Dear editor, Ofuji disease is a rare, eosinophilic inflammatory dermatosis predominantly affecting Asian males. Classic eosinophilic pustular folliculitis (EPF) is more frequent in Japan, with a male predominance 1. This disease has rarely been reported from India due to low incidence or underdiagnosis. It is clinically characterized by recurrent, pruritic, erythematous patches consisting of follicular papules and sterile pustules with peripheral blood eosinophilia. A 35-year-old male with no known comorbidities, presented to us with complaints of high-grade fever associated with chills and malaise of 4 days duration. On the second day, he developed sudden onset pus-filled lesions on the dorsum of his hands, which progressed rapidly to involve his upper trunk, neck, face, and scalp. It was associated with reddish discoloration of the body, severe burning sensation, and mild itching. The general physical examination revealed a fever of 101.1°F tachycardia of 104 beats per minute which was regular in rate, rhythm, and volume, blood pressure of 90/60 mmHg, and a solitary mobile, skin colored, mildly tender 2×2 cm left axillary lymph node. Systemic examination was within normal limits. Dermatological examination revealed diffuse erythema affecting more than 50% of the body surface area predominantly affecting the upper half of the body. There were discrete follicular pustules in the face, scalp, upper trunk, axillae, and genital areas (Fig 1a–f). A routine blood investigation revealed mild eosinophilia (10%) with an AEC of 600. Histopathology from one of the lesions revealed mild spongiosis in the epidermis with eosinophilic infiltrates within and around the follicular units (Fig. 2a, b). The patient was diagnosed as a case of eosinophilic pustular folliculitis or Ofuji disease. He was started on capsule cyclosporine 100 mg twice daily, which led to complete clearance of the lesions in a week, after which cyclosporine was stopped (Fig. 3a–c). The patient has been on follow-up for the last 4 months with no recurrences.Figure 1: (a–f) Involvement of the face and upper trunk in the form of diffuse erythema studded with pustules. The pustules are distributed over hairy areas.Figure 2: (a) The photomicrograph shows the epidermis and deeper reticular dermis with inflammatory infiltrates around the hair follicles. (b): Higher magnification shows these inflammatory infiltrates to be eosinophils (inset) (×10, 40, H&E).Figure 3: (a–c) Complete resolution of erythema as well as pustules after 7 days of oral cyclosporine.EPF is an inflammatory dermatosis that was described for the first time in a case report by Dr Shigeo Ofuji as “a variant of superficial pustular dermatosis” in 1965 2. An additional report on three male patients was made in the year 1970, and the term “EPF” was coined 3. The etiology of EPF remains unknown. It predominantly affects the seborrheic areas, namely, the face and the extensor surfaces of the upper arms and upper back, and it has been reported primarily from northern Asians, particularly in Japanese patients 4. Clinically, it is characterized by folliculocentric papular pustules (1–2 mm in diameter) with a tendency to increase in size and merge, leading to the formation of erythematous plaques. Lesions are often pruritic, chronic, and recurrent, with centrifugal extension and central clearing. Lesions typically take 7–10 days to heal and recur every 3–4 weeks. Its histopathology is characterized by a prominent eosinophilic infiltrate in the dermis within and around the pilosebaceous units, often accompanied by eosinophilic microabscesses. Two additional variants of EPF were reported in the 1980s: immunosuppression-associated EPF and infancy-associated EPF 5. The incidence of immunosuppression-associated EPF has reduced considerably after the introduction of highly active antiretroviral therapy. However, eosinophilic folliculitis is rarely seen in immunocompetent individuals, too, as observed in our patient. Successful therapeutic results have been obtained by administering topical and systemic steroids, dapsone, isotretinoin, interferon-α, acitretin, cetirizine, clofazimine, metronidazole, minocycline, phototherapy, naproxen, and indomethacin. More recently, systemic Th1-skewing reagent interferon-γ and immunosuppressive agents, including oral cyclosporine and topical tacrolimus, have been used for the treatment of EPF. Our case responded to a short course of cyclosporine for 7 days, and there have been no recurrences since then. Acute generalized exanthematous pustulosis is a close differential diagnosis. Predominantly upper body involvement, follicular pustules, peripheral eosinophilia, and eosinophilic folliculitis, favored the diagnosis of Ofuji disease over acute generalized exanthematous pustulosis. It is rarely reported in India, and it is even rarer in immunocompetent individuals. This diagnosis must be considered in young males presenting with recurrent pustular lesions on the upper half of the body. Biopsy confirms the diagnosis and cyclosporine has been proven to be an effective drug, as seen in our case. Acknowledgements Conflicts of interest There are no conflicts of interest.
Das et al. (Fri,) studied this question.