| 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. |
Patient |
U.K., 10-year-old boy |
Duration |
8 years |
Distribution |
Face, trunk and extremities |
| History |
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 |
Images of the patient, U.K.
The patient and his younger brother, Y.K.
The younger brother Y.K. |
| Laboratory Data |
|
| Histopathology |
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.
|
| Diagnosis |
Keratosis Lichenoides Chronica in childhood |
| Reasons Presented |
For interests and therapeutic suggestions
|
| Questions |
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.
|
| References |
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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