Dermatology

Eosinophilic Pustular Folliculitis (Ofuji’s Disease)

Eosinophilic Pustular Folliculitis (Ofuji’s disease)

Are You Confident of the Diagnosis?

Eosonophic postular folliculitis is a rare condition that affects five times as many men as women. Although the average age of onset is 30, the condition can be seen at any age, including infants.

Characteristic findings on physical examination

This condition presents as recurrent eruptive crops of very pruritic follicular papulopustules. Lesions are most commonly seen on the scalp, face, back, and extensor arms. Lesions will often have a base of erythema, and can appear with annular and or serpiginous patterns. Individual lesions usually resolve spontaneously in 7-10 days, and recur every 3-4 weeks.

Expected results of diagnostic studies

Histology of a biopsied lesion will show spongiosis of involved follicles with exocytosis of lymphocytes and eosinophils extending from the sebaceous duct up to and including the infundibular zone. Infundibular eosinophilic pustules can be seen. There may also be a surrounding infiltrate of lymphocytes and eosinophils. Peripheral eosinophilia may also be present.

Diagnosis confirmation

The differential diagnosis includes all other types of folliculitis, including bacterial, fungal (with Pityrosporum being the most common), and viral (Herpes simplex) folliculitis. Additionally, in infants, the differential diagnosis also includes erythema toxicum neonatorum, transient neonatal pustular melanosis, and acropustulosis of infancy.

Who is at Risk for Developing this Disease?

This condition has been most commonly reported in those of Japanese descent.

What is the Cause of the Disease?

Etiology

Pathophysiology

The etiology and pathophysiology are unknown. However, some authors believe that this condition and infantile acropustulosis are the same entity. Other authors believe that both conditions may be reactions to aborted cases of scabies, although there is no good evidence to support this.

Systemic Implications and Complications

Other than the associated eosinophila and the severe pruritus, there are no other systemic implications or complications in children. There have been numerous case reports of eosinophilic pustular folliculitis in patients with HIV/AIDS, but this association has not been reported in children. The Eosinophilic pustular folliculitis seen in patients with HIV/AIDS is usually extremely pruritic and presents late in the course of the HIV infection.

Treatment Options

Treatment options are directed either against the pruritus or against the skin lesions themselves.

Treatments for Pruritus

Oral antihistamines

Mid-potency topical steroids

Treatments for Skin Lesions

First-line treatment

Narrowband ultraviolet (UV) B 3 times per week used along with Indomethacin 50mg orally daily.

Second-line treatments

  • Oral minocycline 100mg PO BID for 1 month, then 100mg PO QD for 1 month, then discontinue

  • Oral colchicine 0.6mg PO BID

  • Oral Dapsone 100mg QD for 2 weeks

  • Oral prednisone given as 1mg/kg/day tapered off over 2 -3 weeks

Optimal Therapeutic Approach for this Disease

The therapeutic ladder is determined based on the clinical extent of the skin lesions, as well as on the severity of the associated pruritus. For relatively localized disease, such as a patient with lesions limited to the scalp, it would be reasonable and appropriate to start out with oral antihistamines such as hydroxizine taken at up to 2mg/kg/day in a dose divided twice to four times daily, along with topical steroids designed for the scalp. A mid- to high-potency topical steroid, such as mometasone ointment, betamethasone valerate foam, or clobetasol solution should be used for 2-3 weeks along with the oral antihistamine.

If this treatment regimen is not effective after several weeks, I would recommend switching to the first-line systemic therapy listed above, followed by the oral second line-therapies listed above.

Based on a trial showing successful responses in 30-40% of patients, Narrowband UVB done 2-3 times per week used along with oral indomethacin taken as 50mg orally daily is considered first-line therapy. If that combination therapy is not effective within 4-6 weeks, the patient should be switched over to minocycline. Each second-line oral agent should be given a 4-6 week trial. Based on the agents’ respective side effect profiles, I would try colchicine after minocycline, followed by dapsone, and then finally a 3-4 week course of oral prednisone.

All of these second-line agents have very well described side effects profiles. Although minocycline is overall a very safe drug, it has rarely been reported to cause a lupus-like syndrome and autoimmune hepatitis. Therefore, it would be contraindicated in a patient with either lupus or liver disease. Colchicine’s main side effect is diarhhea, and therefore would not be a good choice for patients with GI disease also producing this symptom. Dapsone is often a more effective treatment than colchicine, but has much more significant side effects. These include anemia, methemoglobinemia and peripheral neuropathy. Close laboratory monitoring for the anemia and methhemoglobinemia is required for any patient treated with oral dapsone.

Patient Management

Each of the above systemic therapies requires different follow-up. If minocycline is used for more than several months, antinuclear antibody (ANA) and liver function tests should be checked, as minocycline has been associated with both lupus and autoimmune hepatitis. Colchine requires no lab monitoring, but it is important to monitor the extent of dose dependent diarrhea that each individual gets.

As dapsone can cause both anemia and methemoglobinemia, both need to be monitored closely. A complete blood count should be checked several times during the first month after initiation of therapy, with a blood gas checked to evaluate for methemoglobinemia should any symptoms of shortness of breath develop.

Oral prednisone is fairly safe and well tolerated in the short term of 30 days or less. However, should long-term prednisone be required, extensive monitoring of a myriad of side effects is required which is well described elsewhere.

Unusual Clinical Scenarios to Consider in Patient Management

The most concerning unusual clinical scenario would be the presentation of this condition in a very young and or premature infant with either a febrile illness or other severe medical issues. This is possible, as eosinophilic pustular folliculitis has been reported to occur as early as the first few days of life, having even rarely been reported to be present at birth. In this setting, it would be possible for the lesions to be a manifestation of an underlying infection, such as candidiasis, Staphyloccus, or herpes, or of some other underlying condition such as histiocytosis.

Although the work=up in this scenario would still include a skin biopsy, it would also include multiple cultures of the skin lesions for fungus, bacteria and herpes viruses, as well as other cultures such as those from blood, urine and cerebrospinal fluid where appropriate. The other possibility, of course, is that the skin lesions are totally unrelated to the baby’s underlying medical issues.

What is the Evidence?

Ota, T, Hata, Y, Tanikawa, A, Amagai, M, Tanaka, M, Nishikawa, T. "Eosinophilic pustular folliculitis: Indomethacin as a first choice of treatment". Clin Exp Dermatol. vol. 26. 2001. pp. 179-81.

(A relatively recent article on this condition describing successful treatment with the combination of narrowband UVB and indomethacin.)

Ofuji, S. "Eosinophilic pustular folliculitis". Dermatologica.. vol. 174. 1987. pp. 3-56.

(This is an historic review of eosinophilic pustular folliculitis, also known as Ofuji’s disease, by the author for whom the disease would later be named.)

Vicente, J, Espana, A, Idoate, M. "Are eosinophilic pustular folliculitis of infancy and infantile acropustulosis the same entity?". Br J Dermatol. vol. 135. 1996. pp. 807-9.

(These authors argue that infantile acropustulosis and eosinophilic pustular folliculitis of infancy are the same condition. They base their argument on the facts that both conditions present as sterile pustules during infancy. However, one point going against them is that lesions of acropustulosis are filled with predominantly neutrophils as opposed to eosinophils.)

Buckley, DA, Munn, SE, Higgins, EM. "Neonatal eosinophilic pustular folliculitis". Clin Exp Dermatol. vol. 26. 2001. pp. 251-55.

(A case report of this condition occurringat a very young age.)

Giard, F, Marcoux, D, McCuaig, C. "Eosinophilic pustular folliculitis (Ofuji’s disease) in childhood: a review of four cases". Pediatr Dermatol. vol. 8. 1991. pp. 189-93.

(Another historic review of four cases of eosinophilic Pustular polliculitis in childhood.)

Fearfield, LA, Rowe, A, Francis, N. "Itchy folliculitis and human immunodeficiency virus infection: Clinicopathological and immunological features, pathogenesis and treatment". Br J Derm. vol. 141. 1999. pp. 3-11.

(A review article discussing eosionphilic folliculitis in HIV. The authors state that this is a disease of the sebaceous glands where eosinophils and CD8+ lymphocytes predominant. They suggest/ propose that HIV-associated eosinophilic folliculitis is an autoimmune disease with the sebocyte or a component of the sebocyte as the autoantigen.)
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