Nail lichen planus: response to treatment and long term follow-up

ARTICLE

Auteur(s) : Bianca Maria Piraccini, Elena Saccani, Michela Starace, Riccardo Balestri, Antonella Tosti

Department of Internal Medicine, Geriatrics and Nephrology, Division of Dermatology, University of Bologna, Via Massarenti, 1 – 40138 Bologna, Italy

accepté le 1 F�vrier 2010

Nail lichen planus (LP) is not rare, and nail lesions may occur in the absence of cutaneous or mucosal involvement [1-4]. The disease is more common in adults (with a peak incidence at 50-60 years of age) and usually affects several or most nails, with a chronic course.

In the vast majority of patients, nail LP present with ‘typical’ and suggestive clinical features, due to nail matrix involvement, and characterized by nail thinning with longitudinal ridging and fissuring. Pterygium formation is a possible but rare outcome and indicates nail matrix scarring. LP of the nail bed can be associated with matrix disease and produces onycholysis and mild subungual hyperkeratosis. Besides these very typical symptoms, nail lichen planus may produce other nail abnormalities, including yellow nail syndrome-like changes in the toenails [5, 6], trachyonychia [7], idiopathic atrophy of the nails [8], nail bed erosions [9] and pigmentary changes, including longitudinal melanonychia [10] and longitudinal erythronychia [11-13].

The clinical diagnosis is confirmed by an appropriate biopsy from the nail matrix or the nail bed – depending on the clinical symptoms- that shows a dense band-like infiltrate composed mostly of lymphocytes, linear dermo-epithelial junction or irregular epithelial hyperplasia, numerous melanophages in the superficial dermis and diffuse granulosis without a wedge-shaped pattern in the matrix.

Typical nail lichen planus is slowly progressive, nail symptoms are in fact usually stable for months after onset, and pterygium formation takes several months or may not occur. Treatment with intralesional or systemic steroids (the choice is based on the numbers of nails involved) is the gold standard [1], and it produces total or subtotal regression of the nail symptoms. Recurrences after remission are possible, with the need to re-evaluate further treatment options.

Information on the percentage of patients who respond positively to treatment and on the long term outcome of nail lichen planus is not available, all published studies focusing on response to treatment have a small series and/or a short follow-up [1-4, 9].

The aim of our study was to assess the response to treatment of patients affected by nail LP and to evaluate the long-term outcome (more than 5 years) of the disease. For this purpose we retrospectively evaluated the treatment outcomes of 75 patients with pathologically proven nail LP treated at our Department from 1986 to 2007, and the long term outcome of the condition in a subgroup of 27 patients followed up for more than 5 years.

Materials and methods

Patient population

• – Typical nail LP (figure 1), characterized by nail plate thinning with longitudinal ridging and fissuring: 82 patients (26 of whom were children);
• – Trachyonychia: 10 patients;
• – Yellow Nail Syndrome (YNS)- like features in the toenails: 5;
• – Idiopathic atrophy of the nails (IAN): 7 patients;
• – Bullous-erosive LP: 1 patient.

We did not prescribe treatment to patients with trachyonychia due to LP, due to the characteristic benign outcome of this condition [10], nor did we prescribe treatment to the 7 patients with IAN, since the condition is characterized by definitive nail scarring [8].

The patient with erosive LP was treated with intramuscular systemic triamcinolone acetonide 0.5 mg/kg every 10 days (tapered over 2 months), with rapid subsiding of the pain and inflammation but with a scarring outcome with permanent anonychia. Twelve patients were treated and followed by other dermatologists. The 75 patients that we treated and followed are listed in table 1 and include 39 males and 36 females, aged from 7 to 80 years (mean age at the time of diagnosis: 54 years). Some of these cases have already been published [1, 4]. Mean follow-up duration of these patients was 5.2 years (from 1 to 23).

All 20 nails were involved in 29 patients, more than 5 nails in 48, 3 or less nails in 8, toenails were involved in 34 patients. Fingernail involvement was characterized by exclusive matrix involvement (nail plate thinning with longitudinal ridging and splitting) in 48 patients, by matrix and bed involvement (above symptoms associated with onycholysis and mild subungual hyperkeratosis) in 18 patients, and exclusively by nail bed involvement in 9 cases. At the time of our first visit, dorsal pterygium was present in 4 patients (in 3, in one fingernail; in 1, in one toenail). Toenail involvement was always associated with fingernail symptoms and in 5 cases presented as yellow nail syndrome-like changes, characterized by marked nail thickening, transverse over-curvature and yellow discoloration. In 29 cases the toenails showed nail matrix LP, with nail longitudinal ridging and splitting. The mean duration of the disease at the time of our diagnosis was 16 months (range: 6-51 months).

21 of these 75 patients presented cutaneous/mucosal lichen planus: 10 patients oral lichen planus, 2 oral and cutaneous LP, 3 cutaneous LP, 1 cutaneous LP and lichen plano-pilaris of the scalp, 5 lichen plano-pilaris of the scalp. Genital involvement was never observed. Other associated cutaneous and non-cutaneous diseases to nail lichen planus included: psoriasis (3 patients), alopecia areata (7 patients), hypothyroidism (4 patients), plantar keratoderma (1 patient), atopic dermatitis (a 7-year-old child), polymyalgia (1 patient), Sjogren syndrome (1 patient), celiac disease (1 patient), and hypothyroidism (3 female patients).
Table 1 Data of the 75 patients with nail LP treated and followed-up

Treatment

• – Systemic steroids (intramuscular triamcinolone acetonide 0.5 mg/kg a month): 67 patients. In 2 patients this was associated with systemic azathioprine (100 mg/die);
• – Intralesional steroids (triamcinolone acetonide 10 mg/mL diluted in saline solution and injected (0.1 mL into each of four periungual sites monthly, using the de Berker's technique [14]): 8 patients – the ones with few fingernails affected.

Treatment was always prescribed until the proximal half of the nail was normal; this usually took 3-4 months for fingernails and 7-8 months for toenails. Treatment was then interrupted in case of intralesional steroids, or gradually tapered off when systemic, with 2-3 monthly intramuscular injections of triamcinolone acetonide at the dose of 0.25 mg/kg. If the nails did not improve it was stopped after 5-6 months. Second line treatments included azathioprine in association with systemic steroids and systemic etretinate. Azathioprine 100 mg a day was added to steroid treatment in 2 patients who had not responded to systemic steroids after 4 injections. Etretinate at the dosage of 0.35 mg/kg/day was utilized in 5 patients who did not respond to systemic steroids after 5 months of treatment.

Results (table 1)

Treatment outcome

• – Intralesional steroids: 7 of the 8 patients showed a complete regression of the nail symptoms after 4-7 injections. The remaining patient experienced the appearance of nail LP in other nails during therapy and was then treated with systemic steroids, with regression of the nail lesions. Side effects of treatment were pain from anesthesia and transient subungual hemorrhages (in 2 patients).
• – Systemic steroids: 44 patients (44/67 = 65.6%) were completely cured by the treatment, after a mean number of 4 injections of steroids at full dosage (figures 2A, B). Fingernails always responded better than toenails to treatment. Nine patients (9/67=13.4%) showed only mild improvement of nail LP: nail thickness returned to normal with persistence of mild longitudinal ridging, splitting of the distal margin and the presence of longitudinal bands of light leukonychia. Improvement of the toenails with YNS-like changes, obtained in 4 of the 5 patients, was very slow and occurred several months after the fingernail improvement. Fourteen of the 67 patients (14/67 = 20.8%) did not respond to treatment (they did not show any improvement of their nail lesions after 5 or 6 intramuscular injections of triamcinolone acetonide). In 4 of these patients, azathioprine 100 mg a day was added to steroids, without benefit. The 5 patients where steroid treatment was followed by a course of etretinate 0.35 mg/kg/day did not show any result. No important side effects related to treatment have been recorded.

Long- term follow-up (table 1)

Recurrences of nail lichen planus were reported by 11 of the 16 patients (11/16 = 69%) who had achieved a cure after systemic steroids; relapses appeared after 6 months to 3 years after cure (mean 13 months). All these 11 patients were resubmitted to a new course of treatment: one patient received intralesional steroid injections, since the recurrence involved only one digit. At present, 5 patients out of these 11 are cured (5/11 = 45%), 2 are improved (2/11 = 18%) and 4 are worsened (4/27 = 14.8%). The 2 patients treated with intralesional steroids remained cleared from LP in the follow-up period. The 9 patients who had not responded to the initial therapy with systemic steroids presented at the last follow-up visit with unchanged (6 cases) or worsened (3 patients) nail lesions. One patient has developed dorsal pterygium in one fingernail.

Newly emerged systemic or cutaneous disorders were diabetes mellitus, heart-related disorders, osteoporosis, Parkinson's disease, cutaneous melanoma, eczema and distal subungual onychomycosis, diagnosed in 5 patients in the big toenail and in 1 patient in a fingernail. Treatment with systemic antifungals produced a complete cure of the onychomycosis in all patients.

Discussion

Optimal therapy is still lacking, although systemic steroids are undoubtedly the treatment of choice. According to our study, about 2/3 of patients with LP of several nails, presenting with nail thinning with longitudinal fissuring and splitting, respond to a 5-7 month course of systemic steroids, with a complete or almost complete regression of the nail symptoms. Fingernails respond better and more quickly than toenails. Mild fissuring of the nail plate distal margin and longitudinal bands of leukonychia are the outcome in about 10% of patients, who do not show complete regression of the nail symptoms.

Relapses of nail LP occurs in about 60% of the cured patients (11 out of the 16), and in about half of cases are not responsive to treatment. It therefore appears that nail LP have a negative long-term prognosis in a considerably high percentage of patients, if we consider patients who do not respond the initial steroid treatment (20% of cases) and those with non-responsive relapses. Cicatricial outcome with the formation of dorsal pterygium is, however, rare and does not appear to be related to duration of the disease.

We could not find any factor (age, gender, duration, severity, associated skin/mucosal LP) that in our series could predict which patients would benefit from treatment and which would not. Our series is not very large, but it reflects the rarity of LP limited to the nails and the difficulty of maintaining a long-term follow-up for these patients. Patients who do not respond to one or more treatments are in fact not keen to continue to present to follow-up visits.

We suggest intralesional or systemic steroids in all patients with pathologically proven nail lichen planus, for a minimum period of 4 months. Assessment of response to therapy can then be done looking at the proximal 3 mm of the nail plate, which may show an improvement or persistence of symptoms.

Acknowledgements

References

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