Keratosis Lichenoides Chronica: Proposal of a Concept

FROM DERMATOPATHOLOGY: PRACTICAL & CONCEPTUAL
Keratosis Lichenoides Chronica: Proposal of a Concept
Almut Böer, MD
Abstract: It has been a subject of controversy whether keratosis
lichenoides chronica (KLC) is a distinctive inflammatory disease
of the skin or whether it represents a manifestation of another
well-known disease, such as lichen planus, lupus erythematosus,
or lichen simplex chronicus. In search of clear criteria for
diagnosis of KLC the entire literature pertinent to the subject
was studied and findings clinical and histopathologic as they
were telegraphed in them were compared with a patient of my
own experience. Review of the literature reveals more than 60
patients in whom the diagnosis of KLC was made. Three
categories emerge based on whether the findings presented in a
particular article (1) do not permit any diagnosis to be rendered;
(2) do allow a diagnosis specific to be made, such as of lichen
simplex, lichen planus, or lupus erythematosus; or (3) do not
correspond to any disease well defined, such as lichen simplex,
lichen planus, lupus erythematosus, but seem to show attributes
morphologic, clinically and histopathologically, that are repeatable. Patients diagnosed as having KLC obviously represent a
potpourri of different diseases, the most common of them being
lichen simplex chronicus, lichen planus, and lupus erythematosus. Fewer than 25 patients reported on, however, presented
themselves with lesions very similar to one another clinically,
namely, an eruption that involved the face in a manner
reminiscent of seborrheic dermatitis and with tiny papules on
the trunk and extremities, which assumed linear and reticulate
shapes by way of confluence of lesions. Individual papules were
infundibulocentric and acrosyringocentric. Findings histopathologic were those of a lichenoid interface dermatitis affiliated with
numerous necrotic keratocytes and covered by parakeratosis
housing neutrophils in staggered fashion. These patients seem to
have an authentic and distinctive condition that is exceedingly
rare. In conclusion, the diagnosis of KLC should be made only
for patients who present themselves with features clinical and
findings histopathologic that resemble closely those of what is
summarized in this article under category 3.
Key Words: Keratosis lichenoides chronica, lichenoid dermatitis,
criteria for diagnosis, histopathology, lichen ruber, lupus
erythematosus, prurigo simplex, Moritz Kaposi
(Am J Dermatopathol 2006;28:260–275)
From the Dermatologikum Hamburg, Germany.
This article originally appeared in Dermatopathology: Practical &
Conceptual 2005;11(2):4.
Reprints: Dr. Almut Böer, Dermatologikum Hamburg, Stephansplatz 5
20354, Hamburg Germany (e-mail: [email protected]).
260
I
n dermatology, many conditions seem to be extraordinarily rare or at least they are rarely diagnosed. Our
concepts of these diseases are vague and largely based on
case reports. One of these diseases is keratosis lichenoides
chronica (KLC), which has been the subject of controversy. It has been questioned whether KLC represents
a distinctive disease entity and it was proposed that it
might be a variant of lichen planus, a generalized
verrucous epidermal nevus, or simply a manifestation of
rubbing and scratching persistently. Studying KLC in
historical perspective is an intriguing undertaking. An
extensive analysis of all articles devoted to the subject of
KLC, richly illustrated, can be found in the original
version of this article in the journal Dermatopathology:
Practical & Conceptual at derm101.com.1 An abbreviated
version of that study is presented here.
HISTORICAL PERSPECTIVE
The origin of the confusion about KLC goes back
to 2 articles by M. Kaposi, one of them published in 18862
and the other in 1895.3 In 1886, Kaposi told of a patient
who presented himself with widespread papules, nodules,
and keloid-like streaks, the latter being most prominent
on the extremities, the lower part of the abdomen, and the
neck. Kaposi thought that the disease in his patient
resembled lichen planus and, therefore, he named it
‘‘lichen ruber moniliformis’’. In his article were included
drawings of findings histopathologic, but the changes
pictured bear no resemblance to those of lichen planus.
When, in 1895, Kaposi published his article titled
(translated from the German) ‘‘Once again: Lichen ruber
acuminatus and Lichen ruber planus,’’ confusion reigned
about a designation, namely, ‘‘lichen planus,’’ which was
being applied by dermatologists to more than one
condition. In his article, Kaposi attempted to separate a
condition that he called ‘‘lichen ruber acuminatus
(verrucosus et reticularis)’’ from lichen planus. He
reported on 2 patients, one of whom, a 27-year-old
woman, had an exanthem consisting of numerous tiny
papules that became confluent to assume linear and
reticular outlines (Fig. 1). Some of those lesions reminded
Kaposi of ones of lichen planopilaris because of the
infundibulocentricity of them and, to his eye, findings
histopathologic resembled those of lichen planus. Kaposi
made the same diagnosis of ‘‘lichen ruber acuminatus
(verrucosus et reticularis)’’ in another patient, a 10-yearold child, although lesions in that patient were very
different from those in the first patient he presented.
Attributes clinical pictured by Kaposi show features of
what is called atopic dermatitis and of keratosis pilaris.
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Keratosis Lichenoides Chronica
FIGURE 1. A and B, These paintings
appear in the article by Kaposi of 1895.
The condition in this patient is difficult to
classify and the drawings of findings
histopathologic shows a lichenoid infiltrate beneath an epidermis that seems to
be atrophic in the center of the lesion
and acanthotic at the periphery of it. The
lesion is covered by parakeratosis associated with remnants of inflammatory
cells, that combination arrayed in staggered fashion. The biopsy specimen that
exhibited these findings came from the
patient who was studied again, years
later, in 1936, by Nekam.3
The drawings of changes in sections of tissue cut from
biopsy specimens taken from the patients of Kaposi do
not permit a diagnosis to be made with specificity, but the
section from the 27-year-old woman shows a lichenoid
infiltrate beneath an epidermis that at the periphery of the
lesion is acanthotic and in the center atrophic. The lesion
is hyperkeratotic, being associated with parakeratosis
arranged in staggered fashion. From the attributes
pictured, it is conceivable that the 27-year-old woman
had an authentic disease typified by an exanthem
clinically and by a lichenoid dermatitis histopathologically, whereas the child seems to have had a very different
condition, to wit, ‘‘atopic dermatitis’’ in company with
keratosis pilaris. In his article of 1895, Kaposi made only
very brief reference to his previous publication in 1886
and it is likely that he, himself, did not regard the
maladies presented in both articles to be the very same
disease. Nevertheless, in many articles published by
others subsequently the terms ‘‘lichen ruber moniliformis’’ and ‘‘lichen ruber acuminatus (verrucosus et
reticularis)’’ were employed as synonyms.
In 1938, Nekam told of a woman who had been a
subject in the publication of Kaposi in 1895.4 Nekam
took note of hyperkeratosis of acrosyringia, which
prompted him to propose the name ‘‘porokeratosis striata
lichenoides’’ for the condition. In none of his photomicrographs, however, is a cornoid lamella pictured, a
finding requisite for diagnosis of porokeratosis of any
type (Fig. 2). Taking into account all the material
pictorial presented by Kaposi, in 1895 and by Nekam in
1938, it remains difficult to integrate all of the aspects.
Nonetheless, the findings histopathologic are those of a
lichenoid dermatitis different from lichen planus.
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The term ‘‘keratosis lichenoides chronica’’ was
introduced in 1972 by Margolis, when he recorded the
history, attributes morphologic, and course of a patient
who for 15 years had an asymptomatic eruption that
consisted at the outset of the eruption of discrete
perifollicular papules but, during the course of the
process, took on linear and reticular shapes (Fig. 3).5
The photomicrographs reveal a lichenoid dermatitis
affected with parakeratosis. Although the photomicrographs do not allow a diagnosis to be rendered with
confidence, the process seems to be different from both
lichen planus and lupus erythematosus.
Several articles appeared subsequent to the works
by Kaposi, Nekam, and Margolis; however, the conditions seem to represent more than one condition.6–10
Some authors who reviewed what has been written during
the past 120 years came to the conclusion that KLC
represented an inherited type of epidermolysis bullosa,
others regarded it as a variant of lichen planus, and still
others were impressed by similarities of it to lupus
erythematosus.11–13 In 1997, in the journal Dermatopathology: Practical & Conceptual, Massi, Chiarelli, and
Ackerman addressed the issue of KLC in perspective
historical and in a manner more comprehensive than had
been undertaken before.14 Massi and associates interpreted lesions in patients pictured in a number of articles
given to the subject of KLC as being brought about
factitiously entirely,2,3,5,15–26 but some lesions clinical
pictured in articles published prior to the Arbeit of Massi
and coworkers do not have the appearance of ones
created artificially. They appear to have erupted rather
than having been brought into being by trauma external.27–31 Massi et al acknowledged that some patients
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FIGURE 2. A–D, The patient of Nekam
was said by him to be the same woman
who was a subject of Kaposi in his article
of 1895. The lesions on the face and on
the palms seem to represent manifestations of an authentic disease. The findings histopathologic are those of a
lichenoid dermatitis unlike any established disease, such as lichen planus,
lichenoid discoid lupus erythematosus,
lichen striatus, or porokeratosis. Apart
from a lichenoid infiltrate, there also is
marked dilation of acrosyringia by hyperkeratosis and that seems to be the
case, too, for infundibula. Reproduced
with permission.4
FIGURE 3. A–C, The eruption in this
patient of Margolis affected the extremities predominantly. Photomicrographs show a lichenoid dermatitis
beneath an epidermis that in foci is
atrophic and in others acanthotic. Hyperkeratosis seems to consist of parakeratosis that houses a few inflammatory
cells. The lesions do not correspond to
those of lichen planus or of lupus
erythematosus, but are distinctive nonetheless. Reproduced with permission.5
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written about under the title of KLC might have had an
authentic disease underlying, such as lichen planus,
discoid lupus erythematosus, psoriasis, or pityriasis rubra
pilaris, on top of which signs of rubbing and scratching
were imposed. The authors, however, provided no clue to
which of the patients in particular might have had a
primary process pathologic that was complicated by
trauma external. Neither was comment made by Massi,
Charelli, and Ackerman about the attributes clinical and
histopathologic pictured in several reports about KLC
that do not seem to correspond to any well-defined
disease, but were similar to each other and were marked
by a distinctive lichenoid dermatitis and they denied that
some patients diagnosed with KLC might have an
authentic inflammatory disease. In the very same issue
of the journal can be found an article devoted to KLC by
Ruben and LeBoit32 and another by Aloi and Tomasini.33
Ruben and LeBoit told of their experience with 4 patients
diagnosed by them as KLC.32 Contrary to Massi et al,
Ruben and LeBoit stated straightforwardly that they
conceived of KLC as an authentic pathologic process.
Unfortunately, the authors showed only a single photograph of lesions clinical and photomicrographs of but one
section of tissue from a biopsy specimen taken from that
patient. The features clinical are subtle, but seem to
consist of small papules that assumed shapes linear and
reticular by way of confluence of them. The findings
histopathologic are those of a lichenoid dermatitis
centered around acrosyringia of eccrine units, peppered
with neutrophils. These changes do not correspond
precisely to any disease already established and they are
dissimilar from both lichen planus and lupus erythematosus. The authors reviewed the literature dedicated to the
subject of KLC and interpreted what was presented in 26
articles as being consonant with a specific disease but they
failed to indicate to which articles in particular they were
referring to Based on their own observations, Ruben and
LeBoit were the first who attempted to set forth criteria
for diagnosis of KLC. They missed, however, the
opportunity to demonstrate convincingly to readers that
all four patients studied by them really had the same
condition. In their article the authors announced that
they planned a scientific article on KLC about all of their
patients. Tomasini and Aloi, too, had made the diagnosis
of KLC in four patients and, based on their experience
personal, concluded that KLC is a variant disseminated
of an inflammatory verrucous epidermal nevus.33 The
authors illustrated their work with pictures of lesions
clinical and with photomicrographs of sections of tissue
from different patients. Alas, the authors did not attempt
correlation clinicopathologic, patient by patient. The
lesions depicted by them do not seem to represent
manifestations of a single process pathologic.
Even in most recent publications confusion reigns
about criteria for diagnosis of KLC. What follows is an
analysis of each case published under the diagnosis of
KLC or one of its synonyms in search of features in
common among them, both clinically and histopathologically.
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Keratosis Lichenoides Chronica
CLINICOPATHOLOGIC CORRELATION OF
MORPHOLOGIC ATTRIBUTES IN MORE THAN
SIXTY PATIENTS DIAGNOSED WITH KERATOSIS
LICHENOIDES CHRONICA
Review of the literature pertinent to the subject of
KLC uncovers more than 60 patients in whom was made
the diagnosis of KLC or of one of the designations that
have come to be used synonymously with it, among those
being Nekam’s disease, lichen verrucosus et reticularis,
lichenoid tri-keratosis, keratose lichenoide strieé, porokeratosis striata, Morbus moniliformis, and lichen ruber
moniliformis.2–66 Thirteen of these reports were published
subsequent to the work of Massi, Chiarelli, and Ackerman in 1997. A summary of all patients studied for this
article can be found in Table 1 and 2.
In many articles dedicated to the subject of KLC,
the material pictorial and descriptions of attributes
clinical and histopathologic do not allow a diagnosis to
be made with certainty. Analysis critical of the material
provided, however, suggests that more than 10 different
diseases have been published under the title of KLC,
among them lichen simplex chronicus, lichen planus,
lupus erythematosus, and epidermolysis bullosa dystrophica, as well as others. The hodge-podge of conditions
that have been called KLC has made it almost impossible
to forge clear criteria for diagnosis of a single distinctive
disease. Impediments to comprehension of the subject are
many, but the most serious are the small number and the
bad quality of pictorial material printed in articles, the
lack of precise correlation clinicopathologic in many
publications, the misinterpretation of features clinical and
findings histopathologic pictured in them, and the fact
that only rarely did authors have their own experience
personal with more than a single patient, the latter
critique being applicable to me. Another difficulty is that
shapes artificial of lesions pictured may come into being
by way of a Köbner phenomenon in a variety of diseases,
among them being psoriasis and lichen planus. Lesions
evoked by trauma external may assume shapes artificial
that look similar to each other even though the conditions
underlying are different from one another. As but one
example, lesions striate on flexor aspects of extremities
were for long believed to be diagnostic of KLC, but those
may be encountered in conditions unrelated entirely such
as lichen simplex chronicus, lichen planus, and psoriasis.
Unfortunately, peer reviewers for journals often fail to
prevent reports from being published in which the
authors clearly came to a wrong diagnosis. Considering
the lack of precise criteria in the early publications
devoted to the subject of KLC, it is surprising that as a
rule textbooks of dermatology and dermatopathology
present the condition as if a clear concept of it existed.
From review of the entire literature devoted to the
subject of KLC, 3 categories emerge based on whether the
findings presented in a particular article:
(1) Do not permit any diagnosis to be rendered
(2) Do allow a diagnosis specific to be made, such as of
lichen simplex, lichen planus, or lupus erythematosus
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TABLE 1. Summary of Findings in All Patients diagnosed With KLC (or a synonym of it) Reported on as of 2005, Signs and
Symptoms and Illustrations in Articles
Author
Year
#
Sex
Age
Duration
Symptoms
Kaposi
Nekam
1895
1
F
27
7 years
Pruritus
Kaposi only
1936
1895
1
10
9 years
Miller
Meara
Bureau
Chapman
1944
1956
1970
1971
1
1
1
1
F
M
12 years
2 years
F
38
27
60
53
Schnitzler
Margolis
Grupper
Pinol-Aguade
Degos
Mac Donald
Menter
1972
1972
1973
1974
1974
1974
1976
1
1
1
1
1
1
1
1
M
M
M
M
M
M
F
F
79
42
36
15
43
61
55
26
1
1
M
M
43
30
6 years
2 years
30 years
24 years
15 years
2 years
18 month Pruritus
Pruritus
Kint
Petrozzi
Barriere
Duperrat
Chandon
Degos
1976
1976
1977
1977
1977
1977
1
1
1
1
1
1
F
F
M
F
M
M
54
49
43 years
15 years
51
35
45
4 years
6 years
days
Nabai
Lang
1980
1981
1
1
F
M
37
24
21 years
20 years
Panizzon
1981
1
M
33
14 years
Schnitzler
1981
1
49
18 years
Balus
1982
1
M
50
28 years
Ryatt
Kersey
Raynaud
Mehregan
Wätzig
1982
1982
1983
1984
1986
1
1
1
1
1
M
M
F
F
F
16
18
2
55
37
3 years
1,5 years
46 years
13 years
Duschet
David
Fraitag
1987
1989
1989
1
1
1
F
M
F
34
65
74
15 years
30 years
48 years
Braun-Falco
Skorupka
Arata
1989
1992
1993
Stefanato
van de Kerkhof
Patrizi
Amichai
1993
1993
1995
1995
1
1
1
1
1
1
1
1
F
F
F
M
F
M
M
M
61
41
7
8
32
42
4
53
2 months Pruritus
8 years
7 years
8 years
32 years
1 year
2 years Pruritus
4 years
Ezzine-Sebai
Marschalko
1996
1996
1
1
F
M
23
41
20 years
2 years
Aloi
1997
1
1
M
F
56
52
2 years
2 years
1
1
F
M
14
54
4 years
49 years
264
Pruritus
Pruritus
Sites
Clinical Pictures
Photomicrographs
Face, trunk, and
extremities
Yes
Yes
Face, trunk, and
extremities
Face, extremities
Arms and feet
Disseminated
Extremities, mucous
membranes
Extremities, face
Mucosa, hands
Extremities
Mucosa, disseminated
Extremities
Extremities and genitalia
Face and extremities
Face, trunk, extremities,
mucous membranes,
eyes
Extremities
Face, extremities, mucous
membranes
Legs, trunk
Extremities
Extremities
Extremities and face
Extremities
Extremities, mucous
membranes
Extremities, face
Face, trunk, mucous
membranes
Extremities, mucous
membranes, nails
Extremities, nails, eyes,
genitalia
Face, nails, extremities,
mucous membranes
Face, extremities
Trunk, extremities
Face, extremities
Legs, trunk
Face, extremities, mucous
membranes
Trunk and extremities
Disseminated
Face, trunk, and
extremities
Trunk, extremities
Extremities and face
Face and extremities
Face
Disseminated
Hands and feet
Face and extremities
Forearms, abdomen,
thighs
Disseminated
Trunk, extremities, and
mucous membranes
Yes
No
Yes
No
No
Yes
No
No
No
Yes
Yes
No
Yes
No
Yes
Yes
Yes
Yes
Yes
No
Yes
No
Yes
No
No
No
Yes
Yes
Yes
No
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
No
Yes
Yes
Yes
Yes
Yes
No
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
No
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
No
Yes
Yes
Yes
Yes
Yes
Yes
No
No
Yes
The four patients
cannot be
differentiated
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Keratosis Lichenoides Chronica
TABLE 1. (continued )
Author
Year
#
Sex
Age
Duration
LeBoit
1997
1
Grunwald
Konstantinov
1997
1998
1
1
1
1
1
M
M
53
26
4 years
6 month
Kossard
Chikama
Thieulent
Lombardo
1998
1999
1999
2000
1
1
1
1
F
M
M
M
70
60
50
41
7 years
5 years
4 years
40 years
Taberner
Haas
2001
2001
1
1
F
M
37
25
4 years
1 year
Mevorah
Avermaete
2001
2001
1
1
M
M
26
31
16 years
6 years
Remling
Nijsten
Jayaraman
2002
2002
2003
1
1
1
M
F
M
51
48
47
10 years
20 years
5 years
Miller
Vernassiere
Bauer
2004
2004
2005
1
1
1
M
M
M
54
35
90
8 years
months
10 years
Symptoms
Sites
Clinical Pictures
Photomicrographs
The four patients
cannot be
differentiated
Pruritus
Pruritus
Pruritus
Pruritus
Extremities
Trunk and extremities,
face
Trunk, extremities, nails,
Extremities
Palms and soles, nails
Trunk, extremities,
mucous membranes
Trunk and extremities
Extremities, scalp,
mucous membranes
Disseminated, alopecia
Extremities, face, mucous
membranes
Hands and feet
Extremities, trunk, face
Trunk, extremities, scalp,
face
Trunk, extremities
Trunk, extremities
Trunk, extremities
Yes
Yes
No
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
Yes
No
Yes
Yes
Yes
Yes
No
Yes
Yes
Yes
Yes
Yes
Yes
Yes
KLC, keratosis lichenoides chronica.
TABLE 2. Summary of Findings in all Patients Diagnosed With KLC (or a synonym of it) Reported on as of 2005, Diagnoses,
Treatment, and Effect of Treatment
Author
Kaposi
Nekam
Year
1895
1936
Kaposi only
1895
Miller
1944
Meara
Bureau
Chapman
1956
1970
1971
Schnitzler
Margolis
Grupper
Pinol-Aguade
Degos
Mac Donald
1972
1972
1973
1974
1974
1974
Menter
1976
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Author’s Diagnosis
Lichen ruber acuminatus
(verrucosus et reticularis)
Lichen ruber acuminatus
(verrucosus et reticularis)
Lichen verrucosus et reticularis,
Porokeratosis striata (Nekam),
Morbus moniliformis (Wise and
Rein)
Porokeratosis striata
KLC
Lichen verrucosus et reticularis
Lichen verrucosus et reticularis
KLC
KLC
KLC
Lichenoid Tri-Keratosis
Keratose lichenoide striee
Lichen verrucosus et reticularis of
Kaposi (porokeratosis striata of
Nekam)
Lichen verrucosus et reticularis
Kaposi
My Diagnosis
Response to Treatment
Probably KLC
Not known
Not known
Atopic dermatits and
keratosis pilaris
KLC
Not known
Not known
X-ray, Vitamin A
No effect
Not known
Possibly KLC
Probably Lichen planus
Not known
Psoriasis (Reiter’s disease)
Probably KLC
Not known
Probabaly porokeratosis
Probably KLC
Probably KLC
Not known
Not known
Not known
Not known
Methotrexate
Various
Not known
Not known
Not known
Not known
Not known
Not known
Not known
Not known
Improvement
No effect
Not known
Not known
Not known
Not known
Probably lupus
erythematosus
Sulfadiazin and
pyrimethamin for
toxoplasmosis
Sulfadiazin and
pyrimethamin for
toxoplasmosis
Cotrimoxazol
Improvement
Lichen verrucosus et reticularis
Kaposi
Not known
Lichen verrucosus et reticularis
Kaposi
Lichen simplex
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Treatment
No effect
Improvement
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TABLE 2. (continued )
Author
Year
Author’s Diagnosis
Kint
1976
KLC
Petrozzi
Barriere
1976
1977
KLC
Keratose lichenoide striee
Duperrat
Chandon
Degos
Nabai
Lang
1977
1977
1977
1980
1981
Keratose lichenoide striee
KLC
KLC
KLC
Panizzon
Schnitzler
Balus
Ryatt
Kersey
Raynaud
Mehregan
Wätzig
1981
1981
1982
1982
1982
1983
1984
1986
KLC
KLC
KLC
KLC
KLC
KLC
KLC
Lichen ruber moniliformis/
Epidermolysis bullosa
Duschet
1987
KLC
KLC
Probably L. simplex
Not known
Not known, perhaps
lichenoid LE
Probably KLC
KLC
Not known
Probably Lichen planus
Probably KLC
Perhaps KLC
Not known, maybe LE
Subepidermal blistering
disease/Epidermolysis
bullosa
Probably KLC
David
Fraitag
Braun-Falco
Skorupka
Arata
1989
1989
1989
1992
1993
Stefanato
van de
Kerkhof
Patrizi
1993
1993
KLC
KLC
KLC
KLC
KLC
KLC
KLC
Atypical Nekam’s disease
Possibly Lichen planus
Possibly KLC
KLC
Not known
KLC
Not known, possibly LE
Not known
Not known
1995
KLC
Amichai
1995
KLC
Ezzine-Sebai
Marschalko
Aloi
1996
1996
1997
KLC
KLC
KLC
Not known, some features of None
verrucae vulgares
Probably Pityriasis
Erythromycin
lichenoides
Probably KLC
Not known
Severe atopic dermatitis
Not known
Not known
Corticosteroids topically and
systemically, PUVA
KLC in one patient at least Not known
ILVEN in another patient
Not known
Not known
Not known
Not known
Not known
KLC in one patient at least Not known
Not known
Not known
Not known
Not known
Not known
Not known
Not known
Calcipotriol
KLC
Isotretinoin
KLC
Not known
Lichen planus, hypertrophic Various
Probably Lichen planus
Not known
Not known
CHOP for Mantle cell
lymphoma
Lichen planus, Köbner
Various
phenomenon
Lichen planus
Neotigason and Bath PUVA
Not known
Retinoids
Probably KLC
Acitretin
Lichen simplex
Bath PUVA
Probably KLC
Acitretin
Not known
Retinoids
Probably Lichen planus
Not known
Possibly KLC
Not known
Hypertrophic lichen planus Steroids
LeBoit
1997
Grunwald
Konstantinov
Kossard
Chikama
Thieulent
Lombardo
Taberner
1997
1998
1998
1999
1999
2000
2001
KLC
KLC
KLC, LP variant
KLC
KLC
KLC
Haas
2001
KLC
Mevorah
Avermaete
Remling
Nijsten
Jayaraman
Miller
Vernassiere
Bauer
2001
2001
2002
2002
2003
2004
2004
2005
KLC
KLC
KLC
KLC
KLC
KLC
KLC
KLC
KLC
266
My Diagnosis
Subepidermal blistering
disease
Probably KLC
Probably L simplex
opsoriasis
Treatment
Response to Treatment
Not known
Not known
Various
No effect
Not known
Not known
Corticosteroids
Levamisol
Not known
Not known
Improvement
Improvement
PUVA
Retinoids
Retinoids
Retinoids
PUVA
Not known
Tigason
Not known
Improvement
Improvement
Improvement
Improvement
Improvement
Not known
Improvement
Not known
Corticosteroids topically and No effect
systemically, PUVA
Retinoids and PUVA
Improvement
Etretinat
Improvement
Etretinat
No effect
Tigason
Complete resolution
Not known
Not known
Not known
Not known
Not known
Not known
Not known
Not known
r
Spontaneous resolution
Complete resolution
Not known
Not known
No effect
Not known
Not known
Not known
Not known
Not known
Not known
Not known
Not known
Improvement
Improvement
Not known
No effect
Not known
Improvement
No effect
Improvement
No effect
Improvement
Improvement
Improvement
Improvement
Not known
Not known
Improvement
2006 Lippincott Williams & Wilkins
Am J Dermatopathol
Volume 28, Number 3, June 2006
Keratosis Lichenoides Chronica
FIGURE 4. A–D, These pictures come
from an article by Duperrat in 1977.
Lesions were present on the face and
extremities, individual ones being tiny
papules that assumed a linear and a
netlike outline by way of confluence of
them. Histopathologically, there is uneven acanthosis and plugging of infundibula by parakeratosis. The composition
of the infiltrate in the papillary dermis
cannot be determined with surety. Reproduced with permission.19
FIGURE 5. A–D, These images were
published in an article by Fraitag in
1989. Individual lesions tended to become confluent to form a netlike pattern. At sites of long-standing trauma
mechanical, like on the foot, lesions
became keratotic plaques. The photomicrograph shows an interface dermatitis with atrophy in loci and clusters of
necrotic keratocytes. Reproduced with
permission.27
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FIGURE 7. A and B, The findings shown here come from the
patient of Ruben and coworkers reported on in 1997.
Clinically, the lesions are tiny papules that became confluent
to create shapes linear and arcuate. Histopathologically, the
changes are those of a lichenoid dermatitis with a thinned
epidermis and parakeratosis.32
Category 1
FIGURE 6. A–E, This patient reported on by Skorupka in 1992
has lesions on the face and the extremities, assuming a
reticular pattern and formed by a lichenoid dermatitis with
atrophy, jagged acanthosis, and striking hyperkeratosis, most
of it parakeratotic. Necrotic keratocytes are situated in the
basal layer. Reproduced with permission.31
(3) Do not correspond to any well-defined disease, such as
lichen simplex, lichen planus, lupus erythematosus, but
seem to show attributes morphologic, clinically, and
histopathologically, that are repeatable.
268
No small number of patients reported on with a
diagnosis of KLC display features morphologic very
dissimilar from one another and different also from what
was pictured in the seminal articles of Kaposi, Nekam,
and Margolis.9,16,34–45 A denominater in common is that
the findings shown do not enable a diagnosis to be
reached with surety. [Figures from such articles are
reproduced in the original version of this article in the
journal Dermatopathology: Practical & Conceptual at
derm101.com.1] Some patients diagnosed as having KLC
probably had several diseases concurrently rather than a
single distinctive entity.10,36,39,46 In a number of brief
‘‘reports of a case’’, neither photographs of lesions clinical
nor photomicrographs were shown and, moreover, the
findings described by the authors do not permit a
diagnosis specific to be established.18,48
Category 2
Several patients diagnosed as having KLC show
variations on the theme of lichen simplex chronicus,
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Keratosis Lichenoides Chronica
FIGURE 9. A and B, Features clinical and findings histopathologic shown in an article by Petrozzi in 1976. Although the
photograph is wanting in quality, papules are seen to have
assumed linear shape. Histopathologically, a lichenoid infiltrate is situated beneath an atrophic epidermis covered by
hyperkeratosis, foci of parakeratosis being present. Reproduced with permission.11
FIGURE 8. A–C, The findings shown here come from a biopsy
specimen taken from a patient of Aloi and Tomasini reported
on in 1997. Individual papules have become confluent to form
outlines linear and arcuate. Findings histopathologic are those
of a patchy lichenoid dermatitis beneath an atrophic epidermis covered by hyperkeratosis, foci of which are parakeratotic.33
including those known as severe ‘‘atopic dermatitis’’ and
prurigo nodularis.3,9,20,49 Although many authors claimed
to have observed ‘‘typical features’’ histopathologic of
KLC, they usually do not picture them,50 the lesions
clinical actually pictured being those, for example, of
‘‘atopic dermatitis’’. In many patients, ‘‘lichenoid dermatitides’’ of different types established were not excluded
from consideration definitively.17,51 In other articles,
lichen planus16,23,46,52–56,66 or lupus erythematosus41,42,44
could be considered on the basis of findings histopathologic. Two patients had a blistering disease, not KLC; the
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2006 Lippincott Williams & Wilkins
lesions resolved with formation of milia.6,57 Figures of
articles in which authors made a diagnosis of KLC, but
the lesions clinical and the photomicrographs indicate
that the diagnosis is something other than KLC (eg,
lichen simplex, prurigo nodularis, hypertrophic lichen
planus, lupus erythrematosus, and dystrophic epidermolysis bullosa) are reproduced in the original version of this
article in the journal Dermatopathology: Practical &
Conceptual at derm101.com.1
Category 3
Only a few patients reported on presented themselves with lesions very similar to one another clinically—
namely, an eruption that involved the face in a manner
reminiscent of seborrheic dermatitis and with tiny papules
on the trunk and extremities, which assumed linear
and reticulate shapes by way of confluence of
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FIGURE 10. A and B, In this patient of
Kossard et al, tiny papules have become
confluent in loci, the result being a
pattern reticular. Kossard and coworkers
described an interface dermatitis and
took note of prominent parakeratosis in
acrosyringia of eccrine units, a finding
interpreted by them to be cornoid
lamellation. The changes pictured in
the photomicrograph, however, are
scant. Reproduced with permission.62
lesions.19,27,29–31,58 In several reports, the lesions pictured
seem to be ones of the very same condition.3,5,12,21,22,28,59–65 Unfortunately, some of those
reports were devoid of even a single photomicrograph,18,22,29,59–61,64 excluding thereby the possibility of coming to a diagnosis for sure. Pictures of lesions
clinical and photomicrographs are reproduced in
Figures 4 to 15, illustrations of lesions in one patient
of my colleagues and me being included. The findings
in all of those patients seem to be ones of a condition
unique and dissimilar from lichen planus, lupus erythematosus, pityriasis lichenoides, pityriasis rubra pilaris,
psoriasis, porokeratosis, and mycosis fungoides, includ-
ing parakeratosis variegata and ‘‘folliculocentric’’ mycosis fungoides. The patient observed by myself and
my colleagues presented himself with features very
similar to what was set forth by authors of those latter
23 articles.
To forge criteria that are repeatable for diagnosis of
KLC, it seems essential to focus on those 24 patients who,
based on the attributes pictured in articles, presented
themselves with lesions very similar to each other.
Features clinical and findings histopathologic in those
patients are summarized in Table 3. Diseases to be
considered in the differential diagnosis of KLC are listed
in Table 4.
FIGURE 11. A and B, Miller, in 1944,
illustrated his article with these photographs. Unfortunately he did not include
photomicrographs of a section from a
biopsy specimen. Reproduced with permission.29
270
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Keratosis Lichenoides Chronica
FIGURE 12. No photomicrograph was included in the article
by Ryatt in 1982. Reproduced with permission.22
CONCLUSION
Patients diagnosed as having KLC represent a
potpourri of different diseases, the most common of them
being lichen simplex chronicus, lichen planus, and lupus
erythematosus. Some of the patients diagnosed with
KLC, however, presented themselves in a manner very
similar to one another and very different from the diseases
just mentioned. The disease in those patients was widespread, lesions being situated on face, extremities,
especially the acral parts of them, the trunk less
FIGURE 13. Lesions clinical in a patient reported on by Degos
et al in 1974. Papules are tiny and could be infundibulocentric
because some of them seem to be equidistant from one
another. Some have become confluent to form an array linear.
Reproduced with permission.47
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FIGURE 14. These lesions from a patient recorded by
MacDonald and Williams in 1974 are very similar to those
pictured by Duperrat et al, in 1977. Reproduced with
permission.59
commonly, and, occasionally, mucous membranes. Individual papules were infundibulocentric and acrosyringocentric. Sometimes, papules had an atrophic center
accompanied by tiny erosions or crusts. Also encountered
often were comedo-like lesions. Keratotic papules and
plaques could be discrete or, by way of confluence of
them, form linear or netlike shapes. Findings histopathologic were those of a lichenoid dermatitis affiliated with
numerous necrotic keratocytes and covered by parakeratosis in staggered fashion. The patients do not have
lichen planus, lupus erythematosus, pityriasis lichenoides,
pityriasis rubra pilaris, psoriasis, mycosis fungoides, or
lichen simplex chronicus, but an authentic and distinctive
condition that seems to be exceedingly rare. Cause and
pathogenesis of it are not known, the course of the disease
is protracted, and no treatment has been established for
it. Retinoids and/or PUVA may improve the condition in
some patients.
In sum, the diagnosis KLC should be made only for
patients who present themselves with features clinical and
findings histopathologic that resemble closely those
shown in category 3, to which the criteria for diagnosis
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Volume 28, Number 3, June 2006
FIGURE 15. (A–D) These photographs come from a patient studied by me and my colleagues. Individual papules were
infundibulocentric, some of them having a comedo-like horny plug in the center. Individual papules also were situated on palms
and soles, and there seemed to be centered around acrosyringia. Lesions tended to become confluent to form a line or create a
pattern reticular, especially on the acra of extremities. At sites exposed to trauma mechanical, papules became confluent to result
in plaques covered by scales and crusts. (E–L) Findings histopathologic in the patient observed by my colleagues and me were
those of a lichenoid dermatitis, especially around infundibula, but also around acrosyringia. The bandlike infiltrate consisted of
lymphocytes mostly. There were clusters of necrotic keratocytes and atrophy was present in loci, whereas uneven acanthosis was
evident in other loci. Hyperkeratosis was constituted of zones of parakeratosis in fashion staggered, sometimes housing debris of
nuclei of neutrophils. Plasma cells and histiocytes were present in the vicinity of an erosion or a rupture of infundibular epidermis.
TABLE 3. Features in Common of Patients in Category 3
Age and sex
Distribution of lesions
Arrangement of lesions
Configuration of lesions
Individual lesions
Symptoms
Course
Findings histopathologic
272
Men and women are affected equally all, patients being adults.
Face, extremities, especially acral parts of them, the trunk less commonly. Mucous membranes said to be affected in
one fourth of the patients
Widespread
Discrete as well as linear, arcuate, netlike as a consequence of confluence of papules. Lesions may assume shapes that
look artificial by virtue of a Koebner phenomenon
Papules infundibulocentric and papules around acrosyringia, comedo-like lesions, atrophic papules with tiny
erosions or crusts in the center of them, keratotic papules and plaques
Pruritus is a finding inconsistent, being present in less than 20% of the patients
The course is protracted. Resolution complete has never been reported. Patients tell of having had lesions for years
before the diagnosis was made correctly.
Patchy lichenoid infiltrate beneath adjacent surface epidermis
Lichenoid infiltrate often centered around infundibula and acrosyringia
Lymphocytes predominate
Vaculoar alteration along the dermoepidermal junction
Numerous necrotic keratocytes, sometimes in clusters, in surface epidermis and infundibular epidermis, especially in
the lower part of it
Atrophy and sometimes erosion of epithelium in foci where there are many necrotic keratocytes
Uneven acanthosis in foci
Wedge-shaped hypergranulosis sometimes in zones of acanthosis
Keratotic plugging of infundibula and, at times, of acrosyringia
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TABLE 3. (continued )
Correlation
clinicopathologic
Laboratory confirmation
Cause and mechanism
Options for treatment
Parakeratosis in staggered fashion
Remnants of neutrophils in zones of parakeratosis
Hypogranulosis beneath zones of parakeratosis
Plasma cells in the infiltrate in zones adjacent to erosion
Foreign body reaction consequent to rupture of dilated infundibula and spewing of contents of them into the dermis
A discrete papule comes into being by virtue of the lichenoid infiltrate centered around an infundibulum or an
acrosyringium. An erosion is consequent to atrophy of the epidermis. A comedo-like lesion consists of a plug of
ortho- and parakeratosis in an infundibulum. Keratotic papules and plaques are a result of uneven acanthosis
covered by parakeratosis that houses remnants of neutrophils staggered between zones of orthokeratosis
None exists
Not known
The condition is not usually responsive to treatment of any kind. Some improvement has been observed with
retinoids and with PUVA therapy, but never do lesions resolve completely
TABLE 4. Differential Diagnosis of Keratosis Lichenoides Chronica Clinically and Histopathologically
Differential Diagnosis Clinically
Lichen planopilaris
Pityriasis rubra pilaris
Lupus erythematosus
Mycosis fungoides, especially
manifestations infundibulocentric
and parakeratosis variegata
Differential Diagnosis Histopathologically
Lichen planus, including lichen
planopilaris and atrophic lichen
planus
Discoid lupus erythematosus
Lichenoid drug eruption
Mycosis fungoides
In both KLC and lichen planopilaris, lesions are infundibulocentric papules, some of which may be
typified by a keratotic plug in the center of them, but patients with lichen planopilaris often present
themselves with scarring alopecia, a feature never encountered in KLC.
Pityriasis rubra pilaris as well as KLC are typified by infundibulocentric papules, but in pityriasis rubra
pilaris those are joined by interinfundibular papules and, moreover, lesions often become confluent,
even to the extent of an erythroderma within which there are ‘‘islands of sparing’’ of normal skin, a
finding not seen in KLC.
Lupus erythematosus and KLC may present themselves as red macules and patches, papules, and
keratotic plaques on trunk, extremities, and face, but in lupus erythematosus lesions develop especially
on sites exposed to rays of the sun. Lupus erythematosus, but not KLC, may present itself at times with
vesicles and bullae. Scarring alopecia may be encountered in lupus erythematosus, but not in KLC.
Mycosis fungoides as well as KLC may present itself with patches, papules, and plaques, but in mycosis
fungoides those lesions may be joined by nodules and tumors, and lesions are situated especially on
thighs and buttocks. A netlike pattern also may be encountered in both diseases (ie, mycosis fungoides
and KLC), but in mycosis fungoides it is pigmentation that is netlike (parakeratosis variegata),
whereas in KLC papules assume shapes reticular by way of confluence of them. Lesions of mycosis
fungoides sometimes have a predilection for the face, neck, and scalp, where they also present
themselves with infundibulocentric papules (alopecia mucinosa/mycosis fungoides with epithelial
mucinosis), but those papules usually are arranged on a patch; infundibulocentric papules in KLC are
discrete.
Lichen planus may be infundibulocentric and associated with plugs of corneocytes housed in
infundibula, but hyperkeratosis usually is devoid of parakeratosis and remnants of neutrophils are not
encountered in the cornified layer, whereas those findings are expected in KLC. Necrotic keratocytes in
the basal layer may be encountered in both conditions (ie, lichen planus and KLC), but they are more
numerous in KLC than in lichen planus.
Although parakeratosis, at times, may be present in lesions of lupus erythematosus, never is it in the form
of mounds in staggered fashion and usually it does not house remnants of neutrophils. Necrotic
keratocytes in the basal layer may be encountered in both lupus erythematosus and KLC, but they are
more numerous in KLC than in lupus erythematosus. Infiltrates of lupus erythematosus are
accompanied by deposits of mucin in the dermis, a change not encountered in KLC.
Lichenoid drug eruption often is typified by numerous necrotic keratocytes, sometimes clusters of them,
and parakeratosis also is met with commonly. Usually, however, lesions are neither infundibulocentric
nor acrosyringocentric. Atrophy of the epidermis is uncommon, but is present often in KLC. The
infiltrate in a lichenoid drug eruption often contains eosinophils, sometimes many of them, a finding
not typical of KLC.
Infiltrates of mycosis fungoides may be infundibulocentric in lichenoid fashion, but clusters of necrotic
keratocytes and parakeratosis that house neutrophils are not findings of mycosis fungoides.
KLC, keratosis lichenoides chronica.
forged in this article, both clinical and histopathologic,
are applicable. It would be worthwhile for patients who
conform to those criteria to be collected in an effort joint
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of colleagues to hone criteria for diagnosis and to
determine, over time, the cause and pathogenesis of this
distinctive condition.
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