Dermatology Online Journal

Alopecia universalis with twenty-nail dystrophy (trachyonychia)
Peter Chien Jr. MD PhD, Olympia I Kovich MD
Dermatology Online Journal 14 (5): 24

Department of Dermatology, New York University

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Abstract

A 43-year-old man presented with long-standing trachyonychia of all 20 nails, noted worsening of his nail disease after the onset of alopecia universalis 18 months ago prior to presentation. Trachyonychia can be associated with alopecia universalis although the treatment strategies of both conditions differ. The principle of treating trachyonychia may involve regulating the differentiation of keratinocytes and/or reducing inflammation in the nail fold or nail matrix. The treatment of alopecia universalis involves immunomodulation.

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Clinical synopsis

A 43-year-old man initially presented to the NYU Dermatology Associates in April, 2007, for evaluation of nail ridges. The nail ridges have been present for 10 years but have worsened 18 months ago after the onset of hair loss over his entire body. He used fluocinonide lotion previously for the hair loss.

Past medical history and a review of systems were noncontributory. His children have eczema. He takes no medications.

Physical Examination

The hair on the scalp, face, and body was absent. All 20 nails exhibited longitudinal ridges and were rough in texture. Some nails also exhibited onychoschizia and distal onycholysis.

Figure 1	Figure 2

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Histopathology

None

Comment

Trachyonychia clinically appears as if the nails have been sandpapered in the longitudinal direction, which leaves a rough-ridged texture. Sometimes the terms trachyonychia and 20-nail dystrophy are used interchangeably in the literature. However, 20-nail dystrophy should only be used when all of the nails are affected. The cause of trachyonychia is often idiopathic, but when it appears in association with another condition, alopecia areata would be the most common [1]. One study of alopecia areata in children observed that 46 percent also had nail abnormalities and 12 percent specifically had trachyonycia [2]. It has been suggested that trachyonychia may herald the onset of alopecia areata [3], which is consistent with our patient's clinical course. Other associated conditions include lichen planus, psoriasis, immunoglobulin A deficiency, atopic dermatitis, and ichthyosis vulgaris [4].

Histopathologically, the proximal nail fold and nail matrix have been described to be spongiotic, with a mild lymphocytic infiltrate although occasionally lichenoid or psoriatic changes can be observed as well, which corresponds to the associated disease states [1, 3, 5]. When lichen planus and psoriasis were clinically and histopathologically excluded, spongiotic dermatitis in the nail matrix and nail bed with column-like parakeratosis in the nail plate was present when the trachyonychia was due to alopecia areata, atopic dermatitis, or an idiopathic cause [6]. However, another group found that in 40 of 1095 patients with alopecia areata, one had a nail biopsy with histopathologic changes of lichen planus. Therefore, nail biopsies may be useful when the clinical diagnosis is obscure [1].

Treatment for trachyonychia associated with alopecia universalis is challenging. Tazorotene applied overnight to the nail plate, nail folds, and periungual areas for 3 months resulted in acceptable clinical and functional improvement, with the side effects of peeling and erythema of proximal nail fold skin [7]. Trachyonychia due to psoriasis was successfully treated with acitretin at 0.3 mg/kg/day for three months [8]. Topical 5-fluorouracil and cyclosporin also have been used to treat trachyonychia associated with psoriasis whereas prednisolone, antimalarials, and etretinate have been used to treat trachyonychia associated with lichen planus [4]. Intralesional triamcinolone has been used with some success in idiopathic trachyonychia in 4 children, 1 of whom had 20 nails involved and a patch of alopecia areata [9]. Trachyonychia may spontaneously resolve after several years regardless of treatment [10].

Alopecia universalis is the severe form of alopecia areata that results in a complete non-scarring loss of hair of the scalp and body. It occurs equally in men and women, but children and adolescents are more frequently affected [11]. Like alopecia areata limited to patches on the scalp, it is thought to be an autoimmune phenomenon [12]; it can be associated with thyroid disease or atopic dermatitis [13]. It is also associated with certain HLA alleles [14, 15] and T cells also may be involved in the pathogenesis. Although the precise mechanism is unknown, case reports of alopecia universalis developing after hepatitis C treatment with pegylated interferon and ribavirin [16], after treatment of rheumatoid arthritis with adalimumab [17], and in association with HIV infection [18-21] point toward a derangement of the immune system. Psychosocial stress has been implicated as a cause although the study employed patient ratings in a retrospective fashion [22]. Nevertheless, one group reported success in an uncontrolled study with hypnotherapy either as an adjunct or as monotherapy and observed hair regrowth of at least 75 percent in 12 of 28 patients that were refractory to conventional treatments [23]. Nine of these patients experienced complete regrowth, which included 4 patients with alopecia universalis. A neurologic basis is supported in a case report of alopecia universalis that spared a single patch of hair in a denervated right axilla where a lymphadenectomy was performed, which severed cutaneous branches of the intercostobrachial nerve [24]. This patient also had 18-nail dystrophy with longitudinal ridges This case study suggested a neural mechanism interfacing with the immune system akin to vitiligo.

Treatment of alopecia universalis is based on immunomodulation. Although mixed results from case studies have been observed with topical imiquimod [24, 25], topical tacrolimus is a reported failure [26, 27]. Topical clobetasol and diphenylcyclopropenone (a topical sensitizer) have shown some efficacy [28, 29]. Systemically, oral, intramuscular, or intravenous pulse glucocorticoids [30-32], methotrexate [33], and efalizumab [34], have shown efficacy in treating alopecia universalis whereas etanercept [35, 36] did not appear to be effective. A retrospective study with PUVA photochemotherapy showed some efficacy as well [37]. Systemic treatments for unrelated conditions leading to serendipitous resolution of alopecia universalis include allogeneic hematopoietic stem-cell transplantation in a patient with chronic myeloid leukemia [38] and injection with recombinant human bone morphogenetic protein 2 to aid in healing of a fractured tibia [39]. Bone morphogenetic proteins are members of the TFG- superfamily and may be involved in the life cycle of the hair follicle or play a role in T-cell maturation in the thymus.

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