Eosinophilic pustular folliculitis

Volume 14 Number 3 March, 1986

pemphigoid-like histologic features) who benefit from the addition of dapsone to the therapeutic regimen. REFERENCES I. Camisa C, Neff JC, Olsen RG: Use of indirect immunofluorescence in the lupus erythematosus/lichen planus overlap syndrome: An additional diagnostic clue. J AM ACAD DERMATOL 11:1050-1059, 1984. 2. Camisa C, Olsen RG, Yohn 11: Differentiating bullous lichen planus and lichen planus pemphigoides. J AM ACAD DERMATOL 11:1164-1166, 1984. (Letter to Editor.) 3. Olsen RG, DuPlessis DP, Schulz EJ, Camisa C: Indirect immunofluorescence microscopy of lichen planus. Hr J Dermatol 110:9-15, 1984. 4. Ackerman AB: Histologic diagnosis of inflammatory skin diseases. Philadelphia, 1978, Lea & Febiger, pp. 590591; 608-610. 5. Stingl G, Holabar K: Coexistence of lichen planus and bullous pemphigoid: An immune pathological study, Br J Dermatol 93:313-320, 1975. 6. Hintner H, Tappeiner G, Honigsmann H, et al: Lichen planus and bullous pemphigoid. Acta Derm Venereal (Stockh) 59:71-76, 1979.

Bullous lichen planus

7. Mora RG, Nesbitt LT Jr, Brantley JB: Lichen planus 8. 9.

10. 11. 12. 13. 14, 15.

pemphigoides: Clinical and immunofluorescent findings in four cases. JAM ACAD DERMATOL 8:331-336,1983. Blair SE: Lichen planus pemphigoides: Report of a case and discussion of the literature. Arch Dermatol Syph 58:138-148, 1948. Sobel S, Miller R, Shaten H: Lichen planus pemphigoides: Immunofluorescence findings. Arch Dermatol 112:1280-1283, 1976. Schwartz B: Lichen planus pemphigoides. Proc R Soc Med 48:445-447, 1955. Lang PG Jr, Maize JC: Coexisting lichen planus and bullous pemphigoid or lichen planus pemphigoides? J AM ACAD DERMATOL 9:133-140, 1983. Camisa C, Allen CM, Bowen B, Olsen RG: Indirect immunofluorescence of oral lichen planus. J Oral Patho!. (In press.) Gammon WR, Briggaman RA, Woodley DT, et al: Epidermolysis bullosa acquisita-a pemphigoid-like disease. J AM ACAD DERMATOL 11:820-832, 1984. Rogers RS ill, Seehafer JR, Perry HO: Treatment of cicatricial (benign mucous membrane) pemphigoid with dapsone. J AM ACAD DERMATOL 6:215-223, 1982. Falk DK, Latour DL, King LE Jr: Dapsone in the treatment of erosive lichen planus. J AM ACAD DERMATOL 12:567-570, 1985.

Eosinophilic pustular folliculitis Andrea S. Colton, M.D.,* Lawrence Schachner, M.D.,** and Alexander P. Kowalczyk, M.D. *** Miami, FL Eosinophilic pustular folliculitis was first described by Ofuji et al in 1970. It is characterized by pruritic circinate plaques that are studded with follicular papules and pustules. Lesions are located chiefly on the face, trunk, and arms. Biopsies of lesions demonstrate an infiltrate of eosinophils and neutrophils within hair follicles, dermis, and epidermis. Peripheral leukocytosis and eosinophilia are c,ommon. (J AM ACAD DERMATOL 14:469-474, 1986.)

From the Department of Dermatology and Cutaneous Surgery*'**'*** and the Department of Pediatrics,** University of Miami School of Medicine. Accepted for publication Oct. 18, 1985. Reprint requests to: Dr. Lawrence Schachner, University of Miami School of Medicine, Department of Dermatology and Cutaneous Surgery, P,O. Box 016960 (04-8), Miami, FL 33101/305-547-

6742.

Since the first cases of eosinophilic pustular folliculitis were described in 1970, I twenty additional cases have been published. Sixteen of these cases were from Japan and four from Europe. 2-7 An additional five cases of a possible variant of eosinophilic pustular folliculitis in infancy have been reported from the United States and Canada. 8 Pru469

470

Colton et al

Journal of the American Academy of Dermatology

Fig. 1. A plaque of eosinophilic pustular folliculitis on the chest. Fig. 2. Grouped papules and pustules surmount the plaque. Fig. 3. Spongiosis and diffuse infiltration of eosinophils are seen in this hair follicle. (Hematoxylin-eosin stain; X 200.) Fig. 4. Diffuse infiltration with eosinophils of hair follicle, showing spongiosis and partial destruction. (Hematoxylin-eosin stain; X 200.) Fig. 5. Infiltration of sebaceous gland with eosinophils. (Hematoxylin-eosin stain; X 100.) Fig. 6. Spongiosis with exocytosis of eosinophils in overlying epidermis. (Hematoxylineosin stain; x 100.)

Volume 14 Number 3 March, 1986

ritic, red, indurated circinate plaques studded with sterile papules and pustules are the characteristic classical findings of eosinophilic pustular folliculitis in adults. The borders of the plaques show slow centrifugal extension with central clearing of the lesions. The areas of central clearing leave a residual hyperpigmentation. The distribution is usually asymmetric, with greatest involvement of the face, trunk, and upper extremities. Rarely, lesions of the scalp, neck, axillae, groin, legs, palms, and sales have been described. In the infantile formS a predominance of scalp lesions has been cited. The occasional distribution of plaques in areas lacking hair follicles has led some authors to prefer the name eosinophilic pustulosis over eosinophilic pustular folliculitis. The histopathologic picture of eosinophilic pustular folliculitis reveals an eosinophilic and neutrophilic perifollicular and follicular infiltrate. Spongiotic and subcorneal accumulations of epidermal eosinophils and neutrophils have also been reported. Peripheral leukocytosis and eosinophilia are common. The typical clinical course of eosinophilic pustular folliculitis includes spontaneous remission and exacerbations. Treatment with dapsone and/or prednisone may decrease or clear the lesions. CASE REPORT A 39-year-old white man presented with a recurrent eruption after a 12-year remission. The patient, a 39year-old physician, was born in Brazil but had lived and worked in the United States for many years. At the time of the initial consultation the patient had a 6-month history of pruritic, red, annular and circinate plaques studded with pustules. They involved the chest, upper and lower portions of the back, arms, and forehead. Prior to consultation, therapy had been attempted with tetracycline, hydrocortisone cream, fluorinated steroids, topical antibiotics, topical antifungal preparations, and griseofulvin. All of these therapeutic trials had proved unsuccessful. The patient thought that the lesions were becoming increasingly indurated and were spreading centrifugally. He believed that this eruption was similar to one experienced 12 years prior to this presentation. Biopsy 12 years before, in Brazil, was reported to demonstrate "an acute folliculitis." Treatment at that time included topical steroid preparations applied under occlusion. The lesions persisted over 3 months' time and then resolved. The patient's past med-

Eosinophilic pustular folliculitis

471

ical history and review of systems were otherwise unremarkable. He denied any exposure to halogens, including radiocontrast dyes. Physical examination. The patient's forehead, upper arms, chest (Figs. I and 2), and back revealed a large number of discrete and confluent, well-circumscribed, red, annular and circinate plaques. They varied in size from I X 2 em to greater than 8 X to cm. The plaques were infiltrated and caused the forehead to have a leonine appearance. Most plaques demonstrated central clearing with areas of hyperpigmentation. Many ofthe plaques were surmounted by grouped white, 1- to 3-mm pustules and red papules. Laboratory studies. At presentation, Gram's stain, Wright's stain, and bacterial cultures of the pustules were done. Gram's stain revealed numerous polymorphonuclear leukocytes but no bacterial organisms. Wright's stain demonstrated an admixture of eosinophils and polymorphonuclear leukocytes. Quantitative cytologic analysis of Wright's stain revealed 33% eosinophils, 56% polymorphic leukocytes, and 11 % lymphocytes. Quantitative analysis of the follicular infiltrate, on biopsy, demonstrated a predominantly eosinophilic infiltrate. The bacterial cultures were negative. Potassium hydroxide preparations and fungal cultures were likewise negative. Three 3-mm punch biopsies were obtained from different plaques. Diffuse infiltration of hair follicles, with eosinophils, was noted at all levels. Associated spongiosis and focal destruction of the follicular structures were present. Infiltration of sebaceous glands with eosinophils was seen as well. Focal spongiosis with exocytosis of eosinophils was seen in the overlying epidermis (Figs. 3 to 6). A moderately dense superficial and deep perivascular infiltrate, composed predominantly of eosinophils with lymphocytes and histiocytes, was also present. Additional studies showed a peripheral white blood cell count of 9,200 with a differential count of 52% neutrophils, 33% lymphocytes, 10% eosinophils, 2% bands, 2% monocytes, and 1% basophils. A complete chemical profile, platelet count, urinalysis, and determinations of total serum protein, albumin, globulin, and antinuclear antibodies levels were all unremarkable. Patient management. The clinical examination and the histopathologic findings were consistent with the diagnosis of eosinophilic pustular folliculitis. Review of the literature suggested therapeutic trials of prednisone and/or dapsone. Initial therapy with prednisone, 40 mg/day, afforded some improvement within 3 weeks' time. The addition of dapsone, 100 mg daily, brought considerable improvement to both the number and severity of lesions. With this improvement, tapering

472

Journal of the Amencan Academy of Dermatology

Colton et al

Table I. RevIew of twenty-four adult patIents wIth eos1OophIhc pustular folhcuhtts

Case No

Reference

Race/natIOnal ongm

Sex

Age at onset (yr)

Dlstrlbutlon

1

Ise and Ofu]II

Japanese

F

42

Face, trunk, arms

2

OfU]1 et al2

Japanese

M

25

Face, arms, trunk,

legs

WBC/%EOS (durmg active dIsease)

14,100/80% Dexamethasone, 0 102 mg/day Dapsone Sulfapyndme 18,800/41% MPI-Pemcillm Slgmamycm Gnseofulvm Dapsone Sulfapyndme Betamethasone 6,800/15% SUlfapyndme Dapsone

3

OfU]1 et aF

Japanese

M

18

Face, arms,

4

OfU]I et aF

Japanese

M

25

legs Face, anus, back

11,500123%

5 6 7

IshIbashI et a13 IshIbashI et al3 IshIbashI et al 3

trunk,

Rx

Japanese

M

33

Face, chest

16,000/11%

Japanese

F

25

7,100/18%

Japanese

F

44

Face, trunk, arms Face

9,800/?

Lmcomyclll Dapsone Betamethasone va1erdte (0 12% topIcal) Betamethasone (oral) CortIcosteroIds (oral) Oxyphenbutazone Oral antibIOtIcs TopIcal antifungals Betamethasone (oral) Betamethasone + ACTH (m] ) ACTH + topIcal betamethasone (omtment)

Response

+ +

+/-

+ + +/-

+ + + + + +

ACTH Adrenocorticotropic hormone, ROS eosmophlls, InJ lflJectJon, WBC whIte blood cell count

of predmsone to 20 mg every other day WIth contmued utIlIzatIOn of dapsone, 100 mg/day, has provIded a satIsfactory ma10tenance therapy PerSIstent leSIons and new leSIOns have also been attenuated WIth the use of mtraleslOnal tnamcmolone, 3 mg/mI, as well as topIcal o 1% trlamcmolone preparatIOns DISCUSSION

ThIS patIent's chmcal leSIons and hIStologIC findmgs are charactensttc of eOSInOphIlIc pustular follIculItIs, as descnbed by Ise and OfU]I I As of thIS date, there have been at least twelve cases of thIS entlty descnbed 10 the EnglIsh-language lIterature, and addltlonal eleven cases 10 the Japanese

hterature, and five cases of a possIble vanant of eos1OophIhc pustular follIcuhtIs 10 10fancy Table I reVIews twenty-three adult patlents who have been reported WIth thIS entity There have been eIghteen Japanese patIents, one Chmese patient, three whIte Europeans, and one whIte Brazlhan Twenty of the patients were male and three were female The patients ranged 10 age from 13 to 72 years, WIth an average age of 30 years The twelve cases descnbed 10 the EnglIsh-language lIterature all had typIcal annular leSIOns WIth grouped and penpheral pustules and central clear109 Common 10catlOns for leSlOns were the face (ten of twelve), trunk (mne of twelve), and arm

Volume 14 Number 3 March, 1986

Eosinophilic pustular folliculitis

473

Table I. Cont'd

Case No.

Reference

8-18 Ishibashi et aP 19 Holsfl

Race/national origin

Japanese Swedish (white)

22

Face, trunk, soles

15,000/40%

13

Scalp

10,000/17%

M

19

19,000126%

German (white)

M

72

Face, trunk, limbs, buttocks Neck, axillae, groins, feet

Nunzi et al 9

Italian (white)

M

64

Left leg, chest, back, thigh

9,900/54%

Our case

Brazilian (white)

M

24

Face, trunk, arms

9,200/10%

German (white) Chinese

22

Vakilzadeh et aF

23

24

21

M (all) M

Distribution

WBC/%EOS (during active disease)

M

Orfanos and Sterri Cutler6

20

Sex

Age at onset (yr)

(seven of twelve). Rarer sites included the legs and feet, scalp, buttocks, axillae, groin, and fingers, all in two or less of the twelve patients. Analysis of the laboratory studies in these cases revealed peripheral white blood cell counts ranging from 6,800 to 18,800, with an average of 12,600. Peripheral eosinophil counts during active disease ranged from 8% to 41 %, with an average of 21 %. Our patient presented with a white blood cell count of 9,200 and a 10% eosinophilia. Our patient has been in remission for 18 months as of this writing, the eosinophil count has remained below 5%. The literature reports four positive therapeutic responses to dapsone and two treatment failures with dapsone. There was one positive therapeutic response to the combination of dapsone and prednisone. There were three negative responses and

14,200/22%

Rx

Penicillin Griseofulvin Prednisolone Sulfones Sulfapyridine Dapsone Topical steroid Tetracycline Erythromycin Dapsone + prednisone Sulfamethoxypyridazine + topical diflucortolone valerate + prednisone Erythromycin Amoxicillin Bacampicillin Piperacillin Dapsone Prednisolone See text

Response

+ +

+ +

+

one positive response to sulfapyridine. Both systemic and topical corticosteroids used as sale therapy gave mixed results. Our patient seems to have a long-lasting and satisfactory response to dapsone, 100 mg daily used in combination with 20 mg of prednisone every other day. Attempts to taper the dapsone or prednisone further have resulted in moderate exacerbations of his condition. Eosinophilic pustular folliculitis appears to be a rare cutaneous disease. Its cause is now obscure, and a definitive therapy for all patients has yet to be established. See also pages 475-482.

REFERENCES 1. Ise S, Ofuji S: Subcorneal pustular dermatosis: A follicular variant? Arch Dermatol 92:169-171, 1965. 2. Ofuji S, Ogino A. Horio T, et al: Eosinophilic pustular

474

3. 4. 5. 6.

Journal of the American Academy of Dermatology

Colton et al

folliculitis. Acta Derm Venereol (Stockh) 50: 195-203, 1970. Ishibashi A, Nishiyama Y, Miyata C, Chujo T: Eosinophilic pustular folliculitis (Ofuji). Dermatologica 149:240247, 1974. Holst R: Eosinophilic pustular folliculitis. Br J Dennatol 95:661-664, 1976. Orfanos CE, Sterry W: Sterile eosinophile pustulose. Dermatologica 157:193-205, 1978. Cutler TP: Eosinophilic pustular folliculitis. Clin Exp DermatoI6;327-332, 1981.

7. Vakilzadeh F, Suter L, Knop J, Macher E: Eosinophilic

pustulosis with pemphigus-like antibody. Dermatologica 162:265-272, 1981. 8. Lucky AW, Esterly NB, Heskel N, et al: Eosinophilic pustular folliculitis in infancy. Pediatr Dermatoll(3):202206, 1984. 9. Nunzi E, Parodi A, Rebora A: Ofuji's disease: High circulating titers of IgG and IgM directed to basal cell cytoplasm. J AM ACAD DERMATOL 12:268-273, 1985.

ABSTRACTS Oral acyclovir therapy for mucocutaneous herpes simplex virus infections in immunocompromised marrow transplant recipients Shapp DH, Newton BA, Dandliker PA, et al: Ann Intern Med 102:783-785, 1985 Double-blind, well-controlled, and sampled properly, this study convinces us that the oral acyclovir worked well in this sample. P. C. Anderson, M.D.

Scrotal and abdominal skin necrosis complicating intravenous vasopressin therapy for bleeding esophageal varices Gogel HK, Shennan RW, Becker LE: Dig Dis Sci 30:460-464, 1985 Spontaneous necroses of the skin has a long list of causes, all ominous. Among the causes are adverse reactions to drugs, many in the vasopressor group. Necrosis of scrotal and abdominal skin is discussed as due to a drug working with a complex series of synergistic causes, but not to the action of the drug alone in these cases. P. C. Anderson, M.D.

Increased risk of cervical neoplasia in consorts of men with penile condylomata acuminata Campion MI, Singer A, Clarkson PK, et al: Lancet 27:1(8435):943-946, 1985 Who knows for sure about sexual histories? However, the presumed coital partners exclusively of men with penile condyloma often develop genital warts (19 of 25) and atypia of cervical epithelium (9 of 25) that was much worse than the age-matched control group, who supposedly had no such exposure. P. C. Anderson, M.D.

Weber-Christian disease. Analysis of 15 cases and review of the literature Panush RS, Yonker RA, Dlesk A, et a1: Medicine (Baltimore) 64:181-191, 1985 Opinions abound, but little is known about the panniCUlitis group, and this interesting review of cases adds little clarification. P. C. Anderson, M.D.

High prevalence of cardiovascular diseases and enhanced activity of the renin-angiotensin system in psoriatic patients Ena P, Madeddu P, Glorioso N, et al: Acta Cardiol (Brux) 40: 199-205, 1985 An increased prevalence of hypertension and heart disease was measured in this study of 100 psoriatic people. Should both younger and older psoriatic patients be screened for cardiovascular risk factors when they first visit the dermatologist? Possibly screen them only if they have no primary care health adviser? P. C. Anderson, M.D.

Immunological abnormalities in massive cutaneous hyalinosis Maury CP, Teppo AM: Acta Med Scand 217:331336, 1985 An extremely complex immune disorder was studied in a patient who had massive cutaneous depositions of glycoproteins. This is a multiple myeloma variant, apparently. P. C. Anderson, M.D.