INCONTINENTIA PIGMENTI, STAGE FOLLOWING VESICULATION
Note: See any dermatology or dermatopathology textbook for the clinical features of incontinentia pigmenti.

The vesicular stage of incontinentia pigmenti is characterized histologically by spongiotic vesicles that contain numerous eosinophilic leukocytes, and there are dyskeratotic cells within the epidermis. In the next stage, as represented by this case, spongiosis is minimal and the eosinophilic leukocytes are confined to the dermis. Dyskeratotic cells are found in all levels of the epidermis. A peculiar form of hyper/parakeratosis may be present.

Although dyskeratotic cells may be found in all levels of the epidermis in other conditions, notably erythema multiforme and pityriasis lichenoides et varioliformis acuta, the addition of  more than a few eosinophilic leukocytes is distinctive for incontinentia pigmenti. These conditions also have different clinical characteristics if the histological diagnosis is in doubt.


This young female infant developed linear and swirled papulosquamous lesions where there had been vesicles shortly after birth.
 
 
Low power view. The epidermis is minimally acanthotic. Focal hyper/parakeratosis is present.
Medium power view of above. Spongiosis is minimal. Dyskeratotic cells (apoptotic cells) are scattered within all levels of the epidermis.
Composite high power view of above. The bright red blobs are the apoptotic keratinocytes.
High power view from another area. Eosinophilic leukocytes are in the dermis.

Click on your browser's 'Back' button to return to the previous page.