Onychomadesis with Lichen Planus: An Under-Recognized Manifestation.

Onychomadesis or proximal separation of the nail pate usually results from severe, generalized dermatoses like bullous diseases, hand foot and mouth disease, varicella or severe cutaneous drug reactions. Although lichen planus (LP) produces varied nail manifestations (longitudinal onychorrhexis, onychoschizia, nail pigmentation, pterygium etc.), to the best of our knowledge, onychomadesis as a manifestation of nail LP is not recorded. This report presents two children with onychomadesis arising with generalized eruptive LP.

What was known?

Onychomadesis is a manifestation of multitude of disorders affecting the nail, including bullous diseases, severe adverse drug reactions, and viral infections.

Lichen planus preferentially affects the matrix in the nail unit. Varied manifestations in the form of onychorrhexis, melanonychia, pterygiuym etc., have been described.

Introduction

Onychomadesis is the spontaneous proximal separation of the nail pate (NP), considered by many to be a progression of Beau's lines.[1] It usually results from generalized dermatoses like bullous disorders; viral infections like hand, foot and mouth disease or varicella; severe cutaneous drug reactions; intense X-ray therapy; acute and chronic paronychia;[2] or severe psychological distress.[3]

Lichen planus (LP) produces varied nail manifestations in the form of longitudinal onychorrhexis, onychoschizia, nail pigmentation, pterygium, etc.[4] However, to the best of our knowledge and belief, onychomadesis as a manifestation of nail LP has hitherto not been reported. We report two interesting cases of onychomadesis arising with generalized eruptive LP in children. The response to an early institution of intra-matricial corticosteroid therapy is also highlighted.

Case Reports

Case 1

Patient 1 was a 7-year-old girl with multiple, itchy, violaceous eruptions over both hands and feet for 6 months. There was no history of photosensitivity, oral mucosal involvement, hair or nail loss accompanying or preceding the lesions. On examination, multiple flesh-colored as well as violaceous, papules and annular plaques, over dorsae of hands and feet were seen with mild overlying scaling. Her nails, mucosae, palms and soles, scalp and other hair bearing areas were normal. A lesional biopsy confirmed the diagnosis of LP. With normal hematological, biochemical investigations and serum G6PD activity, she was stated on dapsone (50 mg daily), oral antihistaminics, and topical mid potency steroid. However, 2 weeks later, discoloration and surface irregularity was observed in the proximal part of multiple nails [Figure 1a] which progressed to a clearly visible defect in the juxtamatricial portion of NP [Figure 1b]. Similar changes were seen in toenails after 4 weeks [Figure 2a]. The parents or the child were not ready for a nail biopsy. The need for early initiation of steroid therapy was explained, and they agreed for intra-matricial injections. Triamcinolone acetonide (5 mg/ml) was injected in the proximal nail fold area of affected finger and toenails at 4 weekly intervals. Over next 4 months, new skin lesions stopped appearing, and older ones showed signs of regression [Figure 1c]. The treated nails improved slowly and did not develop permanent loss or scarring. A visibly thinner NP was appreciated which normalized over further 3 months.

Figure 1

Hand nails of patient 1. (a) At the start of therapy. Multiple annular lesions of lichen planus appreciated. The thumb nail shows an area of discoloration proximally. (b) After 4 weeks, a visible defect in the proximal nail plate can be appreciated (onychomadesis). (c) After 16 weeks of intra-matricial steroid therapy. The nail plate is improved and there is no evidence of scarring

Figure 2

Toe nails of patient 1 and patient 2

Case 2

Patient 2 was a 12-year-old boy, with eruptive LP of 3 months duration. He presented with extensive lesions, Figure 2b showing koebnerization, over the dorsae of hands and feet and few lesions over the trunk. The nails were essentially normal except for the left great toe nail that had discoloration and proximal painless separation of NP. This child was also started on oral dapsone and intra-matricial injections of triamcinolone acetonide (5 mg/ml) in the affected nail, following which improvement was noticed.

Discussion

Onychomadesis results from a temporary arrest in the proximal nail matrix proliferation resulting in separation of proximal NP by a whole-thickness sulcus.[5] Clinically, a slowly appearing cleavage of NP, which has the potential to progress to complete separation, is seen. Less frequently, a latent form may be noticed where temporary complete inhibition of nail growth shows a transverse split of NP, in the form of a deep Beau's line, nevertheless the nail continues to grow. Causes of onychomadesis reported in literature include local factors, cytotoxic drugs, and systemic diseases. Local factors like acute paronychia and local trauma are the most commonly responsible.[4] Systemic diseases like Kawasaki disease, scarlet fever, hand-foot and mouth disease, thrombocytopenia and systemic lupus erythematosus have been reported to produce onychomadesis.[56] Various drugs implicated in the causation include antineoplastics and valproate.[7] Onychomadesis can also be idiopathic or familial.[6] Nail involvement reportedly occurs in 10% of patients with LP with fingernails being more frequently affected.[8] It is more common in adults, and usually several nails are affected. In the vast majority, nail LP presents with so called “typical” matrix involvement producing nail thinning, longitudinal ridging, onychoschizia, and fissuring.[5] Pterygium formation is a possible outcome indicating matrix scarring. Less commonly, LP of the nail bed is seen producing onycholysis or subungual hyperkeratosis. Other reported features include yellow nail syndrome-like changes,[8] trachyonychia,[9] idiopathic atrophy,[10] nail bed erosions and pigmentary changes.[4] On the basis of a strong temporal correlation between the onset of LP and onychomadesis in these two patients (in the absence of any other local or systemic factors), it is apparent that nail LP produced onychomadesis. Though, nail biopsy would have reinforced the finality of this association, it was neither possible nor ethical in both the cases under the given circumstances. To best of our knowledge, onychomadesis as a presenting manifestation has previously not been reported.

Lichen planus of nail matrix is slowly progressive but potentially scarring, the most dreaded complication being permanent anonychia and pterygium. Hence, prompt treatment with steroids either systemic or intra-matricial is required, producing near total regression of early changes.[1112] Both our patients responded favorably with regrowth of a thinner but normal appearing nails.

To conclude, we wish to highlight onychomadesis as a manifestation of nail LP. LP induced severe inflammation in the matrix leading to a temporary growth arrest was the possible mechanism. A favorable response to therapy suggests that an early recognition and prompt treatment can help reverse changes. Onychomadesis may be not so rare, but an under-recognized and under-reported manifestation of nail LP. Well-designed clinical studies of nail changes in LP may help clarify this issue.

What is new?

Onychomadesis can be a manifestation of nail lichen planus and generally signifies acute progression and severe suppression of nail matrix proliferation.

Early recognition and institution of therapy can help reverse potentially permanent nail los.