A Rare Presentation of Psychosis in an Adult Male Patient with Verrucous Epidermal Nevus

INTRODUCTION Verrucous epidermal nevus (VEN) is a benign dermatological condition, which is present from birth or appears during the early developmental period.[1] The prevalence of VEN is 1 in 1000 live births with an equal male:female ratio. Neuropsychiatric symptoms such as epilepsy, intellectual disability, and hemiparesis have been reported in 66% of patients with certain variants of VEN.[2] Existing literature reports cases of VEN with intellectual disability; however, to the best of our knowledge, there have not been reports of psychosis and catatonia in cases of VEN. This case report aims to document an unusual clinical manifestation of psychosis with symptoms of catatonia in an adult male patient with VEN. CASE REPORT The patient was a 25-year-old male, born by nonconsanguineous marriage, with normal early motor and language development. History was suggestive of below-average intellectual functioning. The patient was below average in academics and failed twice in 9th grade, following which dropped out of school. He would also have difficulty sustaining peer relationships and would be frequently bullied. He would not understand the time and money concepts and required supervision while traveling. The patient had one episode of seizure at 14 years of age. At the age of 17, his parents noticed that the patient would remain withdrawn, would smile, mutter, and gesticulate to self. The patient would tell his parents that he could see one male and one female figure coming near him and speaking to him. For these complaints, the patient was taken to a psychiatrist and was started on tablet risperidone 4 mg, later tablet olanzapine 10 mg was added. As there was poor response, the tablet clozapine was added and increased to 100 mg. The patient was 80%–90% better and was advised to continue tablet clozapine, which he continued to take for the next 4 years. At the age of 21, the patient had an episode of fever, following which similar complaints re-emerged. In addition, the patient also had symptoms of catatonia in the form of mutism, staring spells, posturing, stereotypic movements, and reduced food intake. The patient was dependent on his parents for activities of daily living such as eating, bathing, and toilet needs which was unlike before. Stooped posture with bowing of legs was noticed for 4 years. The patient was taken to several psychiatrists and was treated with tablet clozapine 100 mg, tablet risperidone 4 mg, tablet amisulpride 50 mg, which was increased to 100 mg. Owing to minimal improvement over 4 years, the patient was brought to our tertiary care center. A multidisciplinary approach was followed. On examination, the patient had brown verrucous plaques with linear distribution over the neck, trunk, and bilateral upper limbs which were present since the age of 2 months. The patient was diagnosed to have VEN with systematization, which was confirmed on histopathology. Other investigations done in view of syndromic features revealed the patient to have Vitamin D deficiency osteopenia, metabolic myopathy, Budd-Chiari syndrome, and hiatus hernia. Magnetic resonance imaging of the brain was found to be normal. Tablet clozapine was stopped in view of paralytic ileus and neutropenia. The patient was started on tablet ziprasidone 20 mg along with tablet lorazepam 2 mg. The patient started to speak 1–2 words after initiation of tablet lorazepam and it was increased to 6 mg. The patient was given trial of Electroconvulsive therapy (ECTs) (total 10) owing to poor response to antipsychotic medications and symptoms of catatonia. The patient had extra pyramidal symptoms (EPS) at higher doses of tablet ziprasidone and hence dose was optimized at 60 mg. Following this, hallucinatory behavior and posturing reduced and there was an improvement in nonverbal communication. Patient’s ability to perform activities of daily living (ADL) such as eating and bathing improved. However, the patient regained speech restricted to only a few words. The Bush Francis Catatonia Rating Scale had a total score of 7 at the time of discharge, as compared to a score of 21 at the time of admission. DISCUSSION In the literature, there are reports of intellectual disability, and structural central nervous system defects in patients diagnosed with VEN; however, cases with comorbid psychosis have not been reported [Table 1]. Mutations in genes PIK3CA, KRAS, and FGFR3 are implicated in VEN.[8] Dysregulation of Ras-extracellular signal-related kinase (ERK) signaling is found in the pathogenesis of RASopathies.[9] The phenotypic presentation of our patient was similar to RASopathies described in the literature.[10] However, genetic testing could not be done owing to financial constraints. Although certain symptoms improved, minimal improvement in mutism points toward neuroregression. Treatment of neuropsychiatric manifestations in such patients can be challenging as these patients have several other comorbid conditions, increasing their susceptibility to adverse reactions. This highlights the need to identify underlying pathogenic mechanisms and further develop targeted therapeutic strategies to improve the quality of life of patients with such phenotypic presentations.Table 1: Different clinical manifestations of patients with verrucous epidermal nevus in literatureDeclaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patient understands that his/her/their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.