Case Report Dermatology 1992; 185:276-280

H. Aoyama H. Tagami Department of Dermatology, Tohoku University School of Medicine,

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Eosinophilic Pustular Folliculitis ■ Stditillü lültislly (Mlty WjtH w Palmoplantar Pustular Lesions Report of a Case and Review of the Literature

Eosinophilic pustular folliculitis Palmoplantar lesions

Abstract Wc report a 23-year-old Japanese male with eosinophilic pustular folliculitis (EPF) that had started with palmoplantar rash. Only when follicular pustules appeared on the bilateral cheek 31 months later, we revised our initial diagnosis of pustulosis palmaris et plantaris (PPP) to EPF, and all the skin eruptions cleared mostly with indomcthacin. A review of the Japanese literature for the past 20 years disclosed that in 207 cases of EPF so far reported, palmoplantar pustular lesion was noted in 38 (18%). Among them, in 16 cases (8%) the skin lesions started first from the palmoplantar region with the average period of 26 months until the appearance of other eruptions of EPF. None of them was diag­ nosed as EPF when skin lesions were localized only to the palmoplantar region. When we find pustules on the palmoplantar region, we should suspect the possi­ bility of early lesions of EPF as well as PPP. Histopathologic demonstration of multilocular pustules located in the upper epidermis containing numerous eosin­ ophils in the palmoplantar pustular lesions, together with the dramatic therapeu­ tic response to indomcthacin greatly favor the diagnosis of EPF.

In 1970, Ofuji et al. [1] first established the entity of eosinophilic pustular folliculitis (EPF). To our knowledge, more than 200 cases of EPF have been reported in Japan. There are also sporadic reports from other countries [2-7]. It usually starts as grouped pruritic follicular papulopus­ tules on the face and back. Hence, as described before in our review of the so far reported Japanese cases, in about

Received: February 17. 1992 Accepted: June 17. 1992

one fifth of them grouped pustules also appeared on the palms and/or soles, simulating pustulosis palmaris et plan­ taris (PPP) [8]. We report here a patient with EPF whose illness had started with pustules only in the palmoplantar region long before the appearance of other characteristic lesions together with a review of the Japanese cases that showed the coexistence of palmoplantar pustular eruption.

Dr. H. Aoyama Department of Dermatology Tohoku University School of Medicine l-l Sciryo machi, aobaku. Sendai 9,SO (Japan)

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Key Words

Case Report

Discussion

The present case showed only a PPP-likc palmoplantar eruption at first. Unlike PPP lie responded poorly to ton­ sillectomy to which more than 70% of PPP cases respond favorably [9-11 ]. He also showed a poor response to oral or topical treatment with corticosteroids. Follicular pustules began to appear on the cheeks only 31 months after the development of the palmoplantar eruption, being once prececdcd by transient erythema with follicular papules that lasted only 20 days on the right cheek. At this point we

Fig. 1. Scattered pustules over the right sole. Scales and crusts arc also prominent.

first suspected a diagnostic possibility of EPF and con­ firmed it histopathologically. Thereafter, we could easily manage the lesions including the palmoplantar pustules with oral indomethaein as reported in other Japanese cases of EPF [ 12—14]. Reports of EPF from Japan far exceed those from other countries [8]. Including the original description of Ise and Ofuji [15] we surveyed 207 cases of EPF reported in the Japanese literature for the past 20 years. Among the reported cases, 38 (18%) were accompanied by palmo­ plantar pustular eruption. Moreover, in 16 cases (8%), we found that their skin lesions started from the palmoplantar region as in our case. In 2 cases, the skin lesions were reported to be limited to the palmoplantar region during the whole course. For the differentiation of EPF with palmoplantar involvement from other pustular diseases, i.e., PPP, pustu-

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A 23-year-old Japanese male noted pruritic pustules on the palms and soles in September 1986. He was diagnosed to have PPP at another University Hospital. Fresh pustules continued to develop despite therapeutic trials with various topical and systemic cortico­ steroids. He visited our University Hospital in March 1987. Physical examination showed that there were scaly erythematous patches on the palms and soles and fine pustules on the tips of the fingers. No fungi were obtained from scales and pustules of the lesions on the palms and soles. Laboratory investigations showed a peripheral white blood cell count of 11,700/ul with a differential cell count of 1% eosin­ ophils. Under a temporary diagnosis of PPP we stopped oral administra­ tion of corticosteroids and started oral administration of colchicine with 1.0 mg/day and topical psoralen photochemotherapy. However, pustules continued to appear on the palms and soles (fig. 1). Tonsillec­ tomy which was performed 10 months later was not effective for the palmoplantar lesions. In June 1987. pruritic erythematous patches studded with papules suddenly developed on his right cheek, disap­ pearing after 20 days. In April 1989. pruritic fine pustules began to develop on the checks and within I week annular infiltrated erythem­ atous lesions. 5-7 cm in diameter, appeared. Many follicular pustules 1-2 mm in diameter were noted in the center of the erythematous plaque. We revised the diagnosis to EPF which was later confirmed histopathologically. The rash was treated with oral administration of indomethaein (75 mg/day) for the first time. Within 2 days, most of both the facial and palmoplantar pustular lesions cleared. Though the palmoplantar lesions returned when the indomethaein therapy was stopped 2 months later, they cleared completely again with 75 mg/day of oral indomethaein. The dose of indomethaein was gradually tapered and he was remained disease free while taking the maintenance dose of 25 mg daily. Histologic examination of a biopsy specimen from the follicular pustules on the right cheek revealed a pustule in the hair follicle that contained numerous eosinophils intermingled with neutrophils and mononuclear cells (fig. 2). These inflammatory cells were also observed around the hair follicle, invading the damaged follicular epi­ thelium. Eosinophils also infiltrated into the sebaceous glands. A biopsy specimen from the pustules on the right sole revealed subcor­ neal and intraepidermal multilocular pustules, which were filled with numerous eosinophils and neutrophils (fig. 3). These cells also invaded the surrounding edematous epidermis. These histopathological pic­ tures were consistent with EPF.

Fig. 2. Biopsy specimen from the pustules of the right cheek showing a pustule in the hair follicle filled with numerous eosinophils intermingled with neutrophils and mononuclear cells. HE. a Lower magnification, x 160. b High magnification, x 500.

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ophilia ranging from 10 to 20% was noted in 7 cases and eosinophilia over 20% was noted in 4 and below 10% in 4. The mean time period until the development of the typ­ ical lesions of EPF in the extrapalmoplantar regions was 26 months, ranging from less than 6 months in 5 cases to more than 2 years in 4; the longest was 10 years. Except for the 2 patients whose skin lesions were limited to the paimoplan­ tar region during the whole clinical course, none of them was diagnosed precisely as EPF before the development of the extrapalmoplantar lesion. They had been diagnosed erroneously to have a PPP or tinea pedis. Because the skin lesions started from the palms and/or soles in 8% of the Japanese cases of EPF, and in 2 cases they were limited to the paimoplantar region, the patients with paimoplantar pustules so far diagnosed as PPP might have included those of EPF whose skin lesions were limited to the paimoplantar region. Saruta and Nakamizo [17] reported that the paimoplantar pustules of EPF tended to be larger and often raise above the skin surface with irregu-

Eosinophilic Pustular Folliculitis

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lar psoriasis, pustular dermatophytosis, the histopathologic picture of the paimoplantar lesions seem to be most help­ ful. In contrast to the subcorneal monolocular neutrophilic pustules noted in PPP or to the spongiform multilocular neutrophilic pustules noted in pustular psoriasis, the histo­ pathologic picture of the paimoplantar lesions of EPF con­ sisted of multilocular pustules in the upper epidermis that contained numerous eosinophils together with neutrophils as shown in our case. In the 16 Japanese cases whose skin lesions started first from the palms and/or soles, 13 were males and 3 females. This male-to-female ratio corresponds to that in all cases of EPF [8]. By contrast PPP is much more common in females than in males [16]. The age of onset spread over a wide range, i.e.. the second decade in 4 cases, third in 2. fourth in 4, fifth in 4 and seventh in 2. The tendency for peripheral blood eosinophilia was not so high as compared with cases of EPF without paimoplantar pustules. Peripheral blood eosinophils accounted for 16% on the average, i.e., eosin-

Fig. 3. Biopsy specimen from the right sole showing subcorneal multilocular pustules filled with numerous eosinophils and neutrophils. HF.. a Lower magnification. x 160. b High magni­ fication. X500.

There are several recent case reports describing the dramatic effectiveness of indomethacin for EPF in Japan [12-14]. We also experienced such a therapeutic response in other patients with EPF. On the basis of the remarkable improvement on systemic treatment with the cyclooxy­ genase inhibitor indomethacin, it is possible that some cyclooxygenase-generated metabolites derived from arachidonic acid may play an important role as a chemotactic factor for eosinophils in the development of skin lesions of EPF [12, 13), while the nature of the chemotactic factor is still unknown. Sometimes the effect of indomethacin is so rapid that after 2-3 days of its oral administration most of the skin lesions clear and only a maintenance dose of 25 mg/day may be enough to control the lesions. We think that indomethacin is the first therapeutic choice for EPF and that it can also be used as a dependable diagnostic tool in the cases of palmoplantar pustular disease in addition to histopathological studies.

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lar borders and that they might affect the interdigital region culminating in erosions. Nevertheless, no cases can be surely diagnosed without histopathologic study. Our case did not show any of these clinical characteristics reported by them in his lesions. PPP is common in the West as well as in Japan. Although EPF has been relatively uncommon in the West, we think that EPF may not be rare even in the West. Probably, the awareness of the condition with readiness for biopsy in suspected cases will increase the frequency of this diagnosis in the future. Therefore, whenever we see a patient with PPP poorly responsive to its ordinary therapeutic modalities, we should suspect the possibility of EPF with palmoplantar involvement. Also we should not overlook even fleeting extrapalmoplantar lesions like the transient pruritic erythematous papules noted on the right check of our patient and should exam­ ine blood eosinophilia repeatedly and study the pustules histologically.

References

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7 Jaliman H.D. Phelps R.G. Fleischmajer R: Eosinophilic pustular folliculitis. J Am Acad Dermatol 1986:14:479-482. 8 Takematu II. Nakamura K, Igarasi M. Tagami H: Eosinophilic pustular folliculitis. Report of two cases and review of the Japanese liter­ ature. Arch Dermatol 1985:121:917-920. 9 Ono T. Jono M. Kilo M. Tonoda T. Kageshita T. Egawa K. Kuriya N: Evaluation of tonsillec­ tomy as a treatment for pustulosis palmaris et plantaris. Acta Otolaryngol Suppl 1983:401: 12-16. K) Noda Y. Ura M: Pustulosis palmaris et planta­ ris due to tonsillar focal infections. Acta Oto­ laryngol Suppl 1983:401:22-30. Il Yamanaka N. Shido F. Kataura A: Tonsil­ lectomy-induced changes in anti-keratin anti­ bodies in patients with pustulosis palmaris ct plantaris: A clinical correlation. Arch Otorhinolaryngol 1989:246:109-112.

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12 MiyauchiT. Fujigaki H. Uchara M. Hayashi S. Hironaga M: Effect of indometluicin on eosin­ ophile pustular folliculitis. Acta Dermatol (Kyoto) 1985:80:9-13. 13 Takematu II. Tagami H: Eosinophilic pustular folliculitis. Studies on possible chemotaclic fac­ tors involved in the formation of pustules. Br J Dermatol 1986:114:209-215. 14 Kato 11: Eosinophilic pustular folliculitis. Treated with imlomcthacin. Dcrmatologica 1989:179:217-218. 15 Ise S. Ofuji S: Subcorneal pustular dermatosis: A follicular varient? Arch Dermatol 1965:92: 169-171. 16 Uellgren E. Mobacken H: Pustulosis palmaris et plantaris. Prevalence, clinical observations and prognosis. Acta Derm Vcnereol 1971:51: 284-288. 17 Saruta T. Nakamizo Y: Eosinophilic pustular folliculitis. Rinsho Dermatol 1979:21:689-697.

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1 Ofuji S. Ogino A. Horio T. Ohseko T: Eosin­ ophilic pustular folliculitis. Acta Derma Vencrcol 1970;50:195-203. 2 Holst R: Eosinophilic pustular folliculitis. Report on a European case. Br J Dermatol 1976:95:661-664. 3 Cutler TP: Eosinophilic pustular folliculitis. Clin Exp Dermatol 1981:6:327-332. 4 Nunzi E. Parodi A. Rebora A: Ofuji's disease: I ligh circulating tilers of IgG and IgM directed to basal cell cytoplasm. J Am Acad Dermatol 1985:12:268-273. 5 Colton A.S. Schachner L. Kowalczyk A.P: Eosinophilic pustular folliculitis. J Am Acad Dermatol 1986:14:469-474. 6 Dcncharl S.M. Noppakun N, Solomon A.R. Smith E.B: Eosinophilic pustular folliculitis. J Am Acad Dermatol 1986:14:475-479.

Eosinophilic pustular folliculitis starting initially only with palmoplantar pustular lesions. Report of a case and review of the literature.

We report a 23-year-old Japanese male with eosinophilic pustular folliculitis (EPF) that had started with palmoplantar rash. Only when follicular pust...
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