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Correspondence: Address to Julia S. Lehman, MD, Department of Laboratory Medicine and Pathology, and Department of Dermatology, Mayo Clinic, 200 First Street SW (Gonda Building, 16th floor), Rochester, MN 55905.
A 73-year-old man with a history of bilateral hip replacements developed a unilateral, asymptomatic skin eruption on his left buttock 3 months after a left hip revision. Over the ensuing 3 years, he noticed mild irritation with heat exposure. Hydrocortisone 1% cream provided no benefit. Skin examination revealed linear pink to brown papules with overlying scale, which did not involve his previous surgical scar site (Figure 1). The clinical differential diagnosis included linear lichen planus, lichen striatus, persistent postherpetic granulomatous skin eruption, or other linear skin entities. Punch biopsy showed acantholytic dyskeratosis. A diagnosis of segmental Darier disease (DD) was made (Figure 2).
Figure 1Linear, papular rash on the left buttock near his previous surgical scar.
Figure 2Histopathology of the linear rash showed (A) focal acantholysis (black arrows) within the epidermis. B, Acantholysis is occurring just above the basal layer of the epidermis (yellow arrow). There is dyskeratosis with characteristic “corp ronds” (long black arrow) and “grains” (short black arrow). (Hematoxylin and eosin stain [A] 10x original magnification, [B] 20x original magnification.)
DD, a disease of keratinocyte adhesion, presents with grouped keratotic papules that are aggravated by heat and sweating and tends to develop during the third to fourth decades.
DD affects the body symmetrically. Segmental DD, however, presents with lesions arranged along the lines of Blaschko (segmental distribution). Diagnosis may be delayed because of the rarity of this entity.
DD is an autosomal dominant genodermatosis caused by germline mutations in the ATP2A2 gene, which causes haploinsufficiency of epidermal calcium ATPase.
Segmental DD is thought to be caused by genetic mosaicism of the same gene. This mutation leads to keratinocyte discohesion and acantholytic dyskeratosis (rounding up and separation of individual keratinocytes, with accompanying abnormal keratinization), features that can be observed microscopically with skin biopsy (Figure 2).
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