Eosinophilic pustular folliculitis: Fungal folliculitis? Helen M. Haupt, MD, a Jere B. Stern, MD, b and Charles B. Weber, DO*
Philadelphia, Pennsylvania, Damascus, Maryland, and Falls Church, Virginia Eosinophilic pustular folliculitis is a rare condition with no known cause. However, a fungal cause is suggested by (1) tinea-like annular lesions, (2) initial clinical consideration of fungal folliculitis in many reported cases, (3) an association with the acquired immunodeficiency syndrome, and (4) an association with scaly and vesiculopustular lesions of palms and soles. We report a case histologically identical to eosinophilic pustular foliiculitis but in which hyphal fungal organisms were demonstrated in one of the involved hair follicles. (J AM AcAo DERMA-rOL 1990;23:1012-4.) Eosinophilic pustular folliculitis is characterized by pruritic follicular papules and pustules of the face, trunk, and extremities and is often accompanied by peripheral blood leukocytosis and eosinophilia. 1-s Although the lesions progress in slowly growing, tinea-like fashion with central clearing, 61~ previous attempts to demonstrate fungal organisms by culture have been unsuccessful.i, 2, 8-11 In many reported cases no special stains to demonstrate fungal organisms histologically were described, 1,6,10 or, if performed, no fungus could be identified. 4' 8,11 Because cultures and histologic examination m a y be negative for fungus, the biopsy interpretation is especially important. Therefore the following question is raised: How specific is the histologic pattern in the diagnosis of eosinophilic pustular folliculitis? In a recent report Soeprono and Schinella 12 stated, "Even though the clinical presentation may vary, the histologic pattern of eosinophilic pustular folliculitis is diagnostic. The pustular folliculitis composed predominantly of eosinophils is not a pattern seen in any other entity." We report a case histologically identical to eosinophilic pustular folliculitis but in which hyphal fungal organisms were demonstrated in one of the involved hair folli6les.
Fig. 1. Erythematous patch with follicular papules, pusrules, and nodules on right leg.
CASE REPORT
A 30-year-old woman had a pruritic, 8 cm patch of erythematous papules, pustules, and tender nodules (Fig. From the Pennsylvania I-Iospital,Philadelphia, a and Dermatopathology Consultation Services, DamascusP Reprint requests: Helen M. Haupt, MD, Department of Pathology, Pennsylvania Hospital, Eighth and SpruceStreets., Philadelphia, PA 19t07. *In private practice, Falls Church, Va. 16/4/16616
1012
1). This lesion had gradually progressed from a smaller pruritic, erythematous, vesicular patch during a 4-month period. A biopsy specimen obtained 2 months previously was reported by a different laboratory to show no fungal organisms with periodic acid--Schiff (PAS) stain. Repeated biopsy was performed on the lesion. Histologic examination revealed an acute and chronic folliculitis with almost complete destruction of one hair follicle (Fig. 2). A dense eosinophilic and neutrophilie infiltrate in the central portion of the follicle and a surrounding infiltrate
Volume 23 Number 5, Part 2 November 1990
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Eosinophilic pustular folliculitis." Fungal folliculitis? 1013
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Fig. 2. Folliculitis with almost complete destruction of hair follicle. (Hematoxylin-eosin stain; X30.) of numerous eosinophils, lymphocytes, and occasional neutrophils were present (Fig. 3). An adjacent hair follicle showed numerous eosinophils infiltrating the outer root sheath. The lumen of this follicle on a few of the many sections taken from the paraffin block showed hyphal fungal organisms with PAS stain (Fig. 4). Subsequently a dermatophyte, not further identified, grew in culture. Treatment with griseofulvin (Gris-PEG), 250 mg twice daily for 4 weeks and then 125 mg twice daily for 2 weeks, resulted in complete clearance of the lesion. DISCUSSION Eosinophilic pustular foUiculitis was initially defined as a sterile folliculitis of unknown etiology.1 However, several factors suggest a fungal cause: ( 1) annular or circinate lesions that slowly spread peripherally with central clearing, 6I~ (2) initial clinical consideration of dermatophytosis in some reported cases, 9-11 (3) association with the acquired immunodeficiency syndrome, 1214 (4) association with scaly and/or vesiculopustular lesions of palms and soles, 1~ l l, 15 (5) published clinical photographs strongly suggestive of dermatophytosis, 2' 9-11,15 and (6) concomitant tinea pedis and corporis in one case. 12 The histologic features of this case are identical to those described for eosinophilic pustular follicu-
Fig. 3. High-power view of partially destroyed hair follicle with inflammatory infiltrate composed primarily of eosinophils. (Hematoxylin-eosin stain; X625.) litis.t2, 16 Kuo et al. 17 also recently noted this similarity. They reported a patient with a tinea infection illustrating the histologic identity of eosinophilic pustular folliculitis and fungal folliculitis. However, they concluded that the presence of fungal organisms in their case ruled out the diagnosis of eosinophilic pustular folliculitis. The presence of numerous eosinophils in the inflammatory infiltrate of an occasional example of fungal folliculitis is not surprising. Occasionally a marked dermal eosinophilic infiltrate may be observed in biopsy specimens from superficial tinea infections. 18 Furthermore, the subcorneal pustules from tinea infections of the palms and soles may show predominantly eosinophils, Is similar to the subcorneal pustules reported in some cases of eosinophilic pustular folliculitis. 9 Although fungal organisms have not been readily identified in association with lesions of eosinophilic pustular folliculitis either by special stains performed on histologic sections 1,6,1~ or by culture, l, z, 8-11 the absence of easily demonstrable organisms does not rule out the diagnosis of fungal foiliculitis. In addition to this case, it has been the previously unpublished experience of one of us
1014
Journal of the American Academy of Dermatology
Haupt et al.
dermatophyte cause. The burden is on the pathologist to rule out fungal folliculitis before rendering a histologic diagnosis of eosinophilic pustular folliculitis. REFERENCES
Fig.
4. Adjacent hair follicle with inflammatory infiltrate consisting mostly of eosinophils in outer root sheath and surrounding dermis. Arrows indicate hyphal fungal organisms. (Hematoxylin-eosin stain; • Inset, PAS stain; X825.)
(J. B. S.) that even with multiple PAS-stained sections taken through the entire paraffin block, only one or a few sections may reveal diagnostic fungal organisms in the hair follicles. Failure to demonstrate fungal organisms on sections from the original biopsy specimen in this case is an example of this problem. Another possibility is that the inflammatory process may be sufficiently severe to destroy viable fungal organisms, similar to the occasional difficulty in identifying fungal organisms in cases of tinea capitis or tinea barbae with marked inflammatory reactions. 19,20 A third consideration is that eosinophilic pustular folliculitis may represent a hypersensitivity reaction to dermatophytosis. 12 No therapeutic modality has been consistently effective. In the small number of cases in which griseofulvin has been tried, the results have not been impressive.lO, 11 However, neither dosage nor duration of treatment were stated and steroids also were given in one case. t~ In conclusion, we postulate that at least some cases of eosinophilic pustular folliculitis may have a
1. [se S, Ofuji S. Subcorneal pustular dermatosis: a follicular variant? Arch Dermatol 1965;92:169-71. 2. Orfanos CE, Sterry W. Sterile eosinophile pustulose. Dermatologica 1978;157:193-205. 3. Takematsu H, Tagami H. Eosinophilic pustular folliculitis: studies on possible chemotactic factors involved in the formation of pustules. Br .I Dermatol 1986;114:209-15. 4. Jaliman HD, Phelps RG, Fleischmajer R. Eosinophilic pustular folliculitis. J AM ACAD DERMATOL1986;14:47982. 5. Camaeho-Martinez F. Eosinophilic pustular folliculitis [Letter]. J AM ACAD DERMATOL 1987;17:686-8. 6. Colton AS, Schachner L, Kowalczyk AP. Eosinophilic pustular folliculitis. J AM ACAD DERMATOL1986;14:46974. 7. Malanin G, Helander I. Eosinophilic pustular folliculitis (Ofuji's disease): response to dapsone but not to isotretinoin therapy. J AM ACAD DERMATOL1989;20:1121. 8. Dinehart SM, Noppakun N, Solomon AR, et al. Eosinophilic pustular folliculitis..l AM ACAD DERMATOL 1986; 14:475-9. 9. Ishibashi A, Nishiyama Y, Miyata C, et al. Eosinophilie pustular folliculitis (Ofuji). Dermatologica 1974;149: 240-7. 10. Hoist R. Eosinophilic pustular follieulitis: report of a European case. Br J Dermatol 1976;95:661-4. 11. Steffen C. Eosinophilic pustular folliculitis (Ofuji's disease) with response to dapsone therapy. Arch Dermatol 1985; 121:921-3. 12. Soeprono FF, Schinella RA. Eosinophilic pustular folliculitis in patients with acquired immunodeficiency syndrome: report of three cases. J AM ACAD DERMATOL 1986;14: 1020-2. 13. Jenkins D, Fisher BK, Chalvardjian A, et al. Eosinophilic pustular folliculitis in a patient with AIDS. Int J Dermatol 1988;27:34-5. 14. Buchness MR, Lim HW, Hatcher VA, et al. Eosinophilic pustular folliculitis in the acquired immunodeficiency syndrome: treatment with ultraviolet B therapy. N Engl J Med 1988;318:1183-6. 15. Takematsu H, Nakamura K, Igarashi M, et al. Eosinophilic pustular folliculitis: report of two cases with a review of the Japanese literature. Arch Dermatol 1985;121:91720. 16. Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987;174:53-6. 17. Kuo TT, Chen SY, Chan HL. Tinea infection histologically simulating eosinophilic pustular folliculitis. J Cutan Pathol 1986;13:118-22. 18. Ackerman AB. Histologlc diagnosis of inflammatory skin diseases. Philadelphia: Lea & Febiger, 1978:229, 574. 19. Birt AR, Wilt JC. Mycology, bacteriology, and histopathology of suppurative ring-worm. Arch Dermatol 1954; 69:441-8. 20. Zaslow L, Derbes VJ. The immunologic nature of kerion celsi formation. Dermatol Int 1971;8:1-4.
Philadelphia, Pennsylvania, Damascus, Maryland, and Falls Church, Virginia Eosinophilic pustular folliculitis is a rare condition with no known cause. However, a fungal cause is suggested by (1) tinea-like annular lesions, (2) initial clinical consideration of fungal folliculitis in many reported cases, (3) an association with the acquired immunodeficiency syndrome, and (4) an association with scaly and vesiculopustular lesions of palms and soles. We report a case histologically identical to eosinophilic pustular foliiculitis but in which hyphal fungal organisms were demonstrated in one of the involved hair follicles. (J AM AcAo DERMA-rOL 1990;23:1012-4.) Eosinophilic pustular folliculitis is characterized by pruritic follicular papules and pustules of the face, trunk, and extremities and is often accompanied by peripheral blood leukocytosis and eosinophilia. 1-s Although the lesions progress in slowly growing, tinea-like fashion with central clearing, 61~ previous attempts to demonstrate fungal organisms by culture have been unsuccessful.i, 2, 8-11 In many reported cases no special stains to demonstrate fungal organisms histologically were described, 1,6,10 or, if performed, no fungus could be identified. 4' 8,11 Because cultures and histologic examination m a y be negative for fungus, the biopsy interpretation is especially important. Therefore the following question is raised: How specific is the histologic pattern in the diagnosis of eosinophilic pustular folliculitis? In a recent report Soeprono and Schinella 12 stated, "Even though the clinical presentation may vary, the histologic pattern of eosinophilic pustular folliculitis is diagnostic. The pustular folliculitis composed predominantly of eosinophils is not a pattern seen in any other entity." We report a case histologically identical to eosinophilic pustular folliculitis but in which hyphal fungal organisms were demonstrated in one of the involved hair folli6les.
Fig. 1. Erythematous patch with follicular papules, pusrules, and nodules on right leg.
CASE REPORT
A 30-year-old woman had a pruritic, 8 cm patch of erythematous papules, pustules, and tender nodules (Fig. From the Pennsylvania I-Iospital,Philadelphia, a and Dermatopathology Consultation Services, DamascusP Reprint requests: Helen M. Haupt, MD, Department of Pathology, Pennsylvania Hospital, Eighth and SpruceStreets., Philadelphia, PA 19t07. *In private practice, Falls Church, Va. 16/4/16616
1012
1). This lesion had gradually progressed from a smaller pruritic, erythematous, vesicular patch during a 4-month period. A biopsy specimen obtained 2 months previously was reported by a different laboratory to show no fungal organisms with periodic acid--Schiff (PAS) stain. Repeated biopsy was performed on the lesion. Histologic examination revealed an acute and chronic folliculitis with almost complete destruction of one hair follicle (Fig. 2). A dense eosinophilic and neutrophilie infiltrate in the central portion of the follicle and a surrounding infiltrate
Volume 23 Number 5, Part 2 November 1990
9
-.
Eosinophilic pustular folliculitis." Fungal folliculitis? 1013
i
i::i,
Fig. 2. Folliculitis with almost complete destruction of hair follicle. (Hematoxylin-eosin stain; X30.) of numerous eosinophils, lymphocytes, and occasional neutrophils were present (Fig. 3). An adjacent hair follicle showed numerous eosinophils infiltrating the outer root sheath. The lumen of this follicle on a few of the many sections taken from the paraffin block showed hyphal fungal organisms with PAS stain (Fig. 4). Subsequently a dermatophyte, not further identified, grew in culture. Treatment with griseofulvin (Gris-PEG), 250 mg twice daily for 4 weeks and then 125 mg twice daily for 2 weeks, resulted in complete clearance of the lesion. DISCUSSION Eosinophilic pustular foUiculitis was initially defined as a sterile folliculitis of unknown etiology.1 However, several factors suggest a fungal cause: ( 1) annular or circinate lesions that slowly spread peripherally with central clearing, 6I~ (2) initial clinical consideration of dermatophytosis in some reported cases, 9-11 (3) association with the acquired immunodeficiency syndrome, 1214 (4) association with scaly and/or vesiculopustular lesions of palms and soles, 1~ l l, 15 (5) published clinical photographs strongly suggestive of dermatophytosis, 2' 9-11,15 and (6) concomitant tinea pedis and corporis in one case. 12 The histologic features of this case are identical to those described for eosinophilic pustular follicu-
Fig. 3. High-power view of partially destroyed hair follicle with inflammatory infiltrate composed primarily of eosinophils. (Hematoxylin-eosin stain; X625.) litis.t2, 16 Kuo et al. 17 also recently noted this similarity. They reported a patient with a tinea infection illustrating the histologic identity of eosinophilic pustular folliculitis and fungal folliculitis. However, they concluded that the presence of fungal organisms in their case ruled out the diagnosis of eosinophilic pustular folliculitis. The presence of numerous eosinophils in the inflammatory infiltrate of an occasional example of fungal folliculitis is not surprising. Occasionally a marked dermal eosinophilic infiltrate may be observed in biopsy specimens from superficial tinea infections. 18 Furthermore, the subcorneal pustules from tinea infections of the palms and soles may show predominantly eosinophils, Is similar to the subcorneal pustules reported in some cases of eosinophilic pustular folliculitis. 9 Although fungal organisms have not been readily identified in association with lesions of eosinophilic pustular folliculitis either by special stains performed on histologic sections 1,6,1~ or by culture, l, z, 8-11 the absence of easily demonstrable organisms does not rule out the diagnosis of fungal foiliculitis. In addition to this case, it has been the previously unpublished experience of one of us
1014
Journal of the American Academy of Dermatology
Haupt et al.
dermatophyte cause. The burden is on the pathologist to rule out fungal folliculitis before rendering a histologic diagnosis of eosinophilic pustular folliculitis. REFERENCES
Fig.
4. Adjacent hair follicle with inflammatory infiltrate consisting mostly of eosinophils in outer root sheath and surrounding dermis. Arrows indicate hyphal fungal organisms. (Hematoxylin-eosin stain; • Inset, PAS stain; X825.)
(J. B. S.) that even with multiple PAS-stained sections taken through the entire paraffin block, only one or a few sections may reveal diagnostic fungal organisms in the hair follicles. Failure to demonstrate fungal organisms on sections from the original biopsy specimen in this case is an example of this problem. Another possibility is that the inflammatory process may be sufficiently severe to destroy viable fungal organisms, similar to the occasional difficulty in identifying fungal organisms in cases of tinea capitis or tinea barbae with marked inflammatory reactions. 19,20 A third consideration is that eosinophilic pustular folliculitis may represent a hypersensitivity reaction to dermatophytosis. 12 No therapeutic modality has been consistently effective. In the small number of cases in which griseofulvin has been tried, the results have not been impressive.lO, 11 However, neither dosage nor duration of treatment were stated and steroids also were given in one case. t~ In conclusion, we postulate that at least some cases of eosinophilic pustular folliculitis may have a
1. [se S, Ofuji S. Subcorneal pustular dermatosis: a follicular variant? Arch Dermatol 1965;92:169-71. 2. Orfanos CE, Sterry W. Sterile eosinophile pustulose. Dermatologica 1978;157:193-205. 3. Takematsu H, Tagami H. Eosinophilic pustular folliculitis: studies on possible chemotactic factors involved in the formation of pustules. Br .I Dermatol 1986;114:209-15. 4. Jaliman HD, Phelps RG, Fleischmajer R. Eosinophilic pustular folliculitis. J AM ACAD DERMATOL1986;14:47982. 5. Camaeho-Martinez F. Eosinophilic pustular folliculitis [Letter]. J AM ACAD DERMATOL 1987;17:686-8. 6. Colton AS, Schachner L, Kowalczyk AP. Eosinophilic pustular folliculitis. J AM ACAD DERMATOL1986;14:46974. 7. Malanin G, Helander I. Eosinophilic pustular folliculitis (Ofuji's disease): response to dapsone but not to isotretinoin therapy. J AM ACAD DERMATOL1989;20:1121. 8. Dinehart SM, Noppakun N, Solomon AR, et al. Eosinophilic pustular folliculitis..l AM ACAD DERMATOL 1986; 14:475-9. 9. Ishibashi A, Nishiyama Y, Miyata C, et al. Eosinophilie pustular folliculitis (Ofuji). Dermatologica 1974;149: 240-7. 10. Hoist R. Eosinophilic pustular follieulitis: report of a European case. Br J Dermatol 1976;95:661-4. 11. Steffen C. Eosinophilic pustular folliculitis (Ofuji's disease) with response to dapsone therapy. Arch Dermatol 1985; 121:921-3. 12. Soeprono FF, Schinella RA. Eosinophilic pustular folliculitis in patients with acquired immunodeficiency syndrome: report of three cases. J AM ACAD DERMATOL 1986;14: 1020-2. 13. Jenkins D, Fisher BK, Chalvardjian A, et al. Eosinophilic pustular folliculitis in a patient with AIDS. Int J Dermatol 1988;27:34-5. 14. Buchness MR, Lim HW, Hatcher VA, et al. Eosinophilic pustular folliculitis in the acquired immunodeficiency syndrome: treatment with ultraviolet B therapy. N Engl J Med 1988;318:1183-6. 15. Takematsu H, Nakamura K, Igarashi M, et al. Eosinophilic pustular folliculitis: report of two cases with a review of the Japanese literature. Arch Dermatol 1985;121:91720. 16. Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987;174:53-6. 17. Kuo TT, Chen SY, Chan HL. Tinea infection histologically simulating eosinophilic pustular folliculitis. J Cutan Pathol 1986;13:118-22. 18. Ackerman AB. Histologlc diagnosis of inflammatory skin diseases. Philadelphia: Lea & Febiger, 1978:229, 574. 19. Birt AR, Wilt JC. Mycology, bacteriology, and histopathology of suppurative ring-worm. Arch Dermatol 1954; 69:441-8. 20. Zaslow L, Derbes VJ. The immunologic nature of kerion celsi formation. Dermatol Int 1971;8:1-4.
