Keratosis lichenoides chronica 
Case report 
KERATOSIS LICHENOIDES CHRONICA 
A. Smrkolj*, R. Popovič and T. Lunder 
ABSTRACT 
Keratosis lichenoides chronica is a chronic dermatosis, generally of benign nature, yet refractory to 
treatment. Our 54-year-old man patient, who is the 40th case reported in the literature, presented with a 15-
year history of skin lesions, which were clinically, histopathologically, immunohistologically and electron 
microscopically compatible with keratosis lichenoides chronica. As a child he was taking antimalarial drugs and 
later on he was treated with antituberculous drugs. For the past 18 years he has presented with signs of 
periodic allergic rhinitis. The Re-PUVA therapy produced some improvement in some areas, keratotic papules 
with keratotic plugs had disappeared and the papules flattened. The results were superior to those obtained 
by local selective phototherapy or by systemic treatment with etretinate (Tigason). The patient was 
therapeutically unresponsive to topical application of steroids, 0,5% tretinoin and tar preparations. Systematically 
administered therapy had only a temporary effect on the dermal lymphocytic and histiocytic infiltrate. 
Considering the pathogenesis of keratosis lichenoides chronica and the composition of the dermal infiltrate, 
we think it reasonable to try cyclosporin A but our patient refused it. 
KEY WORDS 
keratosis lichenoides chronica, histopathology, ultrastmcture, treatment 
INTRODUCTION 
Since 1886, when Kaposi first described keratosis 
lichenoides chronica (KLC) until 1991, 38 cases of 
KLC had been described (1). In 1992, a new case 
of KLC was reported (2). As it is still unclear 
whether the dermatosis is an independent disease 
entity, or solely a variant of lichen ruber planus, it 
has been described under a wide variety of names, 
such as lichen ruber moniliformis (Kaposi 1886), 
lichen ruber acuminatus verrucosus et reticularis 
(Kaposi 1895), porokeratosis striata (Nekam 1938), 
dermatose papulohyperkeratosique en stries (Bureau, 
Barriere 1970), keratose lichenoide striee (Degas 
1974), lichenoid trikeratosis (Pinol-Augade 1974), and 
keratosis lichenoides chronica (Margolis 1972) (1). 
* Anica Smrkolj MD, PhD has passed away unexpectedly. A short obituary is included in this issue. 
acta dennatovenerologica A.P.A. Vol 4, 95, No 2 67 
Keratosis lichenoides chronica 
The disease runs a chronic course without 
spontaneous remissions. It is most refractory to 
treatment and affects patients of both sexes and all 
age groups. The disease is characterized by keratotic 
papules and plaques, commonly involving the limbs, 
and by erythematosquamous foci, noticed generally 
on the face. Nail lesions, keratosis of the palmar 
and plantar skin, erosions and cellular infiltrations 
involving the mucous membrane of mouth, genitals, 
eyes, upper respiratory tract and oesophagus may 
be also present. There is no systemic involvement 
and the lesions leave no scarring. Histologically, 
KLC frequently resembles lichen ruber planus. In 
the differential diagnosis Reiter's disease, Kyrle's 
disease, psoriasis vulgaris, keratosis follicularis, lichen 
ruber verrucosus, pityriasis rubra pilaris and lupus 
erythematosus have to be ruled out (1-4). 
CASE REPORT 
A 58-year-old patient, bom in Macedonia, presented 
with a 15-year history of non-itchy skin lesions 
involving tbe upper and lower extremities and tbe 
gluteal area. Tbe lesions were slowly progredient, 
unresponsive to local treatment and of seasonal 
cbaracter. Tbe patient used to squeeze wbitisb 
comedo-like material from keratotic papules, wbicb 
tended to suppurate in tbe summer. Tbe plaquelike 
lesions were scaly and severely inflamed. His nails 
bad reportedly become tbicker and brittle during 
tbe past year. 
He gave a bistory of wbooping cougb in cbildbood. 
As adolescent be bad endemic malaria, wbicb was 
treated by antimalarial agents. At the age of 23 be 
developed pulmonary tuberculosis and was placed 
on a 9-montb course of antituberculous drugs. His 
bistory also revealed uretbritis of unexplained etiology, 
wbicb had never recurred. Since the age of 30 be 
bas been taking antibistamines to treat allergic rbinitis 
wbicb tended to occur in tbe spring due to exposure 
to pollens, as confirmed by tbe prick tests. Five 
years before present admission be was taking antacids 
for erosive gastritis. He also gave a bistory of pain 
in tbe rigbt elbow and sboulder for 2 years. Tbe 
patient is by profession a cellist. He denied drinking 
alcohol and smoking. 
In 1993 be was admitted to tbe hospital twice. 
Since 1985 be bas been treated at tbe Department 
of Dermatology on an outpatient basis, for atopic 
dermatitis. 
On admission, the patient bad erythematosquamous, 
moderately infiltrated plaque-like lesions, some of 
68 
tbem reacbing tbe size of a palm. Tbey consisted of 
keratotic papules witb borny plugs involving extensor, 
side of tbe arms, cubital fossae, tbigbs, popliteal 
fossae and knees (Figs. 1,2). Tbere were individual 
keratotic foci over the dorsal aspect of tbe , bands. 
The distal parts of tbe nails were tbickened and 
split. Tbe scalp sbowed diffuse scaling. Tiny pink, 
scaly papules were noted on the laterni borders of 
the eyebrows. All visible mucous membranes were 
normal. 
Erytbrocyte sedimentation rate, blood screen, serum 
electrolytes, bilirubin, transaminases, alkaline pho-
sphatase, serum amylase, lipid count, urea and 
creatinine levels were witbin normal limits. Faecal 
smears for parasites and stool cultures for Crypto-
sporidium, fungi, Mycobacterium tuberculosis were 
negative. No tubercle bacilli were isolated from the 
urine and sputum. From tbe tbroat smear normal 
flora was recovered. Tbe serologic test for sypbilis 
was negative, wbile the tests for rbeumatoid artbritis 
were positive (AST 1:240 IU/ml, Latex 240 IU/ml, 
Waaler-Rose test 1:512 IU/ml; otber tests were 
negative). The serologic test for toxoplasmosis was 
negative. Tota! serum IgE levels were elevated to 
210 IU/ml. Specific IgE to Phleum pratense and 
Dermatophagoides pteronyssinus were found positive. 
Cbest X-rays and ultrasound of tbe abdomen were 
normal. Roentgenologic examination of tbe right 
elbow and rigbt sboulder sbowed degenerative lesions 
of tbe joints. 
Histopatbology of a plaque on tbe rigbt upper 
arm revealed a predominantly atrophic epidermis 
with intermittent areas of normal tbickness. An area 
of acantbosis and a large invagination of tbe epidermis, 
filled out with abundant byperkeratotic masses, were 
noticed (Fig. 3). A narrow streak of parakeratosis 
was seen at tbe upper border. Tbere were areas of 
liquefaction degeneration of tbe basal cells. In tbe 
upper dermis, a dense licbenoid infiltrate was noticed, 
composed of lympbocytes, bistiocytes and a few 
eosinopbils (Fig. 4). The Kongo red stain failed to 
reveal amyloid. 
Electron microscopic examination showed cytolytic 
cbanges of keratinocytes of tbe basal layer and 
lower spinous layer (Figs. 5,6). Tbere was an 
intercellular oedema. Numerous lympbocytes, mono-
cytes, macrophages and some basopbilic, eosinopbilic 
and neutrophilic granulocytes, otherwise forming dense 
infiltrates in tbe dermal papillary layer, were found 
in tbose areas (Fig. 5). Tbere was a focal thickening 
of tbe granular layer of tbe epidermis witb kera-
tinocytes containing thin tonofilament bundles and 
acta dennatovenerologica A.P A. Vol 4, 95, No 2 
Keratosis lichenoides chronica 
Fig. l. Distribution of skin lesions. 
sparse, rounded keratohyalin granules. The 
thickened horny layer showed no abnormalities, 
apart from focal parakeratosis. Other epidermal 
Fig. 2. Keratotic papules and plaque-like lesions 
on the left upper extremity. 
acta dermatovenerologica A .P A. Vol 4, 95, No 2 
Fig. 3. Massive hyperkeratosis with a narrow streak of 
parakeratosis. Areas of thinned epidermis. Under the epidermis, 
dense band-like dermal cellular infiltrate. HE, 25x 
and dermal elements had normal ultrastructure. 
Direct immunofluorescence studies demonstrated band-
like deposits of fibrin, IgG and C3 at the dermo-epidermal 
junction and globular IgM deposits in the upper dermis. 
Prior to admission to this Department, the patient had 
been treated by PUV A on severa! occasions, yet he 
noticed no improvement. 
Fig. 4. Higher magnification of features in Fig. 3 showing the 
dense lichenoid infiltrate, consisting of lymphocytes and 
histiocytes. Signs of initial liquefaction degeneration in the 
acanthotic epidermis. HE, 63x 
Treatment with nonspecific topical ointments, local steroids
0 
and orally administered antihistamines from October 19.85 
to November 1992 produced no clinical improvement 
either. Also, the patient was therapeutically unresponsive 
Keratosis lichenoides chronica 
Fig. 5. Cytolytic changes in a keratinocyte of the lower 
spinous layer. Electron micrograph, 13000x 
to local selective phototherapy (long-wave UV-B 
and UV-A) received in 1985. Another course of 30 
irradiations given in 1992 caused temporary dete-
rioration of the dermatosis. 
After the histopathologic diagnosis of KLC was 
established, the patient was placed on a 2-month 
course of oral etretinate 30 mg/day. The dose was 
then increased to 40 mg/day (0,5 mg/kg BW) for 2 
months and thereafter reduced to 30 mg daily for 
another 2 months. Later, the patient was placed on 
etretinate 20 mg/day and Re-PUV A therapy; two 
hours before each irradiation he received Oxsoralen 
40 mg. The patient was given 29 irradiations on the 
PUVA 4000 (Waldmann) apparatus. The total dosage 
received was 119 J/cm2• The therapy produced 
flattening of papules and plaques, and arrested 
desquamation. Some areas of papules with horny 
plugs on the upper limbs had disappeared. At the 
end of therapy the patient began to experience 
occasional itching and burning. After the disconti-
nuation of the therapy, sparse fresh papules re-
appeared. During the treatment, the test for lipids 
became slightly pathologic. Other side effects included 
loss of hair, and dryness of the mouth and buccal 
mucosa. The therapy had no effect on nail lesions. 
DISCUSSION 
The term KLC summarizes the leading clinical 
characteristics of the disease (5), but it does not 
clarify its etiology (6) nor does it imply that KLC 
is an independent disease entity (3,4,6,7,9). 
In the literature there is a disagreement concerning 
the factors causing KLC. These seem to include 
antimalarial and antituberculous agents (3,8) - taken 
70 
Fig. 6. Keratinocyte of the granular layer with thin 
tonofilament bundles and sparse pounded grains of 
keratohyalin. Electron micrograph, 13000x 
also by our patient - tetanus antiserum (9) , skin 
damage and coverage with skin grafts (6). KLC may 
be associated with other diseases, such as atopic 
dermatitis (5,10), allergic rhinitis (3), pulmonary 
tuberculosis (5) , hepatitis A (3), chronic lymphatic 
leukemia (2), neurologic disorders (11), clinically 
manifest toxoplasmosis, or positive serologic tests 
for toxoplasmosis (12-14). Chronic asymptomatic course 
of KLC without spontaneous remissions, with the 
onset in adult age was characteristic of our case. 
The disease only rarely occurs in children (10,15,16). 
It usually lasts over 10 years. A 15-year history of 
KLC, as seen in our patient, has been reported by 
some other authors (17,18). As documented in the 
literature, KLC is only rarely associated with skin 
itching (9,19). Our patient experienced it after topical 
application of 0,1 % tretinoin and on completion of 
Re-PUVA therapy. 
Arthralgia was partly due to degenerative changes. 
He also had positive serologic tests for rheumatoid 
arthritis, which constitutes a symptom only rarely 
described in connection with KLC (5,18). 
The course of the illness, the distribution and 
quality of skin lesions are similar to the description 
by Margolis et al. (17) and by other authors (2-8, 
10, 12, 1 7-25). 
Histopathological findings, including acanthosis, 
parakeratosis and subepidermal lichenoid lymphocytic 
and histiocytic infiltrate agree with most observations 
found in the literature (5,6,8-10,16,20,24). Another 
feature reported by some investigator~ was a 
perivascular infiltrate in the upper dermis (3,13,17). 
Infiltrate composed of eosinophils bas also been 
found in some previous studies (3,20). Liquefaction 
acta dermatovenerologica A.P A. Vol 4, 95, No 2 
Keratosis lichenoides chronica 
degeneration of the basal layer has been widely 
reported in the literature (5-7,10,14,17,18,22), and 
so have been hyperkeratotic plugs in the invaginations 
of the epidermis (5,7-9, 11,21,24). Electron microscopic 
results resemble those in Iichen ruber planus (26,27). 
Like Dupperat et al. (23), we found no viral particles. 
As previously observed by some other investigators, 
the results ot direct immunofluorescence microscopy 
of skin lesions partially resembled the findings 
characteristic of lichen ruber planus (1,6,7,9,16,24). 
The treatment modalities described so far do not 
afford complete cure of the disease. Topical application 
of steroids produced no evidence of benefit, regardless 
of the concentration of the glucocorticoid used; 
other authors reported a poor or no response to 
treatment with local mercury preparations, iodine 
compounds, salicylic acid preparations and numerous 
systemic agents , including vitamins A and B, 
chloroquine sulfate, antibiotics, gold salts, griseofulvin, 
methotrexate, zync sulphate, glucocorticoids, sulpho-
namydes , dapsone and levamisole (3,14,17,18,24,25). 
In the literature there are no reports on successful 
treatment with selective phototherapy, while some 
authors described fair improvement after PUV A 
therapy (10,25) ; in our patient selective phototherapy 
and PUV A failed to produce clinical improvement. 
On the other hand, systemically administered etretinate 
afforded the expected improvement (14,21,22,25), 
yet it was only partial, temporary and dependent on 
the dose given. Fairly good response to Re-PUV A 
therapy was reported by Duchet et al. (12). In our 
case, we observed better results of this treatment 
modality than those yielded by administration of 
etretinate alone. Some keratotic plugs disappeared 
and the flattening of skin plaques was noticed; 
however, sparse fresh papules reappeared one month 
after the completion of the therapy. 
In view of the established pathogenesis and the 
predominance of T4 lymphocytes in the cellular 
infiltrations of KLC (12,21 ), we think it reasonable 
to introduce cyclosporin A in the future treatment 
of KLC. It helps regulate the T4 (helper) / T8 
(suppressor) ratio, and depresses the function of T4 
lymphocytes (28,29). Cyclosporin A is also 
recommended in the management of other chronic 
infiltrative dermatoses (30,31,32). Our patient refused 
it because of the possible renal side effects. 
Acknowledgment: 
We wish to thank Dr. Marija Berčič for her valuable 
suggestions and help with histopathological findings . 
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AUTHORS' ADDRESSES 
Anica Smrkolj MD, PhD, dermatologist, Department of Dermatology, Medica! Center, Zaloška 2, 61000 
Ljubljana, Slovenija 
72 
Ranka Popovič MD, MSc, dermatologist, same address 
Tomaž Lunder MD, MSc, dermatologist, same address 
acta dennatovenerologica A.P A. Vol 4, 95, No 2