Twenty-Nail Dystrophy Originating From Lichen Planus

Virenda N. Sehgal, MD; Sonal Sharma, MD; Sujay Khandpur, MD, DNB

Disclosures

Skinmed. 2005;4(1):58-59. 

Abstract and Content

A 6-year-old boy was apparently well 4 months prior reporting to the clinic, when his parents noticed an asymptomatic, progressive change in the texture of his fingernails and toenails. They noticed that his nails had started getting grubby, rough, and brittle. A few of the nails had become distorted. The boy's parents became anxious and correlated the event to his playing outdoors. Frequent use of washing with soap and water was resorted to ease the problem without a favorable outcome. Nail examination was quite conspicuous, and was apparent by the presence of alternating elevation and depression (ridging) and/or pitting, lack of luster, roughening (similar to sandpaper), splitting, and a change to a muddy, grayish-white color. The nails were bilateral and symmetrical (Figure 1). Punch biopsy of the dystrophic nail plate using 3-mm skin punch was performed. It was driven down through the nail plate to the periosteum. Subsequently, the biopsy was lifted with a 26-gauge needle driven through the nail plate of the cylindrical plug along with bound-down tissue underneath. Iris scissors were used to remove the biopsy. The material was processed for histopathology. Hematoxylin-eosin–stained sections were prepared. The sections were marked for changes confined primarily to the epidermis. They were apparent in the form of hyperkeratosis and a corresponding prominent hypertrophy of the stratum granulosum. There were numerous relatively course keratohyaline granules associated with marked acanthosis affecting the stratum malpighii and rete ridges. The latter displayed irregular increase in size; some of which pointed at their lower end (sawtooth). Vacuolization/liquefaction degeneration of the basal cell layer was notable in various areas. In addition, the papillary dermis had an inflammatory component comprising lymphocytes, a few histocytes, and polymorphonuclear leukocytes (Figure 2a, 2b). The morphological changes and microscopic pathology in all of the nails were fairly conducive to form the diagnosis of lichen planus-(lichenoid interface dermatoses-) related twenty-nail dystrophy. Therefore, it was decided to use intra-matrix steroids and oral griseofulvin together. A steroid solution was prepared with 1 mL of lignocaine hydrochloride (2%) with 10 mg triamcinolone and 20 mg hydrocortisone acetate. A 26-gauge needle attached to a tuberculin/insulin syringe was utilized. The skin was penetrated at a 45° angle near a point 2–2.5 mm proximal and lateral to the junction of horn. Sudden feeling of give way (perforation) was a pointer to the precise site of injection. The solution was pushed gradually into the tissue; the success of the procedure was evident through the whitening of the lunula. This procedure was used in all of the fingernails and toenails during the same office visit. General anesthesia through slow IV infusion of ketamine hydrochloride (2 mg/kg body weight) was administered. The intervention was supplemented by oral griseofulvin 187.5 mg/d one-half hour after meals (10 mg/kg body weight/125–250 mg/q.d. in single or divided doses). The child was treated with griseofulvin for a period of 6 months and reported for follow up every month until completion of the treatment. He had a slow perceptible amelioration of twenty-nail dystrophy; presently, there is a restoration of the nails to normal (Figure 3).

Twenty-nail dystrophy (trachyonychia).

Both hyperkeratosis, marked hypergranulosis, prominent and course keratohyaline granules, acanthosis, and vacuolization/liquefaction degenera-tion of the basal cell layer (A] H&E ×100, B] H&E ×40)

Twenty-nail dystrophy (trachyonychia: follow-up 4 months after treatment with intra-matrix steroids (one sitting) and oral griseofulvin

Administration of griseofulvin, an antifungal antibiotic in non-fungal dermatoses, has been a recent focus of attention.[1] Its therapeutic efficacy in lichen planus,[2,3] in particular, is worthwhile to point out. Encouraged by the preceding observation, it was considered valuable to administer the drug in lichen planus-emanated twenty-nail dystrophy (TND). The clinical details of TND were initially described by Hazelrigg, et al.[4] The nail changes in TND are cardinal enough to be referred to as tachyonychia.[5] TND nails are rough (sandpaper-like), lusterless, brittle, split, and have prominent longitudinal ridging and/or pitting. Most often than not, it is an exclusive manifestation without any precursor.[4] All fingernails and toenails are afflicted. TND is associated with several autoimmune disorders[6] such as vitiligo[6,7] and alopecia areata.[8,9] However, its association with lichen planus is unique,[10–12] as is obvious from the current case. In fact, histopathology was contributing to it being lichen planus-(lichenoid interface dermatoses-) related TND. Hence, it was to institute oral griseofulvin for its treatment. Its precise mechanism is unknown; nevertheless, its anti-inflammatory action might be contributory, in addition to inhibition of the nucleic acid metabolism of keratinocytes, and be considered vital in the process of re-epithelization. It was considered an essential adjunct to one-time intra-matrix steroids to carry on its well-conceived anti-inflammatory action. Moreover, such an intervention was difficult to repeat in a young child. Thus it is worthwhile to emphasize the role of oral griseofulvin in lichen planus-related TND, either alone or in combination with intra-matrix steroids. Therapeutic modalities such as intralesional triamcinolone[6] and local psoralen-UV-A[13] therapy have been tried. Triamcinolone, hydrocortisone acetate, and/ or griseofulvin are yet other novel modalities that have never been made use of in the past.

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