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Skin-nontumor / Clinical Dermatology

Blistering disorders

Epidermolytic hyperkeratosis

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 6 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.


● Autosomal dominant disorder with ichthyosis, rows of confluent, verrucous and scaly papules in flexural areas arising shortly after birth and persisting (eMedicine)
● Offensive odor, frequent cutaneous infections
● Due to defective keratin 1 and 10
● Up to 50% of cases may represent new mutations (eMedicine)


● Also called bullous congenital ichthyosiform erythroderma

Clinical images

Various images

Micro description

● Continuous involvement of the entire horizontal epidermis and focal involvement revealing skip areas of normal appearing epidermis along the horizontal epidermis (J Am Acad Dermatol 2008;59:86)
● Thickened granular layer with bizarre, distorted, basophilic keratohyaline granules (epidermolysis) and perinuclear clear spaces
● Stratum corneum is hyperkeratotic
● Also superficial lymphocytic infiltrate

Micro images

Various images


Differential diagnosis

● Focal epidermolytic hyperkeratosis (incidental) is common and described in normal skin, seborrheic keratosis, overlying scar and fibrous histiocytoma, in banal and dysplastic nevus, actinic keratosis, squamous cell carcinoma in situ and melanoma

End of Skin-nontumor / Clinical Dermatology > Blistering disorders > Epidermolytic hyperkeratosis

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