Dermatology

Lichen Nitidus

Are You Confident of the Diagnosis?

What you should be alert for in the history

Characteristic findings on physical examination

Lichen nitidus typically presents in children and young adults, usually as asymptomatic, but at times pruritic, small, monomorphic, Koebnerized, well-demarcated, round or polygonal, slightly shiny, flat-topped, mildly scaly grouped papules. Lesion color varies from hypopigmented to mildly violaceous, classically distributed on the flexor upper extremities, dorsal hands, trunk, and genitalia.

When present on the palms and soles, the papules have a sandpaper-like texture, and the nails can concurrently exhibit linear ridges and pits. Rarely, the buccal mucosa may display small grey flat papules.

Expected results of diagnostic studies

Histopathologic examination shows a lichenoid tissue reaction with basal keratinocyte vacuolization and papillary dermal inflammatory infiltrate (Figure 2). Most characteristically, this infiltrate, composed of lymphocytes, histiocytes, and sometimes epithelioid and multinucleated giant cells, aligns itself closely to a thinned and parakeratotic epidermis with rete ridges flanking the dermal collections into discrete groups, appearing like a claw clutching a ball.

Figure 1.

Truncal lichen nitidus exhibiting koebnerization in a pediatric patient.

Figure 2.

Lichen nitidus with the “ball and claw” appearance on low power.

Diagnosis confirmation

The clinical differential diagnosis includes many other lichenoid eruptions, notably lichen planus; in fact, some consider it a variant of lichen planus, and there are reports of both conditions coexisting in the same patient. Characteristically, lichen planus lesions are more violaceous, larger in size, more pruritic, and exist more often on mucous membranes and in a broader patient age range than lichen nitidus, although the two may present very similarly. Also, biopsy findings in lichen planus typically show a classic lichenoid infiltrate without the aforementioned claw/ball features of lichen nitidus.

Immunohistochemically, the dermal infiltrate in lichen planus is composed of T-helper cells, while that in lichen nitidus is a mixed population of lymphocytes. Additionally, direct immunofluorescence of lichen nitidus does not show the immunoglobulin or complement deposits typically found in lichen planus.

Other conditions in the clinical differential diagnosis are verruca plana, Bowenoid papulosis, papular sarcoidosis, and lichen amyloidosis, all of which can be easily distinguished by histopathologic features.

Who is at Risk for Developing this Disease?

Lichen nitidus typically presents in children and young adults, equally in both genders and all races in most studies, although certain clinical variants, such as the generalized form, can occur preferentially in females. Also, older adult patients can present with an acral form, and an actinic variant can exist in darker-skinned patients. No consistent genetic or inheritance pattern has been identified.

What is the Cause of the Disease?

Etiology

Pathophysiology

The etiology and pathophysiology of lichen nitidus are unknown, but some surmise a link with immune alterations typically associated with lichen planus and atopy.

Systemic Implications and Complications

There are no consistent systemic associations with lichen nitidus. Although small series and isolated reports note possible links to atopy, amenorrhea, Crohn's disease, Down syndrome, HIV, and juvenile chronic arthritis, particularly in generalized and recurrent cases, the condition does not typically warrant systemic workup. If coexistent with lichen planus, a gastrointestinal and infectious history and review of systems should be sought.

Treatment Options

Table I. Treatment options for lichen nitidus

Table 1.

Treatment options for lichen nitidus
Medical Treatment Surgical Procedures Physical Modalities
Topical corticosteroidsTopical tacrolimusTopical dinitrochlorobenzene  No surgical therapies reported PUVANB-UVB
Systemic corticosteroidsAntihistaminesSystemic retinoidsCyclosporineLevamisoleItraconazoleIsoniazid

Optimal Therapeutic Approach for this Disease

The clinical course of lichen nitidus is unpredictable, but it can spontaneously remit; in fact, one series reported that 69% of cases resolve within 1 year. Therefore, if asymptomatic, the first option would be observation/no treatment.

Topical corticosteroids can sometimes expedite lesion resolution and relieve pruritus if present. The dosing regimen and steroid potency would depend on body site and surface area involved due to risk of atrophy, but typically twice daily application would be appropriate, similar to that used in common atopic dermatitis protocols (eg, hydrocortisone 2.5% cream or ointment twice daily for 2 weeks in atrophy-prone sites, or triamcinalone 0.1% cream or ointment twice daily for 2 weeks for other body sites.

Steroid-sparing options, like topical tacrolimus applied twice daily for 4 weeks (both the 0.03% and 0.1% preparations) have been implemented with some success, particularly for genital lesions.

Other reported topical treatment options include dinitrochlorobenzene, a potent contact-sensitizing agent, which in one report, applied in 0.1% concentration (after sensitization with the 1% form, similar to verruca treatment protocols) every 2weeks, resulted in lesion resolution within 2 to 4 months. This option has not been broadly reported, and the risk of symptomatic dermatitic eruptions must be considered, particularly if the patient is atopic.

Systemic options, usually reserved for generalized or particularly pruritic or recalcitrant cases, include corticosteroids, dosed variably based on patient comorbidities and weight, and antihistamines as needed for pruritus. Systemic retinoids are reported to improve severe palmoplantar cases, for example, acitretin 0.5mg/kg per day for 3 to 6 months or isotretinoin (0.1 to 0.3mg/kg per day) for longer periods of time. Low-dose cyclosporine 2mg/kg per day tapered for a 1- to 3-month course has also been shown to be effective. Anecdotal improvement with antimicrobials levimasole, itraconazole, or isoniazid has been reported as well.

Narrowband UVB has been used for generalized cases twice weekly, usually only requiring 2 to 3 months of therapy. Oral psoralen with UVA is scantly reported as treatment for generalized lichen nitidus, but topical psoralen in an acral or body bath preparation could also be utilized in regimens similar to those used for other lichenoid eruptions.

Treatment options for lichen nitidus are summarized in the Table I.

Patient Management

In most cases, a maximum of a few months of therapy, whether topical or systemic, leads to some improvement or spontaneous resolution of lichen nitidus. It typically does not recur, but when associated with atopy, lichen planus, or actinic accentuation, there are possible seasonal and stress-related triggers. With its heterogeneous course, however, particularly if the patient is equipped with appropriate topical therapy and application instructions, little follow-up may be needed. Certainly, with more diffuse involvement, the convenience, or lack thereof, of therapies like UV light, and the safety and laboratory monitoring of systemic medications like cyclosporine, must be delineated.

Unusual Clinical Scenarios to Consider in Patient Management

Intuitively, the more diffuse the involvement, the more bothersome and possibly symptomatic the course, and likely the more intense the therapy. Other than that, it is important to recognize rare but specific clinical variants, such as generalized, palmoplantar, and actinic lichen nitidus. These can have distinctive clinical courses and treatment options.

Since lichen planus has been reported before, during, and after lichen nitidus in up to 30% of lichen nitidus cases, it is important to fully examine the patient's skin on presentation and reevaluate if the patient or parents report a recurrence or change in morphology or symptomatology. This, in turn, would necessitate lichen planus-germane workup and follow-up.

What is the Evidence?

Di Lernia, V, Piana, S, Ricci, C. "Lichen planus appearing subsequent to generalized lichen nitidus in a child". Pediatr Dermatol. vol. 24. 2007. pp. 453-5.

(This case report describes generalized lichen nitidus occurring before lichen planus in the same patient; it also outlines the heterogeneous association between the two.)

Dobbs, CR, Murphy, SJ. "Lichen nitidus treated with topical tacrolimus". J Drugs Dermatol. vol. 3. 2004. pp. 683-4.

(This report outlines the steroid-sparing topical tacrolimus regimen successful in treating a case of lichen nitidus.)

Kano, Y, Otake, Y, Shiohara, T. "Improvement of lichen nitidus after topical dinitrochlorobenzene application". J Am Acad Dermatol. vol. 39. 1998. pp. 305-8.

(This case outlines a topical dinitrochlorobenzene regimen for treatment of lichen nitidus in a patient with lymphocytopenia. It is the only report using this therapy.)

Lapins, NA, Willoughby, C, Helwig, EB. "Lichen nitidus: a study of forty-three cases". Cutis. vol. 21. 1978. pp. 634-7.

(This study summarizes the natural history and clinical, histopathologic, and epidemiologic features of a series of lichen nitidus patients.)

Lestringant, GG, Piletta, P, Feldmann, R, Galadara, I, Frossard, PM, Saurat, JH. "Coexistence of atopic dermatitis and lichen nitidus in three patients". Dermatology. vol. 192. 1996. pp. 171-3.

(This article outlines the possible association of atopy and lichen nitidus, as a basis for further understanding the elusive pathophysiology of lichen nitidus.)

Lucker, GP, Koopman, RJ, Steijlen, PM, van der Valk, PG. "Treatment of palmoplantar lichen nitidus with acitretin". Br J Dermatol. vol. 130. 1994. pp. 791-3.

(This case demonstrates an effective protocol for using acitretin for lichen nitidus and outlines other anecdotal retinoid regimens for this condition.)

Smoller, BR, Flynn, TC. "Immunohistochemical examination of lichen nitidus suggests that it is not a localized papular variant of lichen planus". J Am Acad Dermatol. vol. 27. 1992. pp. 232-6.

(This article examines the dermal infiltrate of lichen planus and lichen nitidus to distinguish the two. )

Stankler, L. "The identity of lichen planus and lichen nitidus". Br J Dermatol. vol. 79. 1967. pp. 125-6.

(This article introduces the possible relationship between these two dermatoses, particularly that lichen nitidus may be a clinical variant of lichen planus.)

Tilly, JJ, Drolet, BA, Esterly, NB. "Lichenoid eruptions in children". J Am Acad Dermatol. vol. 51. 2004. pp. 606-24.

(This is a thorough review of lichenoid dermatoses in children, with tabular comparisons and descriptions, including summaries of clinical, histopathologic, pathogenic, and therapeutic features.)
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