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Keratosis lichenoides chronica
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Introduction
Keratosis lichenoides chronica (KLC), also known as Nekam disease, is a rare, chronic disorder characterized by erythematous to violaceous keratotic papules and plaques arranged in a linear and reticular pattern over the trunk and extremities. Patients may also have a seborrheic dermatitis-like or rosacea-like eruption on the face. KLC can be associated with oral manifestations (recurrent aphthous-like ulcers). Other manifestations, such as palmoplantar keratoderma, ocular manifestations (including blepharitis, conjunctivitis, uveitis, and iridocyclitis), alopecia, and nail dystrophy, have been reported in 20%–30% of patients. KLC has a chronic and progressive course and is often resistant to many therapies. The pathophysiology of KLC is not well understood. Histopathologically, KLC has features of interface dermatitis with lichenoid infiltrate adherent to epidermis, usually consisting of lymphocytes, histiocytes, plasma cells, and few eosinophils, as well as hyperkeratosis, focal parakeratosis, and irregular acanthosis.
Management Strategy
Treatment of KLC represents a significant challenge because of the rarity of the condition. It is also resistant to most therapies. No controlled trials are available, but many case series and case reports suggest that phototherapy and systemic retinoids , alone or in combination, may provide the best treatment option with nearly half of patients reaching complete remission. Other reported treatments include systemic steroids , sulfones , methotrexate , antimalarial agents , and ciclosporin . Topical treatments are usually ineffective. Oral retinoids (mostly acitretin) given at a dose of 0.3–0.6 mg/kg/day have been the most effective treatment for KLC.
First-Line Therapies
Keratosis lichenoides chronica: case-based review of treatment options
Pistoni F, Peroni A, et al. J Dermatolog Treat 2016; 27: 383–8.
A review of all 71 cases reported in literature between 1972 and 2014. Significant clinical response, defined as complete or partial clearance, was seen in 42% (5/12) patients treated with NB-UVB, 50% (6/12) of patients treated with PUVA, and 56.6% (17/30) of patients treated with systemic retinoids. Combination of oral retinoids plus phototherapy resulted in complete remission in 50% (5/10) of patients treated with Re-PUVA and 100% (3/3) of patients treated with Re-UVB.
Keratosis lichenoides chronica: characteristics and response to acitretin
Avermaete A, Kreuter JA, Stücker M, et al. Br J Dermatol 2001; 144: 422–4.
A case report describing a patient with KLC who failed treatment with systemic and topical corticosteroids and PUVA. When give acitretin 0.5 mg/kg, all his lesions flattened considerably after 3 weeks and completely resolved after 6 months of therapy.
A case of pediatric keratosis lichenoides chronica with unusual presentation of severe oral erosions
Nasimi M, Azizpour A, Lajevardi V, et al. JAAD Case Rep 2018; 6; 4: 267–9.
A pediatric patient with KLC with oral aphthous-like ulcers was treated with oral acitretin at a dose of 10 mg daily. After 2 months of retinoid therapy, the keratotic plaques on her extremities flattened, and the erythema was much reduced, but not the oral lesions.
Keratosis lichenoides chronica and eruptive keratoacanthoma-like lesions in a patient with multiple myeloma
Marzano AV, Bellinvia M, Caputo R, et al. J Eur Acad Dermatol Venereol 2005; 19: 129–33.
A case report of patient who developed KLC and multiple keratoacanthomas. Treatment with oral acitretin 25 mg daily for 3 months lead to regression of the keratoacanthomas and good improvement of the KLC skin lesions.
Keratosis lichenoides chronica mimicking verrucous secondary syphilis
Jayaraman AG, Pomerantz D, Robinson-Bostom L. J Am Acad Dermatol 2003; 49: 511.
A case report of a a patient who developed thick keratotic lesions of KLC. Treatment with 25 mg of acitretin daily resulted in partial improvement in his skin lesions.
Keratosis lichenoides chronica treated with acitretin plus narrowband ultraviolet B phototherapy
Koseoglu RD, Sezer E, Yuksek J. J Dermatol 2008; 35: 172–4.
A case of a 19-year-old male patient with KLC. Complete clearance of the facial lesions and regression of the body lesions noted 2 months after initiation of treatment with oral acitretin (1 mg/kg/day) plus narrowband ultraviolet B (NB-UVB) treatment, thrice weekly. There was no recurrence of the lesions during a 12-month follow-up period.
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