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Background Severe nail lichen planus (NLP) does not respond well to treatment and is often poorly considered and described in detail. Objective We sought to describe the characteristics of severe NLP. Methods A retrospective data analysis was performed, including the photographic records of the most compromised nails of patients with NLP over 18 years old, who consulted between 2009 and 2019 at the Instituto de Dermatologia Professor Rubem David Azulay in Rio de Janeiro, Brazil. Descriptive and statistical analysis using Fisher's exact test was performed to verify the hypothesis of independence between characteristics (P < 0.05). Results A total of 102 patients were included. Anonychia was associated with severe thinning of the nail plate, retraction of the nail bed or nail plate, residual nail plate, loss of proximal nail fold limits, and onychoatrophy. Dorsal pterygium was associated with loss of proximal nail fold limits, onychoatrophy, and distal splitting greater than 50%. Conclusion We provide descriptions of the most severe signs in order to facilitate the clinical diagnosis when a biopsy is not feasible and suggest an update of current NLP classifications.

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<b><i>Introduction:</i></b> Tuberculosis is a common infection caused by <i>Mycobacterium tuberculosis</i> in developing countries. Among the extrapulmonary forms of tuberculosis, cutaneous tuberculosis is rare, and even dermatologists are less familiar with its ungual manifestations. <b><i>Case Presentation:</i></b> We present an exuberant case of ungual tuberculosis in a patient with no personal or family history of immunosuppression, which is the first manifestation of the disseminated form of the disease. <b><i>Conclusion:</i></b> In our patient, the initial manifestation was related to the nail, probably originating from the progression of bone TB focus, which further progressed with respiratory symptoms and involvement of other organs. We believe this is the first case in which the ungual presentation allowed the diagnosis of disseminated tuberculosis.