Keratosis lichenoides chronica: The Centenary of Another Kaposi’s Disease

Abstract

In 1895, Kaposi used the term ‘lichen ruber acuminatus verrucosus et reticularis’ to describe the case of a 27-year-old woman with a linear, warty lichenoid eruption. We have reviewed the literature and found 50 cases, of which only 40 had common features reminiscent of the original description of Kaposi. These ‘authentic’ cases of keratosis lichenoides chronica (KLC) showed strong clinical and histological similarity, a uniformity indicating that KLC is an entity and is distinct from lichen planus. KLC may be associated with internal diseases such as glomerulonephritis and lymphoproliferative disorders.

Keratosis lichenoides chronica

“…The nosology of KLC is still a matter of debate [1]. Although some authors consider KLC as a distinct entity, others believe that it is a variant of lichen planus [10].…”

“…Although some authors consider KLC as a distinct entity, others believe that it is a variant of lichen planus [10]. Indeed KLC and lichen planus share many histopathological similarities but also important differences, especially in some clinical features, and in the resistance of KLC to treatment modalities effective in lichen planus [1]. Recently, it has been proposed that at least some cases of KLC may represent a particular form of lichen planus involving primarily the acrosyringium [11].…”

“…Less frequently, palmoplantar keratoderma, nail changes, oral ulcerations and gingivostomatitis, as well as genital lesions in male patients have been described. Finally, one third of patients with KLC manifests some eye involvement [1, 2]. Although spontaneous resolution has been reported [3], the disease usually runs a chronic and progressive course, and treatments are only occasionally effective.…”

“…KLC affects adults and very few cases have been reported in childhood [4, 5]. KLC has been associated with systemic diseases, especially chronic infectious diseases, kidney disorders and lymphoma [1, 6, 7, 8, 9]. Here, we report a case of KLC with skin, nail and mucosal involvement in an adult male patient with onset in childhood and a chronic course who developed a lobular panniculitis on the legs and a non-Hodgkin lymphoma.…”

Keratosis Lichenoides Chronica – a Case Report and Literature Review

“…It has been a subject of controversy whether KLC is a distinctive infl ammatory disease of the (10,41,50,53,74,77,97,86,99,107), or a transition of lichen planus to KLC, due to an increase in the number of focuses (109). Strong similarity between KLC and lupus erythematosus has been established (120).…”