Pustulose sterile eosinophils L73.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.10.2021

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Synonym(s)

Eosinophilic folliculitis; eosinophilic folliculitis (e); eosinophilic pustular folliculitis (e); Eosinophilic pustulous folliculitis of childhood; Eosinophilic sterile pustulose; Eosinophil pustular folliculitis; Eosinophil Pustulose; Folliculitis eosinophil; Folliculitis eosinophilic pustular; Ofuji`s disease; Ofuji Syndrome; Sterile eosinophilic pustulose

History
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Ise and Ofuji, 1965

Definition
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Rare, self-limiting, relapsing, very itchy, pustular dermatitis (blood eosinophilia; no further systemic involvement) with intraepidermal eosinophilic pustules occurring in adults, newborns and children.

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Classification
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  • Classical eosinophils pustular folliculitis (Ofuji folliculitis)
  • Immunodeficiency-associated eosinophils pustular folliculitis
  • Infantile eosinophils pustular folliculitis

Occurrence/Epidemiology
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Rarely. Mainly appearing in Japan, in the last few years occasionally also in Europe.

Etiopathogenesis
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Unknown.

In the infantile form, previous infections(scabies, larva migrans) and bacterial infections (Pseudomonas) have been described.

In the adult form, patients with immunodeficiency (stem cell transplants or haematological systemic diseases (T- and B-cell lymphomas) have been described.

About 10-20% of cases are associated with HIV infection.

Eosinophilia-inducing drugs can also trigger the clinical picture. The triggering by carbamazepine and allopurinol is described (Mizoguchi S et al. 1998).

Manifestation
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Infantile form: Sporadic occurrence in children. It mainly affects infants (the prevalences are given differently: w>m; same distribution) aged 5-10 months. Rarely occurring in newborns. Occasional neonatal cases also exist.

Adult form: The disease mainly affects young men (3rd-4th decade of life). As in the infantile form, men are affected 4-5 times more frequently than women.

Localization
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Infantile form: Capillitium, face, here mainly forehead area.

Adult form: trunk and extremities.

Clinical features
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Adult form: often associated with immunodeficiencies. Disseminated, very itchy and reddened papules and plaques with development of sterile (follicular) pustules. Confluence to larger foci is possible; also anular and polycyclic foci with central regression and peripheral progression may occur. Clinical pictures as in erythema exsudativum multiforme are possible, especially in adults. Healing often occurs with hyperpigmentation. At the capillitium a circumscribed atrophying alopecia of the pseudopélade type is possible.

In the infantile form, highly itchy follicular vesicles and pustules of 0.2-0.3 cm in diameter are observed. Lesions are not anular or serpiginous. No association with HIV or other immunodeficiencies. The children are extremely irritable during a relapse activity.

Laboratory
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Increases eosinophilia in peripheral blood (in about 35% of cases). Increase in total IgE. Suppressor cells in peripheral blood are reduced. Negative reaction to recall antigens.

Histology
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Intraepidermal, also subcorneal pustules with abundant eosinophilic leukocytes in the pustules. Inflammatory, eosinophilic infiltrate also perifollicular and around sweat glands. Spongiosis of the outer hair root sheath with dense eosinophilic infiltrate.

Pattern: Superficial interstitial eosinophilic spongiotic dermatitis with folliculitis

Diagnosis
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Clinic, histology, laboratory (eosinophilia).

Differential diagnosis
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In differential diagnosis, there are significant differences between the adult and infantile forms.

  • Infantile form:
    • Erythema neonatorum: little itching, recess of palmae and plantae, no eosinophilia.
    • Melanosis, transitory neonatal pustular: already manifest at birth. Preferably occurring in coloured people. Typical infestation of Palmae and Plantae, also post-inflammatory brown spots.
    • Incontinentia pigmenti, type Bloch-Sulzberger: mainly occurring in girls; occurring in utero or directly after birth. Efflorescences in stripes or girlades, sometimes also in a whirling arrangement (Blaschko lines). After healing of the acute symptoms typical pigmentation disturbances in splash or stripe-like arrangement. Accompanying symptoms such as nail dystrophy, tooth hypoplasia, hypodontia, etc.; in the laboratory high blood and tissue eosinophilia is present.
    • Infantile scabies: Face, head, neck and often Palmae and Plantae are affected. Efflorescences in children are often very succulent. Excruciating itching persists. Blisters and pustules may occur. These may be clinically formative.
    • Acute Langerhans cell histiocytosis (type Abt-Letterer-Siwe disease): rare; severe consumptive systemic disease; disseminated small, flat, yellow-brownish, scaly or crusty papules, erosions and ulcers. Seborrheic zones are particularly affected. Pustules are rather rare.
    • Impetigo neonatorum: Frequently beginning in the face; vesicles the size of a glass pinhead; pustules; itching; prompt response to antibiotics.
    • Miliaria pustulosa: densely packed pustules the size of a pinhead to small lentils; occurs more frequently in the intertriginous regions.
  • Adult form:

Therapy
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Topical (and/or systemic) glucocorticoids and/or UV-B therapy over a period of several weeks are considered the treatment of choice.

Supplementary for pruritus: 1st and 2nd generation antihistamines.

Among the topical drugs also calcineurin inhibitors, e.g. tacrolimus, are applied.

In eosinophilic folliculitis of the immunosuppressed the most important treatment is cART (combined antiretroviral therapy).

External therapy
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Drying measures with lotio alba and addition of 3-5% clioquinol R050, zinc oxide shaking mixture R292, if necessary topical glucocorticoids such as betamethasone valerate or hydrocortisone in lotio or cream (e.g. Betnesol-V, hydrogals, R120 R030 R029 R259 ).

Radiation therapy
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Successful therapy attempts with PUVA therapy are described.

Internal therapy
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Indomethacin initial 75 mg/day, later reduction to maintenance dose depending on the clinic. The recurrence rate after discontinuation of indomethacin is 80%, therefore long-term maintenance therapy with 50 mg/day.

Experimental use of sulphones such as DADPS (e.g. Dapson Fatol) 100-150 mg/day. Alternatively long-term glucocorticoid medication with the lowest possible maintenance dose (below the Cushing's threshold) or initial combination therapy DADPS with steroid.

Symptomatic therapy of itching with H1 antagonists, e.g. desloratadine (Aerius) 1 tbl/day p.o. or levocetirizine (Xusal) 1 tbl/day p.o.

In addition, therapies with ciclosporin, permethrin or metronidazole have been described in individual cases.

Progression/forecast
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Chronic recurrent course. Spontaneous healing is possible. Healing under hyperpigmentation and rarely under scarring.

Note(s)
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It is currently unclear whether the adult and infantile forms represent variants of the same clinical picture or whether they are different entities. In contrast to classical eosinophilic pustular folliculitis, indomethacin is less effective in HIV-associated variants. Alternatively, therapy successes with metronidazole, itraconazole, topical permethrin and light therapies have been reported.

Literature
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  1. Breit R et al (1991) Classical form of eosinophilic pustulous folliculitis - successful therapy with PUVA. dermatologist 42: 247-250
  2. Ellis E et al (2004) Eosinophilic pustular folliculitis: a comprehensive review of treatment options. At J Clin Dermatol 5: 189-197
  3. Gul U et al (2007) Eosinophilic pustular folliculitis: the first case associated with hepatitis C virus. J Dermatol 34: 397-399
  4. Ise S, Ofuji S (1965) Subcorneal pustular dermatosis. A follicular variant?. Arch Dermatol 92: 169-171
  5. Katoh M et al (2013) Eosinophilic pustular folliculitis: a review of the Japanese published works. J Dermatol 40:15-20.
  6. Kim BW et al (2014) Eosinophilic pustular folliculitis: association with long-term immunosuppressant use in a solid organ transplant recipient. Ann Dermatol 26:520-521
  7. Mizoguchi S et al (1998) Eosinophilic pustular folliculitis induced by carbamazepine.
  8. J Am Acad Dermatol 38:641-643.
  9. Ofuji S et al. (1970) Eosinophilic pustular folliculitis. Acta Derm Venereol 50: 195-203
  10. Ono K et al (2014) Eosinophilic Pustular Folliculitis Clinically Presenting as Orofacial Granuloma: Successful Treatment with Indomethacin, but not Ibuprofen. Acta Derm Venereol doi: 10.2340/00015555-1926
  11. Yoneda K et al (2007) Eosinophilic pustular folliculitis associated with pulmonary eosinophilia. J Eur Acad Dermatol Venereol 21: 1122-1124
  12. Yamamoto Y et al (2014) Clinical Epidemiology of Eosinophilic Pustular Folliculitis: Results from a Nationwide Survey in Japan. Dermatology 230: 87-92.
  13. Zitelli K et al (2014) Eosinophilic folliculitis occurring after stem cell transplant for acute lymphoblastic leukemia: a case report and review. Int J Dermatol doi: 10.1111/ijd.12521.

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Last updated on: 07.10.2021