Eosinophilic pustular folliculitis: Six decades of progress

By Warren R. Heymann, MD, FAAD, March 1, 2026

Vol. 8, No. 3

Eosinophilic pustular folliculitis (EPF) is a quizzical disorder. In 1965, Ise and Ofuji described the case of a 42-year-old woman who “repeatedly developed crops of small pustules on the face, the trunk, and the upper arms without any general symptoms. Histological examinations revealed subcorneal pustules [composed of neutrophils and eosinophils] in the upper parts of the hair follicles.” Although the authors reported this case as a follicular variant of subcorneal pustulosis(1), in 1970, Ofuji et al. reported three similar cases with “the repeated appearance of crops of pruritic follicular sterile papulopustules in fairly well defined areas of the face, trunk, and extremities, and not on the hands and feet. They extended centrifugally (i.e., fresh eruptions appeared at the periphery as old ones faded in the center). When plaques reached a certain size, they subsided leaving pigmentation. Histologic examination revealed vesicles in the outer root sheath containing many eosinophils and extending from the subcorneal portion of the follicular ostium to the level of the entry of the sebaceous gland, occasionally reaching the gland itself.” The authors proposed the term EPF (2), which now has the eponym Ofuji disease (OD). (Dr. Shigeo Ofuji also identified the papuloerythroderma that bears his name.) This commentary will focus on recent literature on EPF/OD.

Subsequent studies have demonstrated an eosinophilic pustular eruption of the palms and soles that may be present in approximately 18% of EPF [there are no hair follicles on the palms and soles], leading to a debate as to whether EPF should be called eosinophilic pustulosis. The counterargument was that the name should remain EPF, using pityriasis rubra pilaris as an analogy, where the word pilaris remains, even though patients have a keratoderma of the palms and soles. The use of the term OD (or Ofuji syndrome) avoids any controversy. (3)

Currently, three distinct subtypes of EPF are recognized: 1) the classical type seen in adults as detailed initially by Ofuji et al. 2) EPF of infancy (EPFI); and 3) immunosuppression-associated EPF (IS-EPF). A pruritic, sterile pustular eruption and histological eosinophilic infiltrates characterize all three variants. Despite the similarities, prognoses and treatment approaches vary with each subtype.(4)

EPFI usually develops early in life, with about 70% having an onset before six months of life and only 5% having their first onset 14 months after birth. The sterile pustules are mainly seen on the scalp and forehead. EPFI is benign and self-limiting. (5)

IS-EPF can be subdivided into HIV-associated (IS/HIV) and non-HIV-associated (IS/non-HIV), with the latter associated with hematologic malignancy, particularly after hematopoietic stem cell transplantation. IS-EPF may also be associated with solid organ immunosuppression, such as in the case of a 69-year-old cardiac transplant recipient treated with sirolimus. (6) Within the realm of immunodysregulation, EPF has been reported in patients with systemic lupus erythematosus, hepatitis C, asthma, and pregnancy. (7) According to Chen et al., “Unlike classic EPF, the lesions observed in IS-EPF are less strongly associated with the formation of pustules and do not show the same predilection for the face, as they tend to affect the upper body and extremities. Moreover, IS-EPF responds more favorably to topical or systemic steroids compared to classic EPF suggesting different pathogenesis.” (6)

The pathophysiology of EPF is incompletely understood, but the sebaceous gland may play a crucial role in the process leading to eosinophil accumulation. EPF is a T-helper 2 (Th2) cytokine-dominant condition (e.g., IL-4, IL-5, and IL-13). Additionally, the upregulation of IL-36 cytokines is chemoattractive for neutrophils and eosinophils. OD is also mediated by prostaglandin (PG)D2, which induces the production of the chemoattractant eotaxin-3 in sebocytes via PPARγ activation, potentially explaining the eosinophilic infiltration around pilosebaceous units and the clinical response to indomethacin. (8) Allergic reactions against microorganisms, such as fungi, viruses, and bacteria, have been implicated in the pathogenesis of EPF. (9)

Therapeutically, topical steroids are used initially in all forms of EPF. The prognosis for EPFI is excellent, as the condition is self-limited. Optimally, IS-EPF should improve if the immunosuppression can be alleviated — such as in treating HIV patients with highly active antiviral therapy. Indomethacin is considered first-line treatment for classical EPF. Symptomatic IS-EPF may benefit from topical calcipotriene, topical tacrolimus, UVB phototherapy, low-dose isotretinoin, systemic corticosteroids, metronidazole, anti-infective agents (tetracycline, metronidazole, itraconazole), or tumor necrosis factor inhibitors such as infliximab. (4, 10) Recent literature has demonstrated the value of targeting the Th2 pathway using dupilumab or mepolizumab.(11, 12) Unsurprisingly, JAK inhibitors (tofacitinib, abrocitinib, and upadacitinib) are increasingly reported to be highly effective in treating recalcitrant cases of refractory, classical EPF. (13, 14, 15)

Point to Remember: Eosinophilic pustular folliculitis (EPF, aka Ofuji disease) is an enigmatic disorder with three variants: classical (adult) EPF, EPF of infancy, and immunosuppression-associated EPF. Although topical steroids and indomethacin are standard first-line therapies, increasingly, JAK inhibitors are being reported as highly effective for recalcitrant, classical adult EPF.

Our expert’s viewpoint

Viktoryia Kazlouskaya, MD, PhD, FAAD
Dermatologist and CEO, Dermatology Circle PLLC, NY

Eosinophilic folliculitis (EF) is a fascinating and somewhat peculiar condition affecting the sebaceous glands. It is classically divided into three main groups: classic Ofuji disease seen predominantly in Japan, the childhood variant, and the type seen in immunocompromised patients.

The clinical presentation of EF is highly variable. It may resemble a classic folliculitis but often appears more erythematous or “juicy.” Some cases show no obvious follicular involvement and are only diagnosed histopathologically. A common presentation includes erythematous plaques with pustules at their periphery, sometimes forming a ring-like pattern, typically accompanied by severe itching. Interestingly, although EF usually affects areas surrounding hair follicles, it can also appear on the palms — an unusual site for follicular-based diseases. (16)

A skin biopsy is often necessary to confirm the diagnosis. Histopathology reveals eosinophilic infiltration of the hair follicles and in some cases sweat glands. Flame figures may be present. (17)

The pathophysiology of EF remains poorly understood. Recently, Human Polyomavirus 6 (HPyV6) has been detected in approximately 69% of patients with EF. (18) This possible viral association may help explain why EF is frequently observed in immunocompromised and HIV-positive individuals. Although further studies are needed to confirm this hypothesis, these findings contribute to the expanding knowledge of Human Polyomaviruses. Other emerging entities, such as polyomavirus-associated pruritic rash (PVARP) linked to HPyV7 and even Kimura disease, suggest this field is rapidly evolving. (19, 20)

Another theory proposes that EF is not a single distinct disease but rather a specific inflammatory reaction pattern of the sebaceous glands triggered by various stimuli. More research is necessary to clarify these mechanisms.

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References

1. Ise S, Ofuji S. Subcorneal pustular dermatosis. A follicular variant? Arch Dermatol. 1965 Aug;92(2):169-71. PMID: 11850921.

2. Ofuji S, Ogino A, Horio T, Ohseko T, Uehara M. Eosinophilic pustular folliculitis. Acta Dermatovener (Stockholm) 1970; 50: 195-203.

3. Wolf R, Kuritzky J. Eosinophilic pustular folliculitis versus eosinophilic pustulosis. Dermatology. 1994;188(3):243. doi: 10.1159/000247151. PMID: 8186520. (This reference includes a reply by H. Aoyama and H. Tagami).

4. Dodia P, Cook C. Eosinophilic Pustular Folliculitis. 2023 Oct 28. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan–. PMID: 37983361.

5. Guo W, Qian G, Zhang C. Eosinophilic pustular folliculitis of infancy. Arch Dis Child Fetal Neonatal Ed. 2023 Jul;108(4):429. doi: 10.1136/archdischild-2022-323930. Epub 2022 Jun 21. PMID: 35728924.

6. Chen J, Beatty CJ, Falcone LM, English JC 3rd, Kazlouskaya V. Eosinophilic Pustular Folliculitis in the Setting of Solid Organ Transplant Immunosuppression. Dermatol Pract Concept. 2023 Apr 1;13(2):e2023078. doi: 10.5826/dpc.1302a78. Epub ahead of print. PMID: 37196266; PMCID: PMC10188130.

7. Cepica TB, Gupta R, Nezafati K. Novel association of eosinophilic pustular folliculitis with systemic lupus erythematosus: A case report. JAAD Case Rep. 2024 Sep 2;52:130-133. doi: 10.1016/j.jdcr.2024.08.012. PMID: 39399234; PMCID: PMC11467468.

8. Mosca S, Ottaviani M, Briganti S, Di Nardo A, Flori E. The Sebaceous Gland: A Key Player in the Balance Between Homeostasis and Inflammatory Skin Diseases. Cells. 2025 May 20;14(10):747. doi: 10.3390/cells14100747. PMID: 40422250; PMCID: PMC12109737.

9. Hasegawa A, Kobayashi N, Fukumoto T, Asada H. A case of eosinophilic pustular folliculitis with response to infliximab. J Am Acad Dermatol. 2012 Oct;67(4):e136-7. doi: 10.1016/j.jaad.2011.10.023. PMID: 22980264.

10. Hara D, Kuroda K, Mieno H, Tajima S. Treatment of eosinophilic pustular folliculitis with tacrolimus ointment. J Am Acad Dermatol. 2004 Nov;51(5 Suppl):S143-5. doi: 10.1016/j.jaad.2004.03.050. Erratum in: J Am Acad Dermatol. 2004 Dec;51(6):1040. PMID: 15577754.

11. Tan X, Nie S, Chen B, Wu Z. Successful Treatment of Eosinophilic Pustular Folliculitis With Dupilumab in a 9-Year-Old Boy. Pediatr Dermatol. 2025 Mar 6. doi: 10.1111/pde.15908. Epub ahead of print. PMID: 40045852.

12. Chersi F, Zelin E, Mazzoletti V, Caro Caposiena DR, Hasa Z, Nan K, Signoretto D, Zalaudek I, di Meo N. Mepolizumab therapy for eosinophilic pustular folliculitis (Ofuji disease). J Eur Acad Dermatol Venereol. 2024 Nov;38(11):e993-e996. doi: 10.1111/jdv.20047. Epub 2024 Apr 30. PMID: 38687252.

13. Wang QX, He HY, Niu YL, Fang S. Refractory eosinophilic pustular folliculitis treated with tofacitinib: a case series and literature review. Clin Exp Dermatol. 2025 May 23;50(6):1196-1200. doi: 10.1093/ced/llaf053. PMID: 39891551.

14. Cai L, Yan Y, Li Y, Lin J, She X, Wang X. Two cases of eosinophilic pustular folliculitis successfully treated with abrocitinib. J Dermatol. 2024 Dec;51(12):1694-1697. doi: 10.1111/1346-8138.17284. Epub 2024 May 28. PMID: 38804644.

15. Cai L, Yan Y, Li Y, Lin J, She X, Wang X. Two cases of eosinophilic pustular folliculitis successfully treated with abrocitinib. J Dermatol. 2024 Dec;51(12):1694-1697. doi: 10.1111/1346-8138.17284. Epub 2024 May 28. PMID: 38804644.

16. Katoh M, Nomura T, Miyachi Y, Kabashima K. Eosinophilic pustular folliculitis: a review of the Japanese published works. J Dermatol. 2013;40(1):15-20.

17. Fertitta L, Bodemer C, Molina T, Frassati-Biaggi A, Fraitag S, Leclerc-Mercier S. Eosinophilic Pustular Folliculitis of Infancy: A Histologic Assessment of 43 Cases. Am J Dermatopathol. 2022;44(6):395-403.

18. Hashida Y, Higuchi T, Nakajima S, Nakajima K, Ujihara T, Kabashima K, et al. Human Polyomavirus 6 Detected in Cases of Eosinophilic Pustular Folliculitis. J Infect Dis. 2021;223(10):1724-1732.

19. Ho J, Jedrych JJ, Feng H, Natalie AA, Grandinetti L, Mirvish E, et al. Human polyomavirus 7-associated pruritic rash and viremia in transplant recipients. J Infect Dis. 2015;211(10):1560-1565.

20. Yorita K, Fujii T, Nagao T, Murakami I, Hashida Y, Higuchi T, et al. Kimura disease forming a human polyomavirus 6-negative parotid gland nodule with prominent squamous metaplasia in a young female: A case report. Radiol Case Rep. 2023;18(5):1933-1938.

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