the Xp11.21 and Xq28. It is believed that affected males usually die in utero.
The first stage of incontentia pigmenti shows a spongiotic eosinophilic dermatitis. The presence of dyskerytotic cells in addition to this eosinophilic spongiosis is a rather specific finding and helps to rule out other causes of eosinophilic spongiosis such as contact dermatitis or arthropod bite reaction. Later, a verrucous stage develops with hyperkeratosis, acanthosis, and increased dyskeratotic cells.
Incorrect
Answer: Incontinentia Pigmenti
Histology: none provided
Discussion: Incontinentia pigmenti is an uncommon genodermatosis. It is a chromosomal instability disorder which is inherited in an X-linked dominant fashion and is linked to the Xp11.21 and Xq28. It is believed that affected males usually die in utero.
The first stage of incontentia pigmenti shows a spongiotic eosinophilic dermatitis. The presence of dyskerytotic cells in addition to this eosinophilic spongiosis is a rather specific finding and helps to rule out other causes of eosinophilic spongiosis such as contact dermatitis or arthropod bite reaction. Later, a verrucous stage develops with hyperkeratosis, acanthosis, and increased dyskeratotic cells.
