Darier’s disease, also known as keratosis follicularis, is a rare autosomal dominant genodermatosis characterized by greasy hyperkeratotic papules, nail abnormalities, and mucous membrane changes.¹ Few cases have been reported during pregnancy² and, to the best of our knowledge, the anesthetic management of such a patient has never been reported. Here, we report the anesthetic management for cesarean section in a parturient with Darier’s disease.

A 26-year-old primigravida at term presented at our hospital in early labor. She was booked for cesarean section in view of cephalopelvic disproportion. Physical examination revealed brownish, greasy and malodorous warty lesions all over her body and nail beds (Figures 1 and 2). She had had such lesions for the last 14 years and complained of itching, for which she received irregular treatment with topical corticosteroids. Her mother also suffered from such lesions. No lesions were seen in the oral cavity. After dermatological consultation, she was diagnosed with Darier’s disease on the basis of histological examination of the lesions, which showed suprabasal acantholysis and overlying dyskeratosis. No evidence of secondary infection was noted on swabs taken from lesions on her back. The rest of the preanesthetic examination was unremarkable and hematological investigations were within normal limits.

Electrocardiogram, blood pressure and oxygen saturation were monitored, and 750 mL balanced saline was infused. The skin on the back was prepared using cetrimide, chlorhexidine and isopropyl alcohol. Through a lesion-free area, subarachnoid block was performed with 10 mg hyperbaric bupivacaine (0.5%) at the L₃−L₄ interspace using a 27-G spinal needle. Sensory block was achieved up to the T₆ level. The intraoperative period was uneventful and the baby had normal Apgar score at birth. The patient was kept under observation for 2 days in the intensive care unit where she remained stable and did not show any signs of central nervous system infection. Subsequently, she was transferred to the postoperative care unit. She remained stable and was discharged from hospital after 5 days.

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Figure 1 Papular, greasy lesions on the patient’s chest.

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Figure 2 Warty lesions on the patient’s back, which showed suprabasal acantholysis and overlying dyskeratosis characteristic of Darier’s disease.

Mutations in the ATP2A2 gene located on 12q23- 24.1 causes Darier’s disease.³ It most commonly manifests from age 6 to 20 years; however, patients have presented as early as 4 years of age and as late as 70 years. Most patients have a family history of the disease. The first skin lesions typically occur in the teenage years and are frequently associated with pruritus. Although the severity fluctuates with time, Darier’s disease is a chronic, unremitting condition. There are various reports of aggravation and reduction in disease severity during pregnancy.^4,5 Involvement of mucous membranes is rare and does not compromise airway management.⁵ Oral antibiotics and acyclovir may be used to treat or suppress secondary bacterial or herpes simplex infection.⁶ Oral retinoid derivatives are often started in non-pregnant females; therefore, side effects like increased intracranial pressure, hypoplastic anemia, leukopenia and hypercalcemia should be looked for in such cases.⁵ Since the lesions are non-infectious, we postulate that spinal anesthesia can be safely administered through lesion-free areas after ruling out secondary bacterial or viral infection.