Eosinophilic pustular folliculitis: A sterile folliculitis of unknown cause?

Eosinophilic pustular folliculitis: A sterile folliculitis of unknown cause? Sarah Brenner, MD, Ronni Wolf, MD, and Joseph Ophir, MD Tel Aviv, Israel Background: Eosinophilic pustular folliculitis (EPF) was initially definedas a sterile folliculitis of unknown cause. Becauseattempts to demonstrate bacterial organisms have been unsuccessful, and antibiotictherapy is usually ineffective, a bacterial infectionis not considered a plausible causative factor for this disease. Objective: Our purposewas to describefive patients with the clinical and histologic characteristics of EPF and to report the results of bacterial cultures. Methods: Biopsy specimens were examined and pustules were cultured. Results: In three of the five patients, Pseudomonas infectionof the hair follicle was the cause of the disease as proven by repeated cultures and the responseto specific therapy. Three patients had a systemic disorder known to cause immunologic alteration: AIDS in one and a myeloproliferative disorder in two. Conclusion: Although EPF was initially defined as a sterile folliculitis of unknown origin, three of our patientshad an identifiable and treatable cause. We believethat thesecaseswarrant the diagnosis of EPF. (J AM ACAD DERMATOL 1994;31:210-2.)

In 1970 Ofuji et al.' first described eosinophilic pustular folliculitis (EPF). They characterized the disease as follows: recurrent crops of pruritic, follicular, sterile papulopustules in fairly well-defined areas; peripheral extension with central clearing; resolution with residual pigmentation; subsequent appearance of new lesions in the areas of pigmentation; sparing of the hands, feet, and mucous membranes; absence of systemic symptoms; unpredictable response to therapeutic agents; and chronicity. The major distinguishing histologic feature is the presence of abundant eosinophils that invadesebaceous glands and the outer root sheath of hair follicles. This is accompanied by nuclearfragmentation, a dermal perivascular infiltrate composed predominantly of eosinophils, and intraepidermal microabscess formation.l? Since its initial description in 1970, the spectrum of EPF has expanded and many cases havebeendescribedthat do not fit allthe criteriaproposed by the From the Department of Dermatology, Tel Aviv Sourasky Medical Center, Ichilov Hospital,andSadder Facultyof Medicine, Tel Aviv University. Accepted for publication Feb. 18, 1994. Reprint requests: Sarah Brenner, MD, Department of Dermatology, Ichilov Hospital,6 Weizman St., Tel Aviv 64239, Israel. Copyright @ 1994 by the American Academy of Dermatology, Inc, 0190-9622/94 $3.00 + 0 16/1/55302

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firstauthors.Thediseasemayoccasionally bechronic and persistent, especially in patients with AIDS,4 rather than exacerbatingand remitting. Lesions are not invariably pruritic- 6 and not necessarily follicular. In 18% of patients with EPF, lesions are also presenton the palms and soles." Therefore the accuracy of the designation folliculitis has been questioned.s 8, 9 The question can be raised asto what specific and diagnostic criteria should be used to establish the diagnosis of EPF. We believe that Soeprono and Schimella'" givea satisfactoryanswer. They stated that "Even though the clinical presentation may vary, the histological pattern of EPF is diagnostic. The pustular folliculitis composed predominantly of eosinophils is not a pattern seenin any other entity." EPF was initiallydefined as a sterilefolliculitis of unknown origin. I, 2 Because previous attempts to demonstrate bacterial organisms have been unsuccessful and antibiotics are usually ineffective, 11-14 a bacterialinfection is not considered a plausible cause for this disease. We describe five patients with the histologic characteristics of EPF and with some clinical features resembling those reportedby Ofuji et al.' The unusual aspect of these cases was that in three of them a causative factor was identified as a Pseudomonas infection of hair follicles.

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Brenner et al. 211

Table I. Clinical and laboratory findings Patient 1

Age (yr)/Sex Distribution of lesions Associated diseases Medication

Isolation from lesion Leukocytes Eosinophils CD4 counts Immunefluorescence

41/F Chest, abdomen, buttocks, extremities Myeloproliferative Prednisone, aldospirone, warfarin, folic acid Pseudomonas 8700 4%

35/M Scalp, face, back

Ofloxacin

Response to treatment and course

Excellent, recurrence after stopping

Patient 3

47/F Back, buttocks, lower abdomen None

Patient 4

PatientS

48/F Back, buttocks, hip

M/36 Scalp, face, chest, back None None

AZT, ddC, Resprim

None

Myeloproliferative None

Negative

Pseudomonas

Pseudomonas

Negative

4100 14.6% 10

7400 1.8% 663 Negative

5700 1.5% 1140

Topicalsteroids (clobetasol propionate) Fair

Ofloxacin

Ofloxacin

Excellent, recurrence after stopping

Excellent, recurrence after stopping

7300 1% 770 Linear IgG DEJ-lesional and nonlesional skin Topical steroids (clobetasol propionate) Fair

AIDS

Negative

Treatment

AZT,

Patient 2

Zidovudine; ddC, dideoxycytidine; DEl, dermoepidermal junction; Resprim, trimethoprin-sulfamethoxazole.

CASE REPORT

The main clinical and laboratory features of the five patientsare summarized in Table I. One illustrative case report is presented. A 41-year-oldwomanhad a 6-weekhistoryofitchyand painful papulopustules on her abdomen. Pseudomonas aeruginosa was isolatedfrom her lesions and a diagnosis of gram-negative folliculitis was made. She deniedexposure to a hot tub, whirlpool, or public swimming pool. Treatment withofloxacin, 200mg twicedailyfor 2weeks, resulted in complete clearance. The lesions recurred 1 month after antibiotic therapy had been stopped and clearedcompletely again withofloxacin. She had another four episodes that responded to reintroduction of antibiotic therapy. While taking a daily maintenance dose of ofloxacin, 200 mg, she remained free of disease. This patient has a myeloproliferative disorder diagnosedin October 1982becauseof splenomegaly resulting from splenic and portal veinthrombosis, thrombocytopenia, prominent ecchymoses, and heavy menses. She was treated with warfarin sodiumand hydroxyurea. Later an immune hemolytic anemia developed. She was treated with steroids, cimetidine, and warfarin sodium. In December 1986she underwentsplenectomy. Sincethen her

generalcondition has remained stable. Shehas been taking low doses of steroids, aldospirone, warfarin sodium, and folic acid. Histologic examination revealed a deepfolliculitis with a dense eosinophilic and neutrophilic infiltrate in the follicle lumen and the surrounding area, consistent with EPF. Direct immunofluorescence on involved and uninvolved skin, as well as indirectimmunofluorescence were negative. The histologic features in this case, as well as in the othercases with Pseudomonas infection (cases 3 and 4), were almost identicalto thosewith negative cultures. DISCUSSION

EPF is considered to be a distinctive clinical and histologic disorder of unknown origin, easily differentiated from other pustular conditions. However, there are some reports of cases with histologic and clinical features identical to those described for EPF, but in whom a causative agent was identified.P:" Thus the question should be raised, "Does the presence of a treatable cause for the disease rule out the diagnosis of EPF?/1 We believe that the answer to this is negative.

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All our patients had histologic features identical to those described for EPF. None of the three patients in whom Pseudomonas infection was considered the cause had the characteristic facial involvement and the typical annular polycyclic configuration of the eruption. However, they did show some of the characteristic clinical features of EPF. They had recurrent crops of pruritic follicular papulopustules in fairly well-defined areas, a chronic course, the appearance of new lesions in previously involved areas, and the absence of systemic symptoms. Although Pseudomonas folliculitis superimposed on EPF cannot be absolutely excluded, the fact that specific antibiotic therapy brought about dramatic resolution of all lesions speaks strongly against this possibility. The question remains whether this and similar cases warrant the diagnosis of EPF or whether this is a different disease of known origin with similar clinical and identical histologicfindingsto idiopathic EPF. We believe the former is true; although previous attempts to demonstrate an etiologic factor for this disease were unsuccessful, this should not exclude all caseswith a knowncause from the category of EPF. EPF was not thought to be associated with systemic disease until 1986 when some cases were observed in patients with AIDS. 10, 18, 19 Only a few cases of EPF associated with other disorders with immunologic alterations have been reported now.20-22 However, three of our patients had a systemic disorder known to cause immunologic alterations.

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4. Moritz DL, Elmets CA. Eosinophilic pustular folliculitis. J AM ACAD DERMATOL 1991;24:903-7. 5. Rosenthal D, LeBoit PE, Klumpp L, et al. Human immunodeficiency virus-associated eosinophilic folliculitis. Arch DermatoI1991;127:206-9. 6. Taieb A, Bassan-Andrieu L, Maleville J. Eosinophilic pustulosis of the scalp in childhood. J AM ACAD DERMATOL 1992;27:55-60. 7. Aoyama H, Tagami H. Eosinophilic pustular folliculitis starting initially only with palmoplantar pustular lesion: report of a case and review of the literature. Dermatology 1992;185:276-80. 8. Saruta T, Nakamizo Y. Eosinophilic pustular folliculitis. Rinsho DermatoI1979;21:689-97. 9. Wolf R. Kuritzky J. Eosinophilic pustular folliculitis vs eosinophilic pustulosis. Dermatology (In press.) 10. Soeprono FF, Schimella RA. Eosinophilic pustular folliculitis in patients with acquired immunodeficiency syndrome. JAM ACAD DERMATOL 1986;14:1020-2. 11. Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987;174:53-6. 12. Cutler TP. Eosinophilic pustular folliculitis. Clin Exp DermatoI1981;6:327-32. 13. Holst R. Eosinophilic pustular folliculitis. Br J Dermatol 1976;95:661-4. 14. Nunzi E, Parodi A, Rebora A. Ofuji's disease: high circulating titers of IgG and IgM directed to basal cell cytoplasm. JAM ACAD DERMATOL 1985;12:268-73. 15. Haupt HM, Stern JB, Weber CB. Eosinophilic pustular folliculitis: Fungal folliculitis? J AM ACAD DERMATOL 1990;23:1012·4. 16. Kuo TT, Chen SY, Can HL. Tinea infection histologically simulating eosinophilic pustular folliculitis. J Cutan Pathol 1986;13:118-22. 17. Czarnetzki BM, Springorum M. Larva migrans with eosinophilic papular folliculitis. Dermatologica 1982; 164:36-40. 18. Jenkins D, Fisher BK, Chalvardjian A, et al. Eosinophilic pustular folliculitis in a patient with AIDS. Int J Dermatol 1988;27:34-5. 19. Buchness MR, Lim HW, Hatcher VA, et al. Eosinophilic pustular folliculitis in the acquired immunodeficiency disorder. N Engl J Med 1988;318:1183-6. 20. Rodger H, Souteyrand P, Bigon YJ, et al. Pustulose folliculaire a eosinophiles relevant un liphone T de haut degre de malignite rapidement fatal. Ann Dermatol Venereal 1988;115:1209-12. 21. Barkley A, Shall L, Millard LG. Simultaneous onset of eosinophilic pustular dermatosis and non-Hodgkin's lymphoma. Abstracts, 1st Congress, European Academy of Dermatology & Venereology, Firenze, Sept 25-28; 1989, P 210. 22. Partizi A, Di Lernia V, Neri J, et al. Eosinophilic pustular folliculitis (Ofuji disease) and non-Hodgkin lymphoma. Acta Derm Venereal (Stockh) 1992;72:146-7.