Peculiar Erythematous Hyperkeratotic Lesions in 2 siblings

presented by

Henry Foong FRCP (1) Samer Ghosn MD (2)

and Jag Bhawan MD (3)

on December 11, 2004

(1) Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, PK, Malaysia

(2) Fellow in Dermatopathology, Department of Dermatology, Boston University Medical Centre, Boston, MA, USA

(3) Professor of Dermatology and Pathology, Boston University School of Medicine, Boston, MA, USA

Abstract Keratosis lichenoides chronica is a rare dermatosis characterized by a distinctive seborrheic dermatitis–like facial eruption, together with violaceous, papular, and nodular lesions on the extremities and trunk. We describe 2 siblings with KLC who had the typical features of this disease.
U.K., 10-year-old boy
8 years
Face, trunk and extremities

Presented with erythematous hyperkeratotic violaceous patches on the right thigh at the age 2 years. These subsequently spread to face, trunk, and both upper and lower extremities. No constitutional symptoms

His younger brother, Y.K. had similar problems.

Physical Examination

Patches of erythematous hyperkeratotic papules and plaques on the face, back of trunk, knees and elbows. The lesions were more prominent on extensor surfaces. No wickham’s striae were noted. Normal oral cavity and nails.


Images of the patient, U.K.

The patient and his younger brother, Y.K.

The younger brother Y.K.

Laboratory Data



2 separate punch biopsies were taken from the elbows.

The histology showed individual necrotic keratinocytes and mild superficial perivascular infiltrate. There were no frank lichenoid lymphocytic infiltrates.



Keratosis Lichenoides Chronica in childhood

Reasons Presented

For interests and therapeutic suggestions


Keratosis lichenoides chronica (KLC) is a rare disorder, with fewer than 50 cases reported in the world literature.(1) The term KLC, introduced by Margolis et al. in 1972, has been commonly used for a condition previously refered to as lichen ruber acuminatus, porokeratosis striata lichenoides, porokeratosis striata, lichen verrucosus et reticularis, lichenoid tri-keratosis, and keratose lichenoide striée.

KLC is characterized by a distinctive seborrheic dermatitis–like facial eruption, together with violaceous, papular, and nodular lesions on the extremities and trunk typically arranged in a linear and reticulate pattern.

It is considered by some to be a variant of lichen planus because of similar clinical and histological findings.

Treatment is difficult. Topical steroids, tar and calcipotriol has been tried with variable success. Oral isotretinoin has been found to be successful in one case.


1. Arata J et al. Peculiar facial erythemato squamous lesions in two siblings with cyclical summer improvement and winter relapse: a variant of keratosis lichenoides chronica? J Am Acad Dermatol 1993 May;28(5 Pt 2):870-3 (view abstract)

2. Konstantinov K et al. Keratosis lichenoides chronica. J Am Acad Dermatol 1998;38:306-9 (view abstract)

3.Torrelo A, Mediero I, Zambrano A. Keratosis lichenoides chronica in a child. Pediatr Dermatol 1994;11:46-8.

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

Comments from Faculty and Members

Khaled El-Hushi MD, Troy, Michigan on December 11, 2004

I personally have not seen this condition before. Judging by morphology/pathology, Tazarotene, or PUVA/UVB may be worth a try?

Irwin Hametz MD, Edison, NJ, USA on December 11, 2004

Based on the clinical and histo findings, this patient may benefit from a combined topical therapy. Try Keralac lotion (urea,zinc,vit e), tetinoin cream, and low potency steoid. There should be no side effects and safe for longterm use. If we believe that this is truly a LP variant a topical immunosuppressive like Protopic or Elidel may help. Thanks for sharing with us.

Doug Johnson MD, Honolulu, HI, USA on December 12, 2004

I've never seen this entity and frankly didn't know it existed. Thank you for sharing.

Nurul Amin MD, Bangladesh on December 12, 2004

For milder cases -Retinoic Acid cream with topical steroid can do which I did. Result was good

Larry Erikson MD, Daytona Beach, FL, USA on December 13, 2004

Have they been tested for HCV? Perhaps you could try pimicrolimus cream for the seborrehic dermatitis-like lesions of face and tacrolimus ointment .1% for the lichen planus-like lesions of extremities.

Abir Saraswat MD, Lucknow, India on December 14, 2004

I have seen one patient with KLC who had a single hyperkeratotic plaque on the lateral malleolus, without the seborrheic dermatitis-like eruption. He failed to respond to intralesional steroids, topical keratolytics and calcipotriol. The 2 cases presented here appear to be fairly classical clinically, although histopathology is not quite confirmatory. I would be interested in any other reports of successful treatment of this entity.

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