Nail involvement may occur in 1% to 10% cases of lichen planus (LP) and mostly in the setting of widespread cutaneous disease.1 Longitudinal ridging, thinning, and distal splitting of the nail plate are the most common nail changes in LP.2
A man in his 60s presented with painful swelling of the proximal nail folds, dripping of blood from his fingernails and toenails for the last year, and occasional pus discharge. There was no history of preceding trauma or drug intake.
On physical examination, the proximal nail folds were found to be swollen and showed violaceous discoloration of 7 fingernails and 3 toenails. The index fingernails also demonstrated longitudinal ridging, thinning, and focal fragmentation of the nail plate, while the remaining fingernails and involved toenails showed partial to complete loss of nail plate with oozing of blood resulting in hemorrhagic crusting of the nail beds and folds (Figure 1A). Oral mucosa showed lichenoid plaques on the right angle of mouth, bilateral buccal mucosa, and erosions covered with hemorrhagic crusts on the upper and lower lips. A provisional diagnosis of bullous lichen planus of the nails was made. Laboratory investigations revealed raised liver enzyme levels and positive anti–hepatitis C virus antibody status.
A, Before treatment, swelling and violaceous discoloration are apparent on the proximal fingernail folds along with oozing of blood, hemorrhagic crusting of the fingernails, and pus discharging from the lateral nail folds. B, After treatment, the hemorrhagic crusting and paronychia have completely resolved, with resultant atrophy of the fingernails.
The patient was treated with oral antibiotics for 1 week and a topical steroid–antibiotic combination for 6 weeks, and the hemorrhagic crusting and nail fold swelling subsided completely, resulting in anonychia of the affected nails (Figure 1B). A skin biopsy was performed from the proximal nail fold of the right index finger 1 month after the initial presentation to avoid secondary changes due to infection that was present at the time of the initial presentation. Histopathologic examination demonstrated hyperkeratosis, hypergranulosis, acanthosis, basal cell degeneration, numerous apoptotic keratinocytes in the epidermis with dense bandlike lymphohistiocytic infiltrate in the papillary dermis consistent with the diagnosis of LP (Figure 2).
Present are hyperkeratosis, hypergranulosis, acanthosis, basal cell degeneration, Civatte bodies in the epidermis, and dense bandlike lymphohistiocytic infiltrate in the upper dermis (hematoxylin-eosin, original magnification ×10).
Nail LP (NLP) usually presents in association with cutaneous, mucosal, or scalp lesions but may be the sole manifestation of the disease. It may involve the nail matrix, proximal and lateral nail folds, nail bed, and/or the hyponychium. Proximal matrix involvement resulting in longitudinal ridging is the most common and the earliest manifestation of NLP.1 Trachyonychia usually occurs in children as an isolated finding in the absence of LP at other sites and of other typical signs of NLP. Pterygium, irregular nail pitting, onychorrhexis, crumbling, and fragmentation of the nail plate are other clinical manifestations of nail matrix involvement. Nail bed involvement may take the form of small red papules visible as violaceous lines or papules through the nail plate, subungual hyperkeratosis, and onycholysis.1 Complete destruction of the nail matrix and nail bed results in total nail atrophy. Idiopathic atrophy of the nails is a distinct manifestation of NLP that results in rapid and diffuse scarring of nails in children. Less frequent signs of NLP described by Tosti et al3,4 include erythematous patches of the lunula, melanonychia, splinter hemorrhages, koilonychia, and yellow nail syndrome–like changes.3,4
Our case exemplifies the unusual presentation of bullous LP of the nails in the form of hemorrhagic lesions resulting in complete shedding of the nail plate and nail atrophy. Bullous LP of the nails is an uncommon and extreme variant of LP that may be associated with bullous and ulcerative lesions on the feet and toes, with or without cicatricial alopecia and oral involvement.5,6 Diagnosis is often challenging, and the presence of characteristic lesions at other sites in conjunction with histopathologic examination aid in confirmation of the diagnosis.
Corresponding Author: Sanjeev Handa, MD, FRCP, Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India (email@example.com).
Published Online: March 4, 2015. doi:10.1001/jamadermatol.2014.5701.
Conflict of Interest Disclosures: None reported.
Khullar G, Handa S, De D, Saikia UN. Bullous Lichen Planus of the Nails. JAMA Dermatol. 2015;151(6):674-675. doi:10.1001/jamadermatol.2014.5701