Darier’s Disease in Pregnancy

Authors: Michelle D. Sim, MD; Maricarr Pamela Lacuesta, MD, FPDS

Abstract

Introduction: Darier’s disease is a rare autosomal dominant disease which can affect both sexes and all ethnic groups. Literature search has revealed limited reports of Darier’s disease among pregnant women.

Case report: A 24 year old, primigravid at 32 weeks AOG, was seen in our clinic due to four months’ history of multiple pruritic, erythematous to brown greasy papules over an intensely erythematous base noted mostly on seborrheic areas of the face, neck, chest, and back. Erythema was noted to be more pronounced after sun exposure. Nail findings included nail fragility, longitundinal erythronychia and distal onycholysis.  Skin punch biopsy was done on lesions from sun-exposed and non-sun-exposed areas. Both specimens showed focal parakeratosis overlying an acanthotic epidermis. Foci of suprabasal acantholysis with dyskeratotic keratinocytes forming “corps ronds” and “corps grains” were also noted while the dermis revealed perivascular predominantly lymphocytic inflammatory infiltrates. The clinical presentation and histopathologic findings were consistent with Darier’s disease. Patient was treated with urea cream, mild emollients and photoprotection which afforded improvement after two weeks.  Furthermore, the patient was given pre-natal counseling regarding possible complications during delivery.

Conclusion: Darier’s disease may have pregnancy-specific implications. Histological skin changes may predispose the patient’s skin to infection through barrier thinning. The condition may also cause pregnancy complications since it can affect skin elasticity which is important for atraumatic vaginal birth. Therefore, prompt diagnosis is vital so patients can be well-informed about possible mode of delivery and proper skin care to maintain skin integrity and avoid opportunistic infections.

 

 

Citation

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Keywords

Darier’s disease, pregnancy, corps ronds and corps grains, longitudinal erythronychia

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