|Year : 2021 | Volume
| Issue : 3 | Page : 132-136
Eosinophilic pustular folliculitis associated with Demodex overgrowth or demodicosis on the face – A report of five cases
Hui-Peng Huang1, Chao-Kai Hsu2, Sheau-Chiou Chao2, Chao-Chun Yang2, Gwo-Shing Chen3, Chih-Hung Lin4, Chang-Ming Huang5, Julia Yu-Yun Lee2
1 Huang Hui-Peng Dermatology Clinic, Tainan, Taiwan
2 Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan
3 Department of Dermatology, Kaohsiung Medical University Hospital, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
4 Department of Pathology, Kaohsiung Municipal Hsiaokang Hospital, Kaohsiung, Taiwan
5 Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan
|Date of Submission||06-Feb-2021|
|Date of Decision||23-May-2021|
|Date of Acceptance||28-May-2021|
|Date of Web Publication||20-Sep-2021|
Dr. Julia Yu-Yun Lee
Department of Dermatology, National Cheng Kung University College of Medicine and Hospital, 138 Sheng-Li Rd, Tainan
Source of Support: None, Conflict of Interest: None
Eosinophilic pustular folliculitis (EPF) is classified into Ofuji disease, immunosuppression-associated EPF, and infancy-associated EPF. The association of EPF with Demodex infestation is rarely reported. We report five cases of EPF with Demodex overgrowth. All patients were young females presenting with recurrent, itchy papulopustules on the face for 2 months to 2 years. Laboratory tests revealed eosinophilia (n = 2) and iron deficiency anemia (n = 1). Skin biopsy of papulopustular lesions was performed in each patient, and all revealed folliculocentric infiltration with eosinophils. Infundibular pustules containing Demodex mites were found in two patients. All patients had high Demodex densities examined by superficial needle-scraping method (7–18 mites/5 pustules) and/or thumbnail-squeezing method (37–255 mites/cm2). Based on the clinical and laboratory findings, the patients were either first treated as having demodicosis or as having EPF. According to the treatment responses, the cases might represent Demodex-induced EPF or EPF-like reaction (Cases 1–3) or demodicosis superimposed on EPF (Cases 4 and 5). In sum, we report five cases of EPF with Demodex overgrowth (demodicosis). These cases illustrate that the possibility of Demodex overgrowth should be considered in cases of EPF with incomplete treatment response to indomethacin and/or oral corticosteroids. Conversely, EPF should be considered in cases of demodicosis with incomplete responses to antiparasitic treatment.
Keywords: Demodex, demodicosis, eosinophilic pustular folliculitis, superficial needle-scraping method, thumbnail-squeezing method
|How to cite this article:|
Huang HP, Hsu CK, Chao SC, Yang CC, Chen GS, Lin CH, Huang CM, Lee JY. Eosinophilic pustular folliculitis associated with Demodex overgrowth or demodicosis on the face – A report of five cases. Dermatol Sin 2021;39:132-6
|How to cite this URL:|
Huang HP, Hsu CK, Chao SC, Yang CC, Chen GS, Lin CH, Huang CM, Lee JY. Eosinophilic pustular folliculitis associated with Demodex overgrowth or demodicosis on the face – A report of five cases. Dermatol Sin [serial online] 2021 [cited 2022 Nov 28];39:132-6. Available from: https://www.dermsinica.org/text.asp?2021/39/3/132/326267
| Introduction|| |
Eosinophilic pustular folliculitis (EPF), eosinophilic folliculitis, or Ofuji disease is characterized by pruritic papulopustules clinically, and eosinophil-rich folliculocentric inflammatory infiltration, often with infundibular pustules and eosinophilic microabscess in the sebaceous lobules histologically. The etiology of EPF is unknown, but EPF-like reaction has been reported in lesions induced by Demodex mites or follicular dermatophytosis. Demodex mites are common commensals of the pilosebaceous unit but can be pathogenic when present in excessive number or penetrate into the dermis.
Herein, we report five cases of EPF associated with high Demodex folliculorum densities. The diagnosis of EPF was based on clinical and pathological findings. High Demodex density (Dd) was defined as ≥12 mites/cm2 by the thumbnail-squeezing method (TSM) or ≥3 mites/5 follicles by superficial needle-scraping method (SNS). The patients gave their written informed consents. The study was approved by the Institutional Review Board of National Chen-Kung University Hospital (IRB No.: A-ER-107-385).
| Methods|| |
We reviewed all EPF patients with Demodex overgrowth from Sept 2013 to Dec 2017.
| Results|| |
Five cases fulfilled the criteria. [Supplementary Table 1] and [Figure 1].
|Figure 1: Clinical features, Demodex overgrowth, and histopathology of five cases with eosinophilic pustular folliculitis associated with demodicosis on the face (H and E, ×100 and ×400).|
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| Report of Cases|| |
A 24-year-old woman presented with pruritic, erythematous papulopustules on the cheeks, chin, and temples for 1 year. The rash initially responded to treatment for eczema but relapsed soon. On her first visit to our clinics, a Dd of 18 mites/5 pustules was detected by SNS. Treatment for demodicosis was initiated, and after 6 weeks of oral metronidazole 250 mg tid and topical crotamiton 10% plus hydrocortisone 0.25% cream, most pustules resolved, but some papular lesions persisted. Biopsy of a papule showed a perifollicular infiltrate of neutrophils mixed with eosinophils. The skin lesions resolved after 1-week prednisolone 5 mg tid and 2-week indomethacin 25 mg tid without recurrence in the ensuing 4 years.
A 24-year-old woman presented with a large erythematous plaque on the right cheek with scattered itchy papulopustules on both cheeks and the chin for 18 months. She visited a medical center 16 months previously. A skin biopsy showed a folliculocentric eosinophil-rich infiltrate, and there was peripheral eosinophilia (10.9%). The rash improved after 7 weeks of prednisolone 5 mg bid. She then was treated with indomethacin 25 mg bid-tid intermittently in the next 14 months. Because the facial rash still waxed and waned, she came to us for further help. Demodex overgrowth (64 mites/cm2) was detected by TSM. Topical benzyl benzoate 20% lotion was added, resulting in marked improvement of the rash with no recurrence during the 3-year follow-up period.
A 24-year-old woman presented with a 2-year history of pruritic erythematous papulopustules on the cheeks and chin and facial erythema with flushing and had been treated for eczema elsewhere. On her first visit, our tentative diagnosis was EPF or demodicosis. A skin biopsy revealed folliculocentric eosinophilic infiltration histopathologically and a Dd of 37 mites/cm2 by TSM and 7 mites/5 pustules by SNS. HIV serology was negative. The papulopustular lesions improved after 3 weeks of indomethacin 25 mg tid, but facial erythema and flushing exacerbated. After adding topical ivermectin 1% cream for 4 weeks, erythema and papulopustules resolved with a undetectable Demodex count, and flushing was well controlled with carvedilol 6.25 mg bid. No recurrence was noted during the 2-year follow-up period.
A 24-year-old woman presented with pruritic red papulopustules and plaques on the face and neck for 2 years. Six months previously, she visited a local dermatologist where the facial lesions responded to indomethacin 25 mg tid, but worsened afterward, requiring prednisolone 10–30 mg/day for 2 months to control. Then, she came to us for further help. Three papules biopsied at different time points all showed a folliculocentric eosinophil-rich infiltrate with infundibular pustules containing multiple Demodex mites in two of the specimens. A Dd of 225 mites/cm2 was detected by TSM after 1-week prednisolone. Hemogram revealed mild leukocytosis (11,500 cells/mm3) with eosinophilia (7%). EPF with overlapping features of demodicosis was considered. The rash showed a severe rebound after discontinuation of prednisolone but eventually resolved after a 2-week tapering dose of prednisolone (30–10 mg/d) and 6 weeks of topical ivermectin. Six months later, a mild recurrence of papulopustules responded to indomethacin 25 mg bid and prednisolone 5 mg bid. The Dd remained very low, 1 mite/5 pustules.
A 38-year-old woman presented with pruritic red papules, nodules, and pustules on the face and neck for 2 months and had been treated with prednisolone and doxycycline by an LMD without improvement. She then came to us. EPF or demodicosis was considered. A skin biopsy revealed an eosinophil-rich perifollicular infiltrate with multiple infundibular pustules containing neutrophils mixed with eosinophils and Demodex mites. The infiltrate also involved sebaceous glands. The findings were consistent with EPF. Hemogram showed microcytic hypochromic anemia. Ferritin levels were 6.1 ng/ml. HIV screening was negative. EPF with overlapping features of demodicosis was considered in this patient. After biopsy, the rash was treated with indomethacin 25 mg tid, γ-BHC biw, and permethrin 5% cream qd for 7 weeks with partial improvement. Since the Dd remained high (10 mites/5 pustules by SNS and 52 mites/cm2 by TSM), the medication was changed to topical ivermectin qd, tacrolimus 0.1% bid, combined with indomethacin and a 7-week tapering dose of prednisolone. The rash almost resolved 3 months after initiation of ivermectin. In the following 8 months, The Dd remained low (2 mites/cm2) and minor recurrences of papules and pustules were treated with tacrolimus ointment and intermittent intralesional triamcinolone (10 mg/ml) injection as maintenance therapy. Finally, the rashes totally cleared 8 more months later.
| Discussion|| |
We described five cases of EPF associated with either Demodex overgrowth or demodicosis, based on the clinical and pathologic features, high Demodex density-associated EPF, and the treatment responses. In Cases 1 and 2, topical crotamiton and benzyl benzoate were used to treat Demodex overgrowth, as topical permethrin and ivermectin were unavailable then.
The histology of all five cases was consistent with EPF or Ofuji disease, but two cases (Cases 4 and 5) also displayed Demodex mites within infundibular pustules, as seen in demodicosis. Ofuji disease is characterized by pruritic papulopustules with annular configuration preferentially affecting the face with postinflammatory hyperpigmentation. The skin lesions in our patients lacked annular configuration. However, it has been suggested that facial papular rash with eosinophilic infiltration of hair follicles and rapid response to oral indomethacin, but lacking pustules and peripheral extension may be proposed the term “episodic eosinophilic dermatosis of the face.”
Histopathology of demodicosis is characterized by excessive Demodex mites in follicular infundibula with infundibular pustules containing mites or mites in the perifollicular infiltrate, which consists of lymphohistiocytic infiltrates, often with abundant neutrophils. Although the Demodex overgrowth in our patients might had been predisposed by rosacea (Case 3) or the prior use of corticosteroids (Cases 1, 3, 4, and 5), the histological changes of perifollicular lymphohistiocytosis and many eosinophils were not the classical features of demodicosis. Reports of Demodex-associated eosinophilic infiltration are scant. Sabater-Marco et al. reported a case of eosinophilic follicular reaction induced by Demodex mites with eosinophilic follicular microabscess and flame figure. The rash resolved completely after 1-month treatment of topical corticosteroids and oral indomethacin. Nara et al. reported a case of eosinophilic folliculitis with a Demodex folliculorum infestation in a HIV-positive man successfully treated with ivermectin. Another case of EPF with Demodex mites presenting with pruritic erythematous papules and pustules and plaques on the face and upper back was effectively treated with indomethacin farnesil within a few weeks. Eosinophils were observed in the perifollicular infiltrate in 16 of 73 patients with rosacea.
The diagnosis and treatment choice in our five patients were challenging or problematic because of the overlapping clinical features of EPF and demodicosis during the clinical course [[Supplementary Table 2] for differences and diagnostic clues of demodicosis, classical EPF, Demodex-induced EPF/EPF-like reaction, and demodicosis superimposed on EPF]. Based on clinical presentation, skin biopsy findings, and Dd, Case 1 was treated for demodicosis first, then for EPF; the opposite order was done for Cases 2 and 3. Cases 4 and 5 seemed to have EPF with Demodex overgrowth in the middle of the clinical course. These five cases could be diagnosed as EPF associated with Demodex overgrowth (demodicosis). However, further clarification of the relationship between EPF and Demodex overgrowth may provide a better treatment strategy for individual patients. To the best of our judgment, Cases 1–3 seemed to have demodicosis mainly with Demodex-induced EPF or EPF-like reaction. The skin rash only responded partially to treatments for EPF or demodicosis alone, but complete or near-complete resolution was achieved after being treated for both diseases without recurrence in 2–4 years. Cases 4 and 5 appeared to have demodicosis superimposed on EPF, which had partial response to indomethacin and prednisolone, but had a good response with low Demodex densities and minor recurrences after adding ivermectin cream and/or tacrolimus. Since Case 3 also had facial flushing, papulopustular rosacea with Demodex overgrowth should be included in the differential diagnosis.
Based on our experience and the previous reports,,,, an algorithm for the diagnosis and treatment of such cases is proposed in [Figure 2]. Alternatively, a combination of indomethacin and topical ivermectin can be initiated after evidence of both diseases are identified, but topical ivermectin is more expensive and may cause transient local flare of papulopustules in the first few days.
|Figure 2: Diagnostic algorithm and treatment suggestions for eosinophilic pustular folliculitis with Demodex overgrowth. In patients unwilling to have a skin biopsy, a therapeutic trial of indomethacin is plausible. CBC: Complete blood count. *By thumbnail-squeezing method or superficial needle-scraping method.|
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| Conclusions|| |
We report five cases of EPF with Demodex overgrowth (demodicosis). These cases illustrate that the possibility of Demodex overgrowth should be considered in cases of EPF with incomplete treatment response to indomethacin and/or oral corticosteroids. Conversely, EPF should be considered in cases of demodicosis with incomplete response to antiparasitic treatments.
Financial support and sponsorship
Conflicts of interest
Dr. Chao-Kai Hsu, Prof. Chao-Chun Yang, Prof. Gwo-Shing Chen & Prof. Julia Yu-Yun Lee, editorial board members at Dermatologica Sinica, had no roles in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.
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[Figure 1], [Figure 2]