Eosiniphilic pustular f olliculitis 
Case report 
EOSINOPHILIC PUSTULAR FOLLICULITIS 
A CASE REPORT 
J. Peč, Z. Frličkova, Ž. Fetisovova, L. Plank, K. Adamicova and M. Peč 
SUMMARY 
A 49-year old man with eosinophilic pustular folliculitis with multiple pruntlc papulopustular, more 
follicular lesions on erythematous base, mainly located in seborrheic areas, less on the shoulders, upper limbs 
and laterni parts of the thorax is presented. Routine laboratory investigations revealed eosinophilia, IgE 
hypergammaglobulinemia, and elevated serum levels of beta-2-micrglobulin. Histopathology from a skin lesion 
(biopsy) revealed enlarged hair follicules, filled with eosinophiles and with eosinophilic infiltrates penetrating 
the sebaceous structures. 
Histopathology from a right axillar lymph node showed histological changes corresponding to the diagnosis 
of dermatopathic lymphadenitis. The presence of plasmocytoid monocytes could possibly indicate a preleukemic 
variant of the disease. 
KEY WORDS 
eosinophilic folliculitis, pustu/ar, skin, hair follicles, lymph nodes, 
INTRODUCTION 
Eosinophilic pustular folliculitis (EPF) was first 
described by Ofuji et al (1) in 1970. EPF - a rare 
chronic disease of unknown etiology, sometimes 
associated with immunodeficiency (HIV), lymphoma, 
leukemia, bone marrow transplantation, haematologic 
diseases, and atopy. But it may also occur in healthy 
persons (2-4). 
We described an extremely rare case of EPF 
(Ofuji disease) in our region. 
acta dennatovenerologica A.P.A. Vol 7, 98, No I 
CASE REPORT 
A 49-year-old man, non-smoker (a motor car 
mechanic), presented with EPF of six years duration. 
The patient had pruritic papulopustular, more follicular 
located lesions about 2 mm in diameter, on an 
erythematous base, gradually confluent to form 
polycyclic plaques with tendency to peripheral 
extension and central healing. The lesions were 
mainly located in seborrheic areas (Figures 1 and 2) 
on the shoulders, upper limbs, and lateral parts of 
the thorax. On the lesions located on legs, palmar 
19 
Eosiniphilic pustu/ar f olliculitis 
Figure l. Eosinophilic pustu/ar folliculitis - polycyclic 
papulopustular, more f ollicular located lesions - f ace. 
and plantar areas no pustulation was visible; there 
were only erythematous plaques covered with small 
scales. A generalized lymphadenopathy accompanied 
the skin lesions. 
Figure 2. Eosinophilic pustu/ar f olliculitis - back. 
20 
Cultures of material obtained from pustules remai-
ned sterile. Routine laboratory tests revealed a mild 
leukocytosis (9.4 - 10.9xl09/l), eosinophilia (17-
21 % ) - absolute number l.9xl09/l (normal up to 
0.5xl09) , IgE hypergammaglobulinemia 275.2 IU/ml 
(normal up to 200 IU/ml), elevated serum levels of 
beta-2 microglobulin 4054 ng/1 (normal up to 3000 
ng/1). Other laboratory examinations e.g., renal and 
!iver function tests, serum electrolytes, glucose, protein 
contents, other immunoglobulins, complement, circu-
lating immunocomplexes, proteins of acute phase of 
inflammation, antinuclear antibodies yielded normal 
results. Erythrocyte sedimentation rate, other hema-
tologic parameters and flow cytometry investigation 
revealed no abnormalities. Urin analysis was normal. 
Antibodies against HIV-1, HIV-2, hepatitis virus A, 
Epstein-Barr virus, toxoplasmosis, toxocariasis, echino-
coccosis, trichinellosis, fasciolosis were negative. The 
samples of stool investigated parasitologically were 
also negative. 
HISTOPATHOLOGY 
In both skin excisions taken from the chest the 
epidermis was acanthotic with faint spongiosis and 
mild parakeratosis. In upper corium foci of infiltrates 
situated perivascularly and periadnexally were found. 
The dermoepidermal junction was involved in only 
a few places. There were two types of infiltrate: 
first one consisted of perivascular lymphoplasmocytic 
cells with few leucocytes with prevailing CD3+ T-
lymphocytes (Dakopatts, Denmark), and polyclonal 
plasmocytes. The second type consisted of primarily 
eosinophilic leukocytes in follicular and perifollicular 
distribution, in places simulating abscesses. The 
hair follicles were enlarged and filled with eosinophiles 
with consequent destruction of follicular epithelia. 
The eosinophilic infiltrates penetrated also the seba-
ceous structures (Fig.3). Direct immunofluorescence 
with anti-IgG, anti-IgA, anti-IgM, and C3, C4 was 
negative. 
In the lymph node of the right axilla reactive 
changes were observed: atrophy of lymphoid follicles 
with the so-called hyperplasia with proliferation of 
CD 68+ macrophages associated antigen, Ki-MlP 
(generous gift from the Department of Pathology, 
University of Kiel, Germany) and interdigitating 
reticular cells S-100 protein + (Dakopatts, Depmark) 
with a distinct phagocytosis of PAS+ (periodic acid-
Schiff staining) pigmented material and melanin-like 
pigment. In the vicinity of these components areas 
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Eosiniphilic pustular f olliculitis 
of the so-called plasmocytoid monocytes were found. 
In addition also formation of tiny epitheloid-cell 
granulomas were observed. In medulla a massive 
polytypic plasmocytic infiltrate (kappa +, lambda+; 
immunostaining with the primary polyclonal antibodies 
anti-kappa, anti-lambda and anti-lysozyme was 
performed using the peroxidase-antiperoxidase -P AP 
complex method) was present. Although the majority 
of the histological changes corresponded to the 
diagnosis of dermatopathic lymphadenitis, the presence 
of plasmocytoid monocytes (previously T-associated 
plasma cells) could represent also to the initial 
stages of proliferation of myelocytary line (with a 
possible transition into the acute myeloid leucaemia). 
Bone puncture showed non-specific and non-cha-
racteristic reactive changes with slightly increased 
eosinophile count, without any visible aggregates and 
Figure 3. Hair follicles were enlarged, filled with 
eosinophiles. Haematoxylin-eosin staining, magnification 
x250. 
acta dermatovenerologica A.P.A. Vol 7, 98, No 1 
without a definitive proof of a myeloproliferative 
disease. 
After prednisone treatment (1.0 mg/kg of body 
weight/day), the doses being gradually reduced to the 
total daily dose of 10.0 mg, clinical signs of the 
disease have receded. This improvement was accom-
panied with decrease of eosinophilia to 9.8%, nor-
malization of leukocytosis, of IgG values to 29.9 
IU/ml, and microglobulin to 2820 ng/1. Because of 
the persisting findings on the lymph nodes, the 
patient is kept under observation. 
DISCUSSION 
The disease is characterized by a chronic course 
(years), follicular papulopustules or pustules on 
erythematous bases in the scalp, in the facial region, 
on the back and shoulders, occurring predominantly 
in males (5,6). Palmoplantar localization of the 
lesions can also occur (7). The content of pustules 
remains usually sterile, in some cases colonies of 
fungi and bacteria, such as Pseudomonas, appear (8). 
Frequent findings are leukocytosis and eosinophilia 
(9). Histological picture reveals several interesting 
morphological changes of the cells. However, characte-
ristic for EPF are aggregates of eosinophils and 
lymphocytes around the pilosebaceous unit (4). Test 
of direct immunofluorescence are usually negative, 
though in indirect immunofluorescence high titres of 
circulating IgG and IgM compared to the cytoplasmic 
cells of epidermis were described (10). 
EPF is an inflammatory dermatosis of unknown 
etiology. However, certain unknown stimuli may 
activate follicular keratinocytes, sebaceous glands 
cells, apposition of the infiltrating T-lymphocytes 
and Langerhans cells. These produce release of 
cytokines, chemotactic factors - interleukin 4, interferon 
gamma, or cyclooxygenase-generated metabolites 
derived from arachidonic acid ( 4,11,12), which may 
be important in attracting eosinophils (5). 
The presented case of EPF is the first one described 
in our region; there is a possibility of preleucaemic 
variant of the disease. 
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Eosiniphilic pustu/ar f olliculitis 
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high circulating titres of IgG and IgM directed to 
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268-73. 
11. Teraki Y, Konohana I, Shiohara T, Nagashima 
M, Nishikawa T. Eosinophilic pustular folliculitis (Ofuji's 
disease). Immunohistochemical analysis. Arch Dermatol 
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12. Teraki Y, Imanishi K, Shiohara T. Ofuji's disease 
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8. 
AUTHORS' ADDRESSES 
22 
Juraj Peč, MD, Zuzana Frličkova, MD, Želmfra Fetisovova, MD, Department of Dermatology, 
University Hospital, Jessenius School of Medicine, Comenius University, Kollarova 2, 
036 59 Martin, Slovak Republic 
Lukaš Plank, MD, PhD, professor, Department of Pathological Anatomy, same address 
Katarfna Adamicova, MD, Department of Pathological Anatomy, same address 
Martin Peč, MD, Department of Medica! Biology, same address 
acta derrnatovenerologica A.P.A. Vol 7, 98, No 1