Nail lichen planus (NLP) is a chronic inflammatory disease of unknown etiology and has been recognized as a nail potentially critical disorder, which can be severe and rapidly worsen with irreversible scarring. Currently, the treatment options are limited based on disease progression. High-potency topical or intralesional corticosteroids are commonly considered first-line therapeutic options; however, these therapies are unsuitable for all patients with NLP, especially those with extensive lesions. As a potential therapeutic target for inflammatory skin diseases, Janus kinase (JAK) inhibitors can suppress both type-1 and type-2 cytokines, thereby reducing the immune response and resultant inflammation. Recent studies have suggested benefit in cutaneous lichen planus and lichen planopilaris with oral JAK inhibitors. Here, we report a case of severe NLP that exhibited a favorable response to tofacitinib treatment. A 41-year-old woman presented to our clinic with a 2-year history of nail dystrophy of all fingers of both hands. The NLP was finally confirmed by histopathology and the above clinical features. After the informed consent signature, tofacitinib monotherapy, 5 mg twice a day, was then begun, and after 6 months, the appearance of her nails had a significant improvement.

Background Isolated nail lichen planus (NLP) without mucocutaneous involvement is rare. Literature about the clinical profile and management is scarce. Aims/Objective We attempted to characterize the clinico-demographic profile and analyze the management outcome of patients with isolated nail lichen planus. Methods Records of 15 patients were evaluated, and their demographic profile, clinical features of the nail matrix and nail bed disease, onychoscopy findings, histopathological features, treatment given, and follow-up progress were analysed. Results Data from 15 patients were collected. The mean age of the patients was 28.6 ± 19.0 years (range 3.5 years to 56 years). The gender ratio was 0.8 with 7 males and 8 females. The average disease duration at presentation was 2.8 ± 2.6 years (6 months-9 years). The average number of nails involved was 16.4 ± 4.6 (8-20 nails). All 20 nails were involved in 9 (60%) patients. Nail matrix -involvement was seen in all, with onychorrhexis being the most common manifestation, which was seen in 11 (73.3%) patients. Nail bed involvement was seen in 11 (73.3%) patients, with onycholysis being the most common presentation. Severe nail disease was seen in 7 (46.7%) patients, and 5 (33.3%) had pterygium involving an average of 3.4 nails. Moderate to good improvement was observed in 9 (60%) patients after an average of 6.1 ± 2.4 (3-9) treatment sessions with intramuscular and intramatricial triamcinolone acetonide injection. Of these, 2/9 (22.2%) developed disease recurrence in a few nails after an interval of 1 and 1.5 years, respectively. Two patients achieved complete clinical cures that persisted beyond 2 years of follow-up. Limitations Retrospective nature of the series and the small sample size are the major limitations. Conclusion The risk of permanent disfigurement is high in NLP and calls for an early diagnosis and prompt treatment. Intralesional and intramuscular steroids are first-line therapeutic options depending upon the number of nails involved.

UNASSIGNED: Recent expert recommendations suggest mycophenolate mofetil (MMF) as a third-line therapy, in severe corticosteroid-dependent or corticosteroid-resistant nail lichen planus (NLP). However, there is currently no literature to support MMF use in this indication. This is a retrospective monocentric French case series of 5 patients with severe corticodependant or corticoresistant NLP treated by oral MMF (2-3 g/day), between 2013 and 2021.
UNASSIGNED: The primary outcome was therapeutic success in a target fingernail. All 5 patients showed some clinical improvement, ranging from mild improvement (1/5) to clinical cure (2/5). Clinical improvement was more significant when the drug was taken for a longer period (24 months vs. 4 months) and at a higher dose (3 g/day vs. 2 g/day). Relapse occurred after stopping or tapering the MMF dose. MMF was well tolerated.
UNASSIGNED: MMF may be a treatment to consider for severe corticosteroid-dependent or corticosteroid-resistant NLP. The long-term safety of this treatment warrants further investigation.

Cogan syndrome and lichen planus represent two autoimmune disorders. Cogan syndrome is a very rare type of ANCA-negative vasculitis affecting the eyes and vestibulocochlear system. It has been associated with other autoimmune disorders, none of them showing any lichenoid inflammation. We herein report the first case of a patient that suffered from Cogan disease and developed isolated lichen planus on all nails a few years after the first diagnosis. The combination of two autoimmune disorders is not unusual and raises the question of common immunogenetic pathomechanisms.