Keratosis lichenoides chronica

ARTICLE

A 60-year old Japanese man was at first seen at a local public hospital 5 years previously because of a 6 month-history of non-pruritic, multiple, firm erythematous papules and nodules on his lower legs. They gradually increased in number and size. His family history showed no similar skin conditions. He had had hypertension for the past 10 years, but had not been under medication. He had been treated with various therapeutic modalities including topical steroids, PUVA, systemic administration of diphenylsulfone, cyclosporine, prednisone, etretinate, and griseofulvin. Since, despite these treatments, the eruptions gradually enlarged and increased in number, he was referred to our hospital, and was hospitalized in July 1994. Physical examination on admission showed multiple papules, some arranged in a linear pattern (Fig. 1), and verrucous nodules, some of which had centrally keratin-filled craters as shown in Fig. 2. Several verrucous keratotic eruptions were also found on the soles. Examinations of the internal organs did not disclose any particular changes. Although the PPD test was strongly positive, no active tuberculous lesion was found. Serum antibody against human immunodeficiency virus (HIV) was negative. His total peripheral white blood cell count was 5,000 cells/mm³, with the ratio of CD4/CD8 was 1.2. Skin biopsy specimens taken from the verrucous nodules on the left thigh and over the Achilles' tendon showed acanthotic epidermis consisting of hypertrophic epidermal cells with pale, eosinophilic and glassy cytoplasms, wedgelike hypergranulosis, focal liquefaction degeneration, and exocytosis of mononuclear cells (Fig. 3).

Keratosis lichenoides chronica

Comments

The term of keratosis lichenoides chronica (KLC) was coined by Margolis et al. in 1972 [2]. It is characterized by hyperkeratotic papules, which are arranged in a reticular [2, 3] or linear pattern [4] and some of which coalesce to form warty hypertrophic plaques. Similar conditions have been described under many different terms; lichen ruber acuminatus verrucosus et reticularis [2], keratose lichenoide striee, porokeratosis striata, lichenoid trikeratosis, Neham's disease, generalized Kyrle's disease, and hypertrophic lichen planus. Thus, there has been confusion about this rare clinical entity. In 1995, one hundred years after the original description by Kaposi [2], Masouyé and Saurat clarified this confusion by presenting evidence that indicated KLC to be an entity [5].

The present case showed the unique clinical features of KLC. Differential diagnosis should be made from the two variants of multiple keratoacanthomas. Our case more resembled eruptive keratoacanthomas (Grzybowski type) than multiple self-healing epitheliomas of the skin (Ferguson-Smith type). However, the lesions in our case were not self-healing and the size of nodules, 2 to 3 mm, was much larger than that of papules usually found in eruptive keratoacanthoma [6]. The main histological findings in our case were liquefaction degeneration and band-like infiltrates of dense mononuclear cells (Fig. 4), resembling those of lichen planus. However, the epidermal acanthosis, consisting of cells with pale, eosinophilic, glassy cytoplasm (Fig. 3), reminiscent of solitary keratoacanthoma, is not characteristic of lichen planus.

Retinoids, with or without irradiation, has been reported to be effective in the patients with KLC [5]. Although our case was treated with many therapeutic modalities, we encountered difficulty in treating the present patient. We also tried cryosurgery, intralesional injection of triamcinolone acetonide or of bleomycin, with marginal effectiveness. Although some nodules flattened slightly, these treatments were discontinued because of the patient's intolorance to pain. Thereafter, we just surgically excised some of the large nodules.

CONCLUSION

REFERENCES

1. Margolis MH, Cooper GA, Johnson SAM. Keratosis lichenoides chronica. Arch Dermatol 1972; 105: 739-43.

2. Kaposi M. Lichen ruber acuminatus et lichen ruber planus. Arch Dermatol Syphilis (Berl) 1895; 31: 1-32.

3. Braun-Falco O, Bieber T, Heider L. Keratosis lichenoides chronica: Krankheitsvariante oder Krankheitssentitat? Hautarzt 1980; 40: 614-22.

4. Chapman RS. Lichen verrucosus and reticularis. Dermatologica 1971; 142: 363-73.

5. Masouyé I, Saurat JH. Keratosis lichenoides chronica: the centenary of another Kaposi's disease. Dermatology 1995; 191: 188-92.

6. Jaber PW, Cooper PH, Greer KE. Generalized eruptive keratoacanthoma of Grzybowski. J Am Acad Dermatol 1993; 29: 299-304.