Ofuji’s disease – A diagnosis to consider in white patients

ARTICLE

Auteur(s) : Ana Nogueira¹, Ana Filipa Duarte^1,2, Sofia Magina^1,2, Áurea Canelhas³, Carlos Resende¹, Filomena Azevedo¹

¹Department of Dermatology and Venereology, Hospital S. João, EPE, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal
²Faculty of Medicine, Oporto University, Hospital S. João, EPE, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal
³Department of Pathology, Hospital S. João, EPE, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal

Classic eosinophilic pustular folliculitis (EPF), also known as Ofuji’s disease, is a rare inflammatory disease that presents mainly on seborrheic areas with erythematous papulopustular lesions in annular configurations. It mostly affects male Asian patients in the third and fourth decades. Most cases are not associated with systemic disorders apart from peripheral blood eosinophilia. Histology shows an infiltrate of eosinophils and lymphocytes around the pilosebaceus unit [1].

A previous healthy eighteen year old Portuguese Caucasian woman presented with a six month history of papulo-pustules on the cheeks and forehead, evoking an annular configuration in the jaw. She also displayed generalized annular plaques, mainly on the trunk, evolving for the past four months. These lesions had grown centrifugally, had an infiltrated violaceus border (figures 1A, B) and were intensely pruritic. She underwent treatment with topical and oral corticosteroids with no benefit. Afterwards, she was treated with oral terbinafine, with no response. A hemogram revealed a normal leukocyte count with relatively high eosinophilia (23%, 2.3 × 10⁹/L). The biochemistry panel was normal. The patient denied any sexual contacts in the past or drug abuse. Serologies for HIV and hepatitis viruses were negative. An immunological study was normal. Parasitological examination of the stools was negative. A skin biopsy revealed eosinophilic infiltration involving the hair follicles (figures 1C,D). Mycologic scraps were negative. The diagnosis of classic EPF was suggested; she was started on oral indomethacin 50 mg/day, with clearing of the lesions and resolution of eosinophilia within two weeks (figure 1E). She underwent treatment for three months, but EPF relapsed when she decided to stop indomethacin. No association with systemic disease was found meanwhile.

Classic EPF usually affects the face, dorsum and extensor surfaces of the upper extremities and, rarely, the palms and soles. In most patients, lesions first appear on the face [1, 2]. The diagnosis is made when typical skin lesions have a compatible histology. As this is a folliculocentric process, serial sections may be needed to find the affected follicle [1]. Peripheral blood eosinophilia supports the diagnosis. Differentials in adults include infectious folliculitis, dermatophytia, papular urticaria, graft-versus-host disease, seborrheic dermatitis, acne and rosacea [1]. The natural history is of chronic recurrent lesions, with occasional residual skin hyperpigmentation [3]. The pathogenesis is unclear, although some causative factors have been reported, such as infections, infestations, medications or silicone tissue injections [1, 4]. Immune-suppression associated EPF occurs with HIV infection or hematological diseases and is clinically distinct, although identical histologically [1]. Some authors claim that EPF is an inflammatory response to eliminate a yet unidentified organism, whereas others hypothesise it is essentially a Th2 cytokine imbalance [1].

An etiological treatment is unavailable, justifying the wide range of therapies with variable success [2]. For localised disease, topical corticosteroids seem adequate. With generalised disease, most of the literature favours indomethacin, 50 to 75 mg/day. As it inhibits ciclooxygenase activity, it probably prevents the formation of arachidonic acid metabolites involved in the pathogenesis of EPF [2, 5]. This drug is generally well tolerated and the response takes 1 to 2 weeks, with concomitant resolution of the eosinophilia. Therapy should be continued for up to one year with a partial response, increasing the dose to 150 mg/day or associating other therapies, such as low dose oral corticosteroids or UVB phototherapy [5, 6]. However, there may be a relapse upon indomethacin interruption, so it seems advisable to gradually taper it to the lowest possible dosage, averaging 50 mg/day [6].

We emphasise the unusual characteristics of this case, namely the patient’s young age, female gender and Caucasian race, highlighting the need for a greater awareness of EPF in the western world.

Acknowledgements

References

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