Trachyonychia secondary to pediatric alopecia areata responding to oral tofacitinib therapy: a case report and review of literature

A 14-year-old male presented to the dermatology outpatient department with a 1-year history of sudden, asymptomatic hair loss on the scalp along with rough, fragile nails causing pain and impairment in routine daily activities. There was no history of atopy, autoimmune disorders, or family history of a similar condition. The patient had previously been treated with oral betamethasone pulse (0.1 mg/kg/day twice weekly for 3–4 months) and cyclosporine (5 mg/kg/day for 5 months) over the past year. While hair recovery was temporary, relapsing upon medication tapering, there was no improvement in his nail condition. On examination, the baseline Severity of Alopecia Tool (SALT) score was 56.3 (Figure 1a). The nails were brittle, rough, and opaque, with a sandpaper-like appearance (Figure 1b). Based on these findings, the patient was diagnosed with alopecia areata subtotalis with trachyonychia. Given the poor response to conventional therapies, treatment with the pan-JAK inhibitor tofacitinib was initiated. Baseline investigations, including complete blood count, liver function tests, renal function tests, fasting lipid profile, and Mantoux test, were all within normal limits. Tuberculosis interferon-gamma release assay (TB-IGRA) and viral serology for hepatitis B, C, and human immunodeficiency virus (HIV) were negative. The patient was started on oral tofacitinib (5 mg twice daily according to body weight) along with topical minoxidil (5%) for the scalp. After 8–12 weeks of therapy, the nails showed increased thickness and became less fragile, with the sandpaper-like texture becoming less coarse. Hair regrowth was observed after 12–16 weeks onward, with complete hair growth at around 6 months of therapy (SALT score = 0) (Figure 1c). The nails improved significantly, becoming smooth and shiny, with mild superficial regular pitting (Figure 1d). Routine investigations were repeated at 1, 3, and 6 months. Treatment with oral tofacitinib was continued for a total of 9 months, followed by stoppage. Currently, at the 6-month post-treatment follow-up, the patient's disease is in remission. Alopecia areata (AA) is an autoimmune disorder affecting the hair follicles, leading to nonscarring alopecia. Nail involvement occurs in 10–66% of AA patients, with more severe cases seen in alopecia totalis and universalis. The nails may be affected before or during hair loss and are a poor prognostic factor for AA. The most common nail findings are pitting, longitudinal ridging, trachyonychia, and leukonychia, which are found in descending order of frequency.1 Tofacitinib is a Janus kinase (JAK) inhibitor that targets JAK1 and JAK3 receptors. Nail matrix keratinocytes share similar gene expression profiles with hair follicle keratinocytes. Tofacitinib works by inhibiting CD8+ effector T cells, which target the nail matrix, thereby reversing dystrophic changes. The severity of inflammation correlates with the severity of nail changes. Ferreira et al. reported significant improvement in nail changes in a patient with alopecia areata universalis after 10 months of tofacitinib therapy.2 We have summarized the previous limited literature on the efficacy and safety of all oral JAK inhibitors on nail changes in alopecia areata (Table 1). Case 1–38 years/M Case 2–36 years/F Case 1–10 years Case 2–15 years Case 1: CS, MTX, CyA, PUVA Case 2: minoxidil, CS Hence, JAK inhibitors are a potentially effective treatment for children and adolescents with alopecia areata with nail involvement. However, there have been occasional reports of disease flares while on treatment.5 As a result, it is important to counsel parents and children thoroughly about the unpredictable nature of the condition and the potential need for dose adjustments or additional treatments during flares to minimize further hair loss. Our patient showed remarkable nail improvement within 3–4 months of tofacitinib therapy, with no adverse effects and a prolonged remission period even while off treatment. This report underscores the potential efficacy of tofacitinib in reversing treatment-resistant nail changes in AA. However, further research is needed to validate the findings of this report. Informed consent was obtained from the patient.