Keratodermia palmoplantaris papulosa 
Case repo,t 
KERATODERMIA PALMOPLANTARIS PAPULOSA 
(BUSCHKE-FISCHER-BRAUER). 
CASE REPORT. 
M. Košiček, T. Perkovič, T. Lunder, M. Penko and A. Kansky 
ABSTRACT 
A case of hereditary palmoplantar keratoderma (HPPK) papulosa in a 68-year-old man is presented. The 
large number of isolated hyperkeratoses symmetrically distributed on the palms and soles appeared at the age 
of 30 years. The similar changes on the palms and soles had also the patient's father and grandfather as well 
as has his son. 
Histologically the lesions consisted of a thickened horny layer with keratotic plugs. There were hypergranulosis 
and acanthosis. Dermoepidermal junction was normal. 
Compared to other types of HPPK, the disease is rare. Until now, sixteen cases have been detected in 
Slovenia. 
KEY WORDS 
hyperkeratosis, hereditary, palmoplantar, papular, micromorphology 
INTRODUCTION 
The term hereditary palmoplantar keratodermas 
(HPPK) includes a number of various clinical types, 
characterized by the main symptom: hyperkeratoses 
of the palms and soles (1). The most frequent type 
seems to be HPPK Unna-Thost, which can not be 
clinically differentiated from HPPK Vorner type. 
Certain authors even believe that the majority of 
Unna-Thost type cases are rather HPPK Vorner 
type. However, this problem is stili not clarified (2, 
3). In the group of HPPK other types (nosologic 
24 
entities) may be distinguished, e. g. keratosis palmo-
plantaris transgrediens (Mljet type ), keratosis extre-
mitatum hereditaria progrediens, HPPK Papillon-
Lefevre type ( 4), HPPK Jadassohn-Lewandowsky 
(pachyonychia congenita) (5), as well as others (1, 
6). 
HPPK papulosa was first described in 1910 by 
Buschke and Fischer and later in 1913 by Brauer 
(7, 8). It is a relatively rare disease. Different 
synonyms are used: keratosis palmoplantaris papulosa 
Buschke-Fischer-Brauer, keratoma hereditarium dissi-
patum palmare et plantare, keratodermia symmetrica 
acta dermatovenerologica A.P A. Vol 7, 98, No 1 
Keratodermia palmoplantaris papulosa 
Fig. l. Com-like hyperkeratoses on the palms of the 
patient with heredita,y palmoplantar papular keratoderma. 
Fig. 3. Histopathology: unusually thickened and compact 
stratum comeum, hypergranulosis, and mild acanthosis. 
250x 
acta dennatovenerologica A.P.A. Vol 7, 98, No 1 
Fig. 2. Com-
like hyper-
keratoses on 
the soles of 
the patient 
with heredita,y 
palmoplantar 
papular kerato-
derma. 
Fig. 4. Histopathology: hyperkeratosis, the granular layer is 
large, comprising up to 1 O layers of cel/s, in the upper layers 
there are numerous large keratohyalin granules. 400 x 
25 
Keratodermia palmoplantaris papulosa 
II. 
IV. 
v. 
Fig. 5. The familly tree of the patient T M with 
heredita,y palmoplantar papular keratoderma 
synonyms are used: keratosis palmoplantaris papulosa 
Buschke-Fischer-Brauer, keratoma hereditarium dissi-
patum palmare et plantare, keratodermia symmetrica 
maculosa disseminata palmaris et plantaris, disse-
minated clavus of the hands and feet (1). The 
disorder is transmitted in a regular autosomal-dominant 
mode (1). Sporadic cases have been also reported 
(9). This condition must be differentiated from the 
acquired forms. In both instances (hereditary and 
acquired forms) it may be linked to interna] 
malignancies (10,11). Compared to other HPPKs, 
the disease appears later in life, usually at the age 
15 - 30 years (1,9). 
The disease is clinically manifested by a large 
number of isolated, corn-like, cone-shaped hyper-
keratoses, symmetrically distributed on the palms 
and soles. Hyperkeratoses may be removed without 
Figure 6. Electron microscopy: keratinocytes of the 
granular layer display thick tonofilament bundles. 
26 
bleeding, leaving basin-shaped indentations (1). Asso-
ciated abnormalities are uncommon (12,13). 
A few cases of HPPK papulosa with concomitant 
disorders have been reported: cases of HPPK papulosa 
and superficial dystrophy of comea, HPPK papulosa and 
lipomas (13). 
Patients usually complain of painful sensations 
when walking, standing or doing manual work. There 
is no spontaneous remission (1). 
CASE REPORT 
A 68-year-old man, T. M. bom in 1930, was 
examined by a dermatologist because of severa! 
hyperkeratoses of palms and soles. The lesions started 
at the age of 30 years. 
Family history: similar changes on the palms and 
soles had also patient's father and grandfather; his 
son has similar symptoms. Two patient's sisters, one 
brother, a 25-year-old grandson, as well as one 
nephew are symptom-free (Figure 5). 
Personal history: the patient was treated for pulmonary 
tuberculosis in 1958, a duodenal ulcer was detected 
in 1975 and three cysts of the left kidney in 1990. 
He survived an acute myocardial infarction in 1996; 
two times he suffered from pulmonary edema. In 
1996, he was treated with radioactive iodine because 
of the hyperthyreosis. 
Social and occupational history: the patient is 
married and has one son. He completed four years 
of primary school and worked as a coal-miner. Due 
Figure 7. Electron microscopy: large keratohyalin 
granules in the granular celi layer. 
acta dermatovenerologica A.P.A. Vol 7, 98, No 1 
Keratodermia palmoplantaris papulosa 
to inconvenience caused by the hyperkeratosis, he 
managed to obtain the qualification as a radio-
mechanic. He is now retired. 
A derrnatological inspection revealed a large number 
of isolated hyperkeratoses on the palms and soles. 
Corn-like changes were symmetrically distributed on 
the palms and soles as well as on flexural aspects of 
fingers and toes (Figures 1 and 2). A mild 
hyperkeratosis was expressed on the both elbows; 
the knees were free of symptoms. 
Histopathology: The horny layer was unusually 
thickened and compact, the number of sweat glands' 
ducts was increased. The granular layer was large 
comprising up to 10 layers. There was a mild 
acanthosis of the Malpighian layer, dermoepidermal 
junction was normal. In the papillary layer there was 
an increased number of slightly dilated vessels, some 
of them filled up with erythrocytes (Figure 3). With 
higher power numerous large, irregularly shaped 
keratohyaline granules were seen in the upper layers 
of stratum granulosum (Figure 4). 
Electron microscopy demonstrated hyperkeratosis, 
hypergranulosis, and acanthosis of the epidermis. 
Focally, the keratinocytes in the spinous and 
granular layers contained thick bundles of tono-
filaments. Very large keratohyaline granules were 
found in the granular layer. The ultrastructure of 
the dermis and of the dermoepidermal junction 
was normal (Figures 6 and 7). 
Therapy: Keratolytic ointments were applied topically. 
Systemic retinoids were not recommended, due to 
the presence of mentioned interna! symptoms. 
DISCUSSION 
The therapy of HPPK papulosa is symptomatic; 
keratolytic ointments are mostly used. The effect of 
systemic retinoids may differ according to Christiansen: 
out of nine patients treated with etretinate 0,5 mg/ 
kg/day, in three the results were good, in four moderate, 
while in two no effect was observed (14). 
Epidemiological data on HPPK papulosa are scarce 
and inconsistent (12). In Slovenia, twelve cases in 
four families were detected until now, and five of 
them were examined (15). In an extensive population 
study in Croatia a prevalence of HPPK papulosa of 
1.17 per 100.000 inhabitants was reported (12). 
CONCLUSION 
A patient with keratosis palmoplantaris papulosa, 
which is rather rare form of HPPK, is presented. 
Including the presently reported four cases, altogether 
16 cases of this disorder have been detected in 
Slovenia. No relationship between previous cases and 
presented family could be established up to now. 
ACKNOWLEDGEMENT 
The preparation of the manuscript was supported by 
the Slovenian Ministry of Science and Technology; 
Grant No 13-9105. 
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AUTHORS' ADDRESSES 
28 
Metoda Košiček MD, Department Dermatology, Clinical Center, Zaloška 2, 1525 Ljubljana, Slovenia 
Tatjana Perkovič MD, pathologist, Department of Pathology, Medical Faculty, 
Korytkova 2, 1525 Ljubljana, Slovenia 
Tomaž Lunder MD, MSc, dermatologist Department Dermatology, Clinical Center, 
Zaloška 2, 1525 Ljubljana, Slovenia 
Marta Penko MD, dermatologist, Chairman of Department Dermatology, same address 
Aleksej Kansky MD, PhD, professor of dermatology, same address, corresponding author 
acta dermatovenerologica A.P.A. Vol 7, 98, No 1