BJD
British Journal of Dermatology
C O N CI S E C O M M U N I C A T I ON
Facial and extrafacial eosinophilic pustular folliculitis: a clinical and histopathological comparative study W.J. Lee, K.H. Won, C.H. Won, S.E. Chang, J.H. Choi, K.C. Moon and M.W. Lee Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnapdong Songpagu, Seoul 138-736, Korea
Summary Correspondence Mi Woo Lee. E-mail: [email protected]
Accepted for publication 23 November 2013
Funding sources None.
Conflicts of interest None declared. DOI 10.1111/bjd.12755
Background Although more than 300 cases of eosinophilic pustular folliculitis (EPF) have been reported to date, differences in clinicohistopathological findings among affected sites have not yet been evaluated. Objectives To evaluate differences in the clinical and histopathological features of facial and extrafacial EPF. Methods Forty-six patients diagnosed with EPF were classified into those with facial and extrafacial disease according to the affected site. Clinical and histopathological characteristics were retrospectively compared, using all data available in the patient medical records. Results There were no significant between-group differences in subject ages at presentation, but a male predominance was observed in the extrafacial group. In addition, immunosuppression-associated type EPF was more common in the extrafacial group. Eruptions of plaques with an annular appearance were more common in the facial group. Histologically, perifollicular infiltration of eosinophils occurred more frequently in the facial group, whereas perivascular patterns occurred more frequently in the extrafacial group. Follicular mucinosis and exocytosis of inflammatory cells in the hair follicles were strongly associated with facial EPF. Conclusions The clinical and histopathological characteristics of patients with facial and extrafacial EPF differ, suggesting the involvement of different pathogenic processes in the development of EPF at different sites.
What’s already known about this topic?
•
Although the eruption of eosinophilic pustular folliculitis (EPF) primarily affects the face, rare lesions may be located at extrafacial sites.
What does this study add?
• •
The clinical features and histopathological findings of facial and extrafacial EPF are different. These differences suggest that different pathogenic mechanisms may participate in the development of EPF, depending on the affected site.
Ofuji disease, or eosinophilic pustular folliculitis (EPF), initially described in 1970,1 primarily affects the face, but lesions can also be located on the trunk and extremities, i.e. extrafacial sites. EPF is characterized by repeated pruritic follicular papules and pustules superimposed on plaques with central clearing and peripheral extension. EPF has been divided into several subtypes, including the classical, infantile and immunosuppression-associated types.2,3 These three subtypes are
considered to be within the spectrum of EPF as they present with similar clinicopathological features. However, many differences exist, and it has been proposed that these three subtypes be viewed as different disease entities.4,5 A key histopathological finding in patients with EPF is a dense inflammatory infiltrate around the pilosebaceous unit, consisting predominantly of eosinophils and mononuclear cells.6 Perivascular and dermal infiltrates of eosinophils and lymphocytes
© 2013 British Association of Dermatologists
British Journal of Dermatology (2014) 170, pp1173–1176
1173
1174 Facial and extrafacial EPF, W.J. Lee et al.
have also been observed in patients with this disease.6–8 Pustules containing eosinophils are frequently observed but are not necessarily required for the diagnosis of EPF, as some patients with EPF lack this characteristic.9,10 Although approximately 300 cases of EPF have been reported to date, differences in clinicohistopathological findings relative to affected sites (facial or extrafacial) have not been analysed. This study was therefore designed to compare the clinical and histopathological features of patients with facial and extrafacial EPF.
Materials and methods All patients diagnosed with EPF who were evaluated at Asan Medical Center, Seoul, Korea, between June 1998 and July 2013 were retrospectively assessed. Only patients in whom infectious processes had been ruled out and had tissue eosinophilia confirmed by skin biopsy were included. Patients with simultaneous involvement of facial and extrafacial areas and those without eosinophil infiltration around pilosebaceous units were excluded. Our study cohort consisted of 46 patients, 33 with facial and 13 with extrafacial EPF. Histopathological analysis Eosinophil infiltration patterns were classified as perivascular, perifollicular or interstitial. The intensity of the infiltrate was scored between 0 and 3 (0, absence of infiltrate; 1, faint infiltrate; 2, moderate infiltrate; and 3, intense infiltrate). The presence of follicular mucinosis, flame figure, exocytosis of inflammatory cells in the hair follicle, and epidermal changes such as crust, hyperkeratosis and acanthosis was evaluated. Sebaceous lysis with a cellular infiltrate and spongiosis in the sebaceous gland were also assessed, as was the presence of infiltrates of other inflammatory cells such as neutrophils. Statistical analysis All data were analysed statistically using SPSS version 18.0 (IBM, Armonk, NY, U.S.A.). P-values < 005 were considered statistically significant. The presence of certain clinical and histopathological findings were compared in the facial and extrafacial EPF groups using the v2 test or Fisher’s exact test. The intensity of eosinophil infiltrates in three patterns (perivascular, perifollicular and interstitial) was compared using a linearby-linear association test.
Results Clinical and laboratory findings The clinical features of the patients with EPF who were analysed in this study are summarized in Table 1. Age at presentation was similar in the facial and extrafacial EPF groups (P = 0924; Fig. 1a). The percentage of male patients was significantly higher in the extrafacial (11 of 13, 85%) than in the facial (11 of 33, 33%) EPF group (P = 0003; Fig. 1b). British Journal of Dermatology (2014) 170, pp1173–1176
Table 1 Clinical and histopathological characteristics of patients with facial (n = 33) and extrafacial (n = 13) eosinophilic pustular folliculitis (EPF) Facial EPF, n (%)
Extrafacial EPF, n (%)
Age, years (range) 380 (9–64) 393 Sex (male: female) 1:2 55 : 1 Clinical subtypes Classical type 31/33 (94) 8/13 IS-associated type 2/33 (6) 5/13 HIV-associated 0/33 1/13 Malignancy1/33 (3) 4/13 associated Transplantation1/33 (3) 0/13 associated Infantile type 0/33 0/13 Clinical morphology Plaque formation 15/33 (45) 3/13 Annular plaque 9/33 (27) 0/13 formation Multiple papules 17/33 (52) 10/13 Pustules 6/33 (18) 3/13 Pruritus 22/33 (67) 10/13 Hypereosinophilia 4/29 (14) 2/9 in blood Histopathological features Eosinophil infiltration (estimated scores) Perifollicular 191 153 pattern Perivascular 075 146 pattern Interstitial 103 062 pattern Follicular 16/33 (48) 2/13 mucinosis Exocytosis of 21/33 (64) 3/13 inflammatory cells Sebaceous lysis 12/33 (36) 0/13 Flame figure 3/33 (9) 1/13 Epidermal change 12/33 (36) 4/13 Neutrophil 5/33 (15) 3/13 infiltration
P-value
(12–69)
0924 0003*
(62) (38) (3) (31)
0014* 0014*
(23) (0)
0197 0035*
(77) (23) (77) (22)
0184 0698 0724 0613
0031* 0013* 0143 (15)
0049*
(23)
0021*
(8) (31) (23)
0011* 0637 1 0372
*Statistically significant. IS, immunosuppression.
Extrafacial EPF lesions were located on the legs of five patients, the arms of four, the buttocks of three, the inguinal area of two, the trunk of two, the hand of one patient and the scalp of one patient. Immunosuppression-associated EPF was significantly more common in the extrafacial than in the facial EPF group (P = 0014; Fig. 1c). Plaque formation was observed in 15 of 33 patients (45%) with facial EPF and in three of 13 (23%) with extrafacial EPF. Annular plaques were significantly more common in the facial group (P = 0035), but pustule formation was similar in the two groups (P = 0698). The frequency of blood hypereosinophilia was similar in the facial and extrafacial groups (14% vs. 22%, P = 0613). Of the 46 patients, 21 (46%) were treated with dapsone, 13 (28%) with minocycline, seven (15%) with naproxen and three (7%) with © 2013 British Association of Dermatologists
Facial and extrafacial EPF, W.J. Lee et al. 1175
(a)
group (P = 0013). However, there were no between-group differences in the frequency of interstitial patterns (P = 0143; Fig. 2c). Exocytosis of inflammatory cells in the hair follicle (P = 0021; Fig. 2d), follicular mucinosis (P = 0049; Fig. 2e) and sebaceous lysis (P = 0011) occurred more frequently in the facial EPF group.
Discussion
(b)
(c)
Fig 1. Epidemiological characteristics of groups of patients with facial and extrafacial eosinophilic pustular folliculitis (EPF). (a) Ages of patients with facial and extrafacial EPF, showing no significant difference. (b) Sex distribution and (c) EPF subtypes in the facial and extrafacial groups. Males and immunosuppression-associated EPF were relatively more common in the extrafacial than in the facial group.
topical steroid. Of 42 patients, 19 (45%) showed cleared lesions, 11 (26%) showed the presence of lesions at last followup, and the remaining 12 (29%) could not be followed up. Histological findings Analysis of eosinophil infiltrates showed that a perifollicular pattern was the most common in both the facial and extrafacial groups with the highest score (Table 1). However, differences became evident when the patterns of eosinophil infiltration were compared. Perifollicular infiltration (Fig. 2a) was significantly more common in the facial than in the extrafacial EPF group (P = 0031), whereas perivascular patterns (Fig. 2b) were significantly more common in the extrafacial © 2013 British Association of Dermatologists
The three subtypes of EPF differ significantly in clinical features and in their response to treatments.7,9,11 For example, most cases of classical and infantile EPF form plaques, whereas plaque occurs in only 50% of patients with immunosuppression-associated EPF.11 In addition, subtypes differ in their sites of occurrence. Classical-type EPF arises mainly on the face (88%), but can also involve the extremities (26%) and hands/feet (18%).11 By contrast, although the face is the most frequent site of immunosuppression-associated EPF, the face is not affected in 33% of these patients and the hands and feet are not affected.11 Hence, although both classical and immunosuppression-associated EPF most commonly affect the face, the latter more frequently involves extrafacial areas.11 Despite differences in the distribution of lesions among EPF subtypes, clinicohistopathological characteristics according to affected site had not been evaluated previously. Therefore, to our knowledge this study is the first to describe the clinicohistopathological differences between facial and extrafacial EPF. The most commonly involved sites of extrafacial EPF in our patient cohort were the extremities, followed by the buttocks, trunk and scalp. Although both sexes were similarly affected by EPF, the male to female ratio differed by affected site. We found that men were more frequently affected by extrafacial EPF than women, with a ratio of 55 : 1.0, but women were more frequently affected by facial EPF, with a ratio of 2 : 1. Consistent with previous findings,11 we found that extrafacial EPF was more frequently associated with immunosuppressionassociated EPF (38%) than was facial EPF (6%). We also observed differences in the clinical morphology of facial and extrafacial EPF. Whereas the frequency of pustule formation did not differ, annular plaques were more frequently observed in our facial EPF group. Moreover, although a perifollicular pattern of eosinophil infiltration was the most common in both EPF groups, perivascular and interstitial patterns were also observed, with the patterns of eosinophil infiltration differing in patients with facial and extrafacial EPF. The frequencies of perifollicular eosinophil infiltration, follicular mucinosis and exocytosis of inflammatory cells in the follicle were higher in the facial than in the extrafacial EPF group, suggesting that inflammatory reactions around the hair follicles are more intense in facial EPF. By contrast, perivascular eosinophil infiltration occurred more frequently in patients with extrafacial EPF. The pathophysiology of EPF remains unclear. The differences in clinical features and histopathological findings we observed in groups of patients with facial and extrafacial EPF British Journal of Dermatology (2014) 170, pp1173–1176
1176 Facial and extrafacial EPF, W.J. Lee et al.
(a)
(b)
(c)
(d)
(e)
Fig 2. Representative histopathological findings in patients with eosinophilic pustular folliculitis (EPF). (a) Perifollicular eosinophil infiltration in a patient with facial EPF. (b) Perivascular eosinophil infiltration in a patient with extrafacial EPF. (c) Interstitial infiltration of eosinophils between collagen bundles. (d) Exocytosis of inflammatory cells in the hair follicle and (e) follicular mucinosis around the affected follicle in facial EPF. Haematoxylin and eosin stain, original magnification (a–d) 9100; (e) 9200.
suggest that different pathogenic mechanisms may participate in the development of this disease, depending on the affected site. Differences in the form, distribution and histopathology of skin lesions in different classes of EPF suggest fundamental clinical differences among subtypes that largely derive from the affected site. However, further studies are needed to elucidate the pathogenesis of facial and extrafacial EPF, and to clarify whether EPF subtypes represent different diseases.
References 1 Ofuji S, Ogino A, Horio T et al. Eosinophilic pustular folliculitis. Acta Derm Venereol (Stockh) 1970; 50:195–203. 2 Fujiyama T, Tokura Y. Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis. J Dermatol 2013; 40:419–23. 3 Takematsu H, Nakamura K, Igarashi M, Tagami H. Eosinophilic pustular folliculitis: reports of two cases with review of the Japanese literature. Arch Dermatol 1985; 121:917–20. 4 Rosenthal D, LeBoit PE, Klumpp L, Berger TG. Human immunodeficiency virus-associated eosinophilic folliculitis. A unique
British Journal of Dermatology (2014) 170, pp1173–1176
5
6
7
8
9 10
11
dermatosis associated with advanced human immunodeficiency virus infection. Arch Dermatol 1991; 127:206–9. Keida T, Hayashi N, Kawashima M. Eosinophilic pustular folliculitis following autologous peripheral blood stem-cell transplantation. J Dermatol 2004; 31:21–6. Tang MB, Tan E, Chua SH. Eosinophilic pustular folliculitis (Ofuji’s disease) in Singapore: a review of 23 adult cases. Australas J Dermatol 2003; 44:44–7. Fearfield LA, Rowe A, Francis N et al. Itchy folliculitis and human immunodeficiency virus infection: clinicopathological and immunological features, pathogenesis and treatment. Br J Dermatol 1999; 141:3–11. Ishiguro N, Shishido E, Okamoto R et al. Ofuji’s disease: a report on 20 patients with clinical and histopathologic analysis. J Am Acad Dermatol 2002; 46:827–33. Nervi SJ, Schwartz RA, Dmochowski M. Eosinophilic pustular folliculitis: a 40 year retrospect. J Am Acad Dermatol 2006; 55:285–9. Lee JY, Tsai YM, Sheu HM. Ofuji’s disease with follicular mucinosis and its differential diagnosis from alopecia mucinosa. J Cutan Pathol 2003; 30:307–13. Katoh M, Nomura T, Miyachi Y, Kabashima K. Eosinophilic pustular folliculitis: a review of the Japanese published works. J Dermatol 2013; 40:15–20.
© 2013 British Association of Dermatologists
British Journal of Dermatology
C O N CI S E C O M M U N I C A T I ON
Facial and extrafacial eosinophilic pustular folliculitis: a clinical and histopathological comparative study W.J. Lee, K.H. Won, C.H. Won, S.E. Chang, J.H. Choi, K.C. Moon and M.W. Lee Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnapdong Songpagu, Seoul 138-736, Korea
Summary Correspondence Mi Woo Lee. E-mail: [email protected]
Accepted for publication 23 November 2013
Funding sources None.
Conflicts of interest None declared. DOI 10.1111/bjd.12755
Background Although more than 300 cases of eosinophilic pustular folliculitis (EPF) have been reported to date, differences in clinicohistopathological findings among affected sites have not yet been evaluated. Objectives To evaluate differences in the clinical and histopathological features of facial and extrafacial EPF. Methods Forty-six patients diagnosed with EPF were classified into those with facial and extrafacial disease according to the affected site. Clinical and histopathological characteristics were retrospectively compared, using all data available in the patient medical records. Results There were no significant between-group differences in subject ages at presentation, but a male predominance was observed in the extrafacial group. In addition, immunosuppression-associated type EPF was more common in the extrafacial group. Eruptions of plaques with an annular appearance were more common in the facial group. Histologically, perifollicular infiltration of eosinophils occurred more frequently in the facial group, whereas perivascular patterns occurred more frequently in the extrafacial group. Follicular mucinosis and exocytosis of inflammatory cells in the hair follicles were strongly associated with facial EPF. Conclusions The clinical and histopathological characteristics of patients with facial and extrafacial EPF differ, suggesting the involvement of different pathogenic processes in the development of EPF at different sites.
What’s already known about this topic?
•
Although the eruption of eosinophilic pustular folliculitis (EPF) primarily affects the face, rare lesions may be located at extrafacial sites.
What does this study add?
• •
The clinical features and histopathological findings of facial and extrafacial EPF are different. These differences suggest that different pathogenic mechanisms may participate in the development of EPF, depending on the affected site.
Ofuji disease, or eosinophilic pustular folliculitis (EPF), initially described in 1970,1 primarily affects the face, but lesions can also be located on the trunk and extremities, i.e. extrafacial sites. EPF is characterized by repeated pruritic follicular papules and pustules superimposed on plaques with central clearing and peripheral extension. EPF has been divided into several subtypes, including the classical, infantile and immunosuppression-associated types.2,3 These three subtypes are
considered to be within the spectrum of EPF as they present with similar clinicopathological features. However, many differences exist, and it has been proposed that these three subtypes be viewed as different disease entities.4,5 A key histopathological finding in patients with EPF is a dense inflammatory infiltrate around the pilosebaceous unit, consisting predominantly of eosinophils and mononuclear cells.6 Perivascular and dermal infiltrates of eosinophils and lymphocytes
© 2013 British Association of Dermatologists
British Journal of Dermatology (2014) 170, pp1173–1176
1173
1174 Facial and extrafacial EPF, W.J. Lee et al.
have also been observed in patients with this disease.6–8 Pustules containing eosinophils are frequently observed but are not necessarily required for the diagnosis of EPF, as some patients with EPF lack this characteristic.9,10 Although approximately 300 cases of EPF have been reported to date, differences in clinicohistopathological findings relative to affected sites (facial or extrafacial) have not been analysed. This study was therefore designed to compare the clinical and histopathological features of patients with facial and extrafacial EPF.
Materials and methods All patients diagnosed with EPF who were evaluated at Asan Medical Center, Seoul, Korea, between June 1998 and July 2013 were retrospectively assessed. Only patients in whom infectious processes had been ruled out and had tissue eosinophilia confirmed by skin biopsy were included. Patients with simultaneous involvement of facial and extrafacial areas and those without eosinophil infiltration around pilosebaceous units were excluded. Our study cohort consisted of 46 patients, 33 with facial and 13 with extrafacial EPF. Histopathological analysis Eosinophil infiltration patterns were classified as perivascular, perifollicular or interstitial. The intensity of the infiltrate was scored between 0 and 3 (0, absence of infiltrate; 1, faint infiltrate; 2, moderate infiltrate; and 3, intense infiltrate). The presence of follicular mucinosis, flame figure, exocytosis of inflammatory cells in the hair follicle, and epidermal changes such as crust, hyperkeratosis and acanthosis was evaluated. Sebaceous lysis with a cellular infiltrate and spongiosis in the sebaceous gland were also assessed, as was the presence of infiltrates of other inflammatory cells such as neutrophils. Statistical analysis All data were analysed statistically using SPSS version 18.0 (IBM, Armonk, NY, U.S.A.). P-values < 005 were considered statistically significant. The presence of certain clinical and histopathological findings were compared in the facial and extrafacial EPF groups using the v2 test or Fisher’s exact test. The intensity of eosinophil infiltrates in three patterns (perivascular, perifollicular and interstitial) was compared using a linearby-linear association test.
Results Clinical and laboratory findings The clinical features of the patients with EPF who were analysed in this study are summarized in Table 1. Age at presentation was similar in the facial and extrafacial EPF groups (P = 0924; Fig. 1a). The percentage of male patients was significantly higher in the extrafacial (11 of 13, 85%) than in the facial (11 of 33, 33%) EPF group (P = 0003; Fig. 1b). British Journal of Dermatology (2014) 170, pp1173–1176
Table 1 Clinical and histopathological characteristics of patients with facial (n = 33) and extrafacial (n = 13) eosinophilic pustular folliculitis (EPF) Facial EPF, n (%)
Extrafacial EPF, n (%)
Age, years (range) 380 (9–64) 393 Sex (male: female) 1:2 55 : 1 Clinical subtypes Classical type 31/33 (94) 8/13 IS-associated type 2/33 (6) 5/13 HIV-associated 0/33 1/13 Malignancy1/33 (3) 4/13 associated Transplantation1/33 (3) 0/13 associated Infantile type 0/33 0/13 Clinical morphology Plaque formation 15/33 (45) 3/13 Annular plaque 9/33 (27) 0/13 formation Multiple papules 17/33 (52) 10/13 Pustules 6/33 (18) 3/13 Pruritus 22/33 (67) 10/13 Hypereosinophilia 4/29 (14) 2/9 in blood Histopathological features Eosinophil infiltration (estimated scores) Perifollicular 191 153 pattern Perivascular 075 146 pattern Interstitial 103 062 pattern Follicular 16/33 (48) 2/13 mucinosis Exocytosis of 21/33 (64) 3/13 inflammatory cells Sebaceous lysis 12/33 (36) 0/13 Flame figure 3/33 (9) 1/13 Epidermal change 12/33 (36) 4/13 Neutrophil 5/33 (15) 3/13 infiltration
P-value
(12–69)
0924 0003*
(62) (38) (3) (31)
0014* 0014*
(23) (0)
0197 0035*
(77) (23) (77) (22)
0184 0698 0724 0613
0031* 0013* 0143 (15)
0049*
(23)
0021*
(8) (31) (23)
0011* 0637 1 0372
*Statistically significant. IS, immunosuppression.
Extrafacial EPF lesions were located on the legs of five patients, the arms of four, the buttocks of three, the inguinal area of two, the trunk of two, the hand of one patient and the scalp of one patient. Immunosuppression-associated EPF was significantly more common in the extrafacial than in the facial EPF group (P = 0014; Fig. 1c). Plaque formation was observed in 15 of 33 patients (45%) with facial EPF and in three of 13 (23%) with extrafacial EPF. Annular plaques were significantly more common in the facial group (P = 0035), but pustule formation was similar in the two groups (P = 0698). The frequency of blood hypereosinophilia was similar in the facial and extrafacial groups (14% vs. 22%, P = 0613). Of the 46 patients, 21 (46%) were treated with dapsone, 13 (28%) with minocycline, seven (15%) with naproxen and three (7%) with © 2013 British Association of Dermatologists
Facial and extrafacial EPF, W.J. Lee et al. 1175
(a)
group (P = 0013). However, there were no between-group differences in the frequency of interstitial patterns (P = 0143; Fig. 2c). Exocytosis of inflammatory cells in the hair follicle (P = 0021; Fig. 2d), follicular mucinosis (P = 0049; Fig. 2e) and sebaceous lysis (P = 0011) occurred more frequently in the facial EPF group.
Discussion
(b)
(c)
Fig 1. Epidemiological characteristics of groups of patients with facial and extrafacial eosinophilic pustular folliculitis (EPF). (a) Ages of patients with facial and extrafacial EPF, showing no significant difference. (b) Sex distribution and (c) EPF subtypes in the facial and extrafacial groups. Males and immunosuppression-associated EPF were relatively more common in the extrafacial than in the facial group.
topical steroid. Of 42 patients, 19 (45%) showed cleared lesions, 11 (26%) showed the presence of lesions at last followup, and the remaining 12 (29%) could not be followed up. Histological findings Analysis of eosinophil infiltrates showed that a perifollicular pattern was the most common in both the facial and extrafacial groups with the highest score (Table 1). However, differences became evident when the patterns of eosinophil infiltration were compared. Perifollicular infiltration (Fig. 2a) was significantly more common in the facial than in the extrafacial EPF group (P = 0031), whereas perivascular patterns (Fig. 2b) were significantly more common in the extrafacial © 2013 British Association of Dermatologists
The three subtypes of EPF differ significantly in clinical features and in their response to treatments.7,9,11 For example, most cases of classical and infantile EPF form plaques, whereas plaque occurs in only 50% of patients with immunosuppression-associated EPF.11 In addition, subtypes differ in their sites of occurrence. Classical-type EPF arises mainly on the face (88%), but can also involve the extremities (26%) and hands/feet (18%).11 By contrast, although the face is the most frequent site of immunosuppression-associated EPF, the face is not affected in 33% of these patients and the hands and feet are not affected.11 Hence, although both classical and immunosuppression-associated EPF most commonly affect the face, the latter more frequently involves extrafacial areas.11 Despite differences in the distribution of lesions among EPF subtypes, clinicohistopathological characteristics according to affected site had not been evaluated previously. Therefore, to our knowledge this study is the first to describe the clinicohistopathological differences between facial and extrafacial EPF. The most commonly involved sites of extrafacial EPF in our patient cohort were the extremities, followed by the buttocks, trunk and scalp. Although both sexes were similarly affected by EPF, the male to female ratio differed by affected site. We found that men were more frequently affected by extrafacial EPF than women, with a ratio of 55 : 1.0, but women were more frequently affected by facial EPF, with a ratio of 2 : 1. Consistent with previous findings,11 we found that extrafacial EPF was more frequently associated with immunosuppressionassociated EPF (38%) than was facial EPF (6%). We also observed differences in the clinical morphology of facial and extrafacial EPF. Whereas the frequency of pustule formation did not differ, annular plaques were more frequently observed in our facial EPF group. Moreover, although a perifollicular pattern of eosinophil infiltration was the most common in both EPF groups, perivascular and interstitial patterns were also observed, with the patterns of eosinophil infiltration differing in patients with facial and extrafacial EPF. The frequencies of perifollicular eosinophil infiltration, follicular mucinosis and exocytosis of inflammatory cells in the follicle were higher in the facial than in the extrafacial EPF group, suggesting that inflammatory reactions around the hair follicles are more intense in facial EPF. By contrast, perivascular eosinophil infiltration occurred more frequently in patients with extrafacial EPF. The pathophysiology of EPF remains unclear. The differences in clinical features and histopathological findings we observed in groups of patients with facial and extrafacial EPF British Journal of Dermatology (2014) 170, pp1173–1176
1176 Facial and extrafacial EPF, W.J. Lee et al.
(a)
(b)
(c)
(d)
(e)
Fig 2. Representative histopathological findings in patients with eosinophilic pustular folliculitis (EPF). (a) Perifollicular eosinophil infiltration in a patient with facial EPF. (b) Perivascular eosinophil infiltration in a patient with extrafacial EPF. (c) Interstitial infiltration of eosinophils between collagen bundles. (d) Exocytosis of inflammatory cells in the hair follicle and (e) follicular mucinosis around the affected follicle in facial EPF. Haematoxylin and eosin stain, original magnification (a–d) 9100; (e) 9200.
suggest that different pathogenic mechanisms may participate in the development of this disease, depending on the affected site. Differences in the form, distribution and histopathology of skin lesions in different classes of EPF suggest fundamental clinical differences among subtypes that largely derive from the affected site. However, further studies are needed to elucidate the pathogenesis of facial and extrafacial EPF, and to clarify whether EPF subtypes represent different diseases.
References 1 Ofuji S, Ogino A, Horio T et al. Eosinophilic pustular folliculitis. Acta Derm Venereol (Stockh) 1970; 50:195–203. 2 Fujiyama T, Tokura Y. Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis. J Dermatol 2013; 40:419–23. 3 Takematsu H, Nakamura K, Igarashi M, Tagami H. Eosinophilic pustular folliculitis: reports of two cases with review of the Japanese literature. Arch Dermatol 1985; 121:917–20. 4 Rosenthal D, LeBoit PE, Klumpp L, Berger TG. Human immunodeficiency virus-associated eosinophilic folliculitis. A unique
British Journal of Dermatology (2014) 170, pp1173–1176
5
6
7
8
9 10
11
dermatosis associated with advanced human immunodeficiency virus infection. Arch Dermatol 1991; 127:206–9. Keida T, Hayashi N, Kawashima M. Eosinophilic pustular folliculitis following autologous peripheral blood stem-cell transplantation. J Dermatol 2004; 31:21–6. Tang MB, Tan E, Chua SH. Eosinophilic pustular folliculitis (Ofuji’s disease) in Singapore: a review of 23 adult cases. Australas J Dermatol 2003; 44:44–7. Fearfield LA, Rowe A, Francis N et al. Itchy folliculitis and human immunodeficiency virus infection: clinicopathological and immunological features, pathogenesis and treatment. Br J Dermatol 1999; 141:3–11. Ishiguro N, Shishido E, Okamoto R et al. Ofuji’s disease: a report on 20 patients with clinical and histopathologic analysis. J Am Acad Dermatol 2002; 46:827–33. Nervi SJ, Schwartz RA, Dmochowski M. Eosinophilic pustular folliculitis: a 40 year retrospect. J Am Acad Dermatol 2006; 55:285–9. Lee JY, Tsai YM, Sheu HM. Ofuji’s disease with follicular mucinosis and its differential diagnosis from alopecia mucinosa. J Cutan Pathol 2003; 30:307–13. Katoh M, Nomura T, Miyachi Y, Kabashima K. Eosinophilic pustular folliculitis: a review of the Japanese published works. J Dermatol 2013; 40:15–20.
© 2013 British Association of Dermatologists
