Keratosis lichenoides chronica

Abstract

Keratosis lichenoides chronica (KLC) is a rare chronic disorder of keratinization, characterized by the progressive development of erythematosquamous plaques and violaceous lichenoid hyperkeratotic papules, nodules and ridges. These lesions are most commonly found in a symmetrical distribution on the trunk and extremities and display a unique linear and/or reticulate pattern. The etiology of KLC remains unknown and the eruptions tend to be refractory to treatment. We describe a case of keratosis lichenoides chronica that improved with systemic retinoids.

Keratosis lichenoides chronica: a diagnostic and therapeutic challenge.

“…Differential diagnosis includes lichen planus, pityriasis lichenoides et varioliformis acuta, mycosis fungoides and connective tissue diseases. [5][6][7] Our patient initially had skin lesions and histological and immunological findings (antineutrophil antibodies, IgG in the dermoepidermal layer) suggesting a connective tissue disease, possibly lupus erythematosus. Multiple skin biopsies from several sites were taken to reach the correct diagnosis.…”

“…KLC is a rare disorder, which had been regarded as a variant of lichen planus because both entities share similar clinical and histopathological features 1–5 . KLC typically occurs in adolescence or early adulthood.…”

“…Keratosis lichenoides chronica (KLC) is a rare progressive chronic disorder of keratinization of unknown aetiology, first described in 1895 by Kaposi, 1,2 who named the disease as lichen ruber moniliformis and lichen ruber acuminatus. Others have reported KLC under a variety of names 3–5 . Only about 50 cases of KLC have been published, mostly as single case reports.…”

“…Others have reported KLC under a variety of names. [3][4][5] Only about 50 cases of KLC have been published, mostly as single case reports. We describe a patient in whom the variability of the histopathology led to difficulty in diagnosis.…”

Keratosis lichenoides chronica in pediatric patients: A different disease?

“…9 Treatment has been disappointing in most cases; however, in the majority of reports, natural light, ultraviolet A and ultraviolet B light phototherapies, photochemotherapy, and oral retinoids (acitretin or isotretinoin) plus phototherapy have been reported as the most effective treatment modalities. 2,7,14 …”