Case Presentation: We present a 70 year old Caucasian male with an established diagnosis of Darier’s disease (DD) since childhood. Our patient was recently diagnosed with Intraductal Papillary Mucinous Cystic Neoplasm of the pancreas and underwent a pancreatectomy, splenectomy with hepaticojejunostomy and Billroth II gastrojejunostomy. The stress of his recent surgery exacerbated his DD, and he presented to the hospital with debilitating pain and worsening skin lesions. The maculopapular lesions appeared bright red to skin colored, firm, greasy and were located around his gluteal clefts, back, scalp and forehead. The lesions in the gluteal region were most clinically relevant since they caused profound distress. He complained of severe physical deconditioning and had fallen a number of times prior to his admission. Despite having had recent abdominal surgery, he had no abdominal symptoms and his abdominal exam was benign. He did not have any systemic symptoms suggestive of infection. Blood cultures initially grew Streptococcus dysgalactiae followed by Pseudomonas aeruginosa a few days later. He was on multiple broad spectrum antibiotics which ultimately led to clearance of bacteremia. Dermatology was consulted and he was started on Acitretin for his DD. Our patient has had recurrent exacerbations of DD in the past, particularly after stressful events. Acitretin controlled symptoms only for a limited time. He has also had recurrent complications such as superimposed cellulitis and bacteremia over the years. Living with this disease has led him to feel physically and emotionally exhausted and his quality of life was significantly impaired. Pain and palliative services were involved in his care which eventually made him feel better and after informed discussions, he decided to change his status from full code to DNR/DNI.

Discussion: DD or keratosis follicularis is a rare autosomal dominant genodermatosis with a prevalence of 1 in 100,000. It occurs due to mutations in the ATP2A2 gene on chromosome 12q23-24 that codes for Sarco/Endoplasmic reticulum Calcium ATPase resulting in desmosomal breakdown and acantholysis. The disease usually persists for life and is characterized by a relapsing-remitting course. Topical agents like retinoids and 5- Flurouracil have shown to benefit limited DD. More severe presentations, like our patient, usually require high dose, or a prolonged course of low dose retinoids. Steroids may provide symptomatic benefit, but were not used in our patient as he recently underwent a gastric bypass procedure. Avoiding triggers like sunlight, heat, occlusive clothing and friction may prevent exacerbations. The disease can be partially controlled by the above measures, but there is no definitive cure as of yet. The condition can be challenging to treat and can cause several complications that may result in frequent hospitalizations. Sepsis, with the damaged skin as the portal of entry, and widespread herpes can occur. Studies have shown genetic links and associations between DD and psychiatric conditions like mental retardation, recurrent depression and bipolar disorder.

Conclusions: DD is a rare inherited skin disorder causing frequent inpatient admissions in those affected by it. Hospitalists must be aware that these patients require a multidisciplinary approach involving the primary care provider, dermatologists, infectious disease specialists, palliative care experts, psychiatrists and social workers as the disease can have profound effects on both physical and mental wellbeing.

IMAGE 1: Skin lesion of Darier’s Disease in the gluteal region.