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Keratosis Lichenoides Chronica: Report of a New Case, with Success of PUVA Therapy

  • Author(s): Ghislain, Pierre-Dominiquer
  • Beir, Anne De
  • Creusy, Colette
  • Modiano, Philippe
  • et al.
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Keratosis lichenoides chronica : report of a new case, with success of PUVA therapy
Pierre-Dominique Ghislain MD1, Anne De Beir MD1, Colette Creusy MD2, Philippe Modiano MD1
Dermatology Online Journal 7(1): 4

1. Department of Dermatology, Saint-Philibert Hospital, Catholic University of Lille, Lomme 2. Department of Pathology Saint-Philibert Hospital, Catholic University of Lille, Lomme

Abstract

Keratosis lichenoides chronica is a rare disorder. It consists of the progressive development of asymptomatic verrucosities on the limbs and the trunk, with a partially linear distribution. We report the case of a 78-year-old woman who presented with keratotic plaques and papules of the trunk and the limbs for several years. A diagnosis of keratosis lichenoides chronica was clinically and histologically evident. PUVA-therapy was started, with excellent results.


Keratosis lichenoides chronica (KLC) is a rare disorder, characterized by asymptomatic development, over several years, of thick, rough and scaly papules on the limbs and the trunk, sometimes distributed in a linear pattern. Treatment is very difficult, and often disappointing. Topical therapies are usually ineffective. Only systemic retinoids and PUVA therapy produced some remissions. We report a new observation of KLC with a good response to PUVA-therapy which should be considered first line treatment for this rare condition.


Case Report

A 78-year-old woman of Polish origin presented in January 1998 with a six year history of a warty dermatosis localized on the thighs, upper limbs, abdomen and upper back (Figure 1). Face, palms and soles were spared as were mucous membranes, hair and nails. The asymptomatic dermatosis consisted of many firm red or brown papules, with a rough keratotic surface. The surrounding skin appeared normal. In the beginning, these papules were well individualized and presented in a linear distribution, especially on the thighs.


Figure 1
Figure 1. View of the abdomen demonstrating keratotic papules in linear distribution.

No family history of a similar dermatosis was found. The patient had a medical history of ischemic cardiopathy with myocardial infarction and thyroid insufficiency. Current medications included digoxin, amiodarone, bumetanide, losartan, levothyroxine and fluindione. Clinical chemistry and hematological investigations did not show any abnormality.

Histology showed orthokeratotic hyperkeratosis, with neither spongiosis nor exocytosis. There was an irregular acanthosis. In the upper dermis, there was a lymphocytic infiltrate with a few eosinophils. The infiltrate was mostly superficial with some diffuse areas. Within the areas of infiltrate melanin pigment was observed (Figure 2). Colloid bodies could be observed near the dermo-epidermal junction. Intra-epidermal keratotic plugs (Figure 3), with thinning of the stratum spinulosum, were locally noted.


Figure 2Figure 3
Figure 2. Mononuclear infiltrate in the upper dermis
Figure 3. Other aspect of the epidermis

PUVA-therapy was started twice per week, for a total of twenty-six sessions (86.5 Joules). At the end of the treatment, the keratotic lesions had resolved; the skin was smooth. The only traces left were brown halos. The patient was extremely satisfied by the convenience of the treatment and by its cosmetic result. After two years, there was only a minimal relapse, which did not require re-treatment.


Discussion

The term keratosis lichenoides chronica was proposed by Margolis in 1972 to describe this disorder which was first described at the end of the nineteenth century (Kaposi 1895, Nekam 1938). It is a disorder which involves the limbs and the trunk, with relative symmetry. Its areas of predilection are the dorsal surfaces of thighs and forearms, but it can also extend to the neck and the feet or become almost generalized. Clinically, keratotic papules disposed in parallel lines or small networks can be seen. This pattern explains the old terms proposed for this disorder, such as "lichen ruber acuminatus verrucosus et reticularis" and "porokeratosis striata lichenoides". The elementary lesions can converge to form warty plaques. These keratotic structures are dry, purple to brown, and sometimes telangiectatic. The papules of the trunk are classically thinner. Moreover, in 75% of the cases, an erythematosquamous eruption on the mediofacial area is found, mimicking seborrheic dermatitis.[1,2] In 50 % of cases, the disorder involves the oral or genital mucous membranes, presenting as ulcerations, infiltration or inflammation.[2] The ocular area may be involved [3], as in the case initially described by Nekam.[4] Extension to the palmo-plantar zones is often reported, with an involvement of the nails in 30% of cases.[1,2] The disorder which does not appear to have a sexual predisposition, has been described in patients from adolescence to adulthood, with rare childhood cases.[1]

Histologically, the local parakeratotic hyperkeratosis forms some intra-epidermal keratotic plugs, which can sometimes be bulky. The layer of Malpighi is often atrophic. The basal layer is often widely liquefied, with the presence of many colloid bodies. The upper dermis is occupied by a band-like lymphohistiocytic infiltrate, with distinct lower limits. Mononuclear cells may migrate into the epidermis. The histology mimics warty lichen planus,[5] which is the main condition in the differential diagnosis. However, in keratosis lichenoides chronica lesional pattern and distribution are different and pruritus is usually absent.

Several publications reported keratosis lichenoides chronica with a visceral pathology such as hepatitis, chronic lymphoid leukemia or nephropathy, but it has not been possible to establish a link between KLC and any particular visceral disorder. In our patient, we did not find any associated pathology.

The course is chronic and progressive, extending over many years. Some elements may disappear spontaneously, leaving no scar or leaving residual pigmented atrophy.[5] Exceptionally, complete spontaneous remissions may be observed.[6]

A review of the literature shows that the treatment of the keratosis lichenoides chronica is difficult. Topical therapy is ineffective (tar, local corticosteroids).[2,7] However, a recent publication reports on a case successfully treated with topical calcipotriol.[8] Systemic steroids do not have any effect. Among the other systemic treatments tested, several observations reported partial or complete improvement with oral etretinate or isotretinoin, PUVA therapy or RePUVA.[1,3,8] Though some other cases were resistant to these same treatments, our case illustrates an excellent response to PUVA, which should be considered a useful and convenient treatment for this disorder.

References

1. Patrizi A, Neri I, Passarini B, Varotti C. Keratosis lichenoides chronica: a pediatric case. Dermatology 1995;191(3):264-7. PubMed

2. Masouye I, Saurat JH. Keratosis lichenoides chronica: the centenary of another Kaposi's disease. Dermatology 1995;191(3):188-92. PubMed

3. Schnitzler L, Bouteiller G, Bechetoille A, Verret JL. [Keratosis lichenoides chronica with mucous membrane involvement and ocular pseudo-pemphigus. Follow-up study over 18 years. Retinoid therapy (author's transl)] Ann Dermatol Venereol 1981;108(4):371-9. PubMed

4. Nekam L. Sur la question du lichen moniliforme. Presse Med 1938;51:1000-1003.

5. Degos R. Kératose lichénoïde striée. In: Degos R, Civatte J, Belaïch S. Dermatologie. Flammarion Médecine-Sciences, Paris, 1975, p. 671f-671h.

6. van de Kerkhof PC. Spontaneous resolution of keratosis lichenoides chronica. Dermatology 1993;187(3):200-4. PubMed

7. Konstantinov KN, Sondergaard J, Izuno G, Obreshkova E. Keratosis lichenoides chronica. J Am Acad Dermatol 1998;38(2 Pt 2):306-9. PubMed

8. Grunwald MH, Hallel-Halevy D, Amichai B. Keratosis lichenoides chronica: response to topical calcipotriol. J Am Acad Dermatol 1997;37(2 Pt 1):263-4. PubMed

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