Eosinophilic pustular folliculitis (EPF) is a rare idiopathic inflammatory disease characterized by sterile pruritic follicular papules and pustules located on the face, trunk and extremitites. The pathogenesis of the EPF is stil unrevealed. Classical type of EPF is described mainly in the Japanese. Pruritic papullo-pustular lesions coalesce to form broad, annular, serpiginous, erythematous plaques. Lesions are predominantly seen on the face, dorsum and extensor surfaces of the upper extremities; rarely the palms and soles are also affected. Immunosupression-associated EPF (IS-EPF) is mostly related to HIV as well as lymphoma, leukemia, mylodysplastic syndrome, polycythemia vera and after bone marrow transplantation. In the immunosuppressed and patients with hematopoietic disorders EPF may be persistent. A rare case of eosinophilic pustular folliculitis with concomitant polycythemia vera is presented herein.
Eosinophilic pustular folliculitis (EPF) is a rare disease characterized by noninfectious eosinophilic infiltration of the hair follicle. Men in third and fourth decades constitute the majority of the patients. Pruritic papullo-pustular lesions coalesce to form broad, annular, serpiginous, erythematous plaques [1, 2]. Lesions are predominantly seen on the face, dorsum and extensor surfaces of the upper extremities; rarely the palms and soles are also affected. Involvement of the mucosae is rare in all types of EPF. The clinical course is in the form of exacerbations and remissions . EPF has four clinical subtypes including the classical type, immunosuppression-associated, infantile type and EPF associated with other reasons . EPF cases associated with pregnancy, drugs (carbamazepine, allopurinol foscarnet), infections such as dermatophytes, larva migrans, pityrosporum, retrovirus and hepatitis C have been recently reported [2, 4]. Immunosupression-associated EPF is mostly reported with HIV infection. EPF may also ocur in patients with lyphoma, leukemia, mylodysplastic syndrome, polycythemia vera (PV) and after bone marrow transplantation . PV is a disease characterized by monoclonal proliferation of bone marrow stem cells giving rise to an increase in the erythtocyte quantity and hematocrite up to 55-80%. Leucocytes and platelets are also abundant in the primary type . In 50-60% of PV essential thromocytemia and idiopathic myelofibrosis is present. Increased viscosity of the blood and platelet abnormalities may result in cardiovasular events and thrombosis. Aquagenic pruritus, purpura, urticaria and erythromelalgia frequently accompany PV [5, 6]. We decided to present a case of eosinophilic pustular folliculitis concomitant with PV since it is rare case reported in the literature.
A 45 year old male patient consulted to our clinic with pimples and one week old rush starting from the face and spreading to the trunk. He described a history of transient ishchemic attack presenting with the lisping of the tongue and paresthesia in the arm 3 weeks earlier. He didn’t have any history of drugs. There were no significant medical events in his family. Diffuse erythematous papullo-pustular lesions with patchy excoriations in his face, trunk and scalp were noticed in dermatologic inspection (Figure 1,2). Erythromelalgia accompanied lesions in the face and the neck. Erythema, edema and pustules were noted in the palmoplantar areas. Mucosae and nails were not involved. Routine blood tests showed leucocytosis and thrombocytosis. Sedimentation rate was 1 and CRP was 2.82. Peripheral smear had no findings. Anti HIVantibody was negative (< 0.05). Few fungal mycelia and spores were detected microscopically in the facial scrapings. Abdominal ultrasonograpghy showed hepatomegaly and splenomegaly. After consultation to neurology the patient was diagnosed as polycythemia vera. JAK2 (V617F) was positive in the PCR result. For the treatment of PV hydroxyurea 500 mg (2x1, tablets) was initiated and 1 unit of phlebotomy was performed. Acetylsalicylic acid 300 mg (1x1, tablets) was initiated by neurology. Pathologically interstitial inflammatory infiltrate predominated by eosinophils in the papillary dermis and around the hair follicle beneath a slightly acanthotic epidermis were observed in the biopsy (Figure 3). Histopathologic findings were compatible with EPF. Clarithromycin 500 mg (2x1, IV) fucidic acid,topical cream, eau boriquee’ %2 (2x1, solution] were initiated. There was no response to therapy after 10 days. Similarly, terbinafine treatment was ineffective. Therefore the patient was switched to methylprednisolone 40 mg/day. Two days later the dose was reduced to 16 mg/day. The treatment was stopped on the fifth day with amelioration in the lesions. Two weeks after discharge an exacerbation occured. Methylprednisolone 40 mg/day was initiated again and was stopped gradually by reducing 10 mg/week. Indometacin tratment was initiated after the remission. The patient is being followed for 1 year with methylprednisolone 8mg/day, hydroxyurea 500 mg (2x1), acetylsalicylic acid 300 mg (1x1) and phlebotomy during exacerbations of the eosinophilic lesions.
Eosinophilic pustular folliculitis (EPF) is a rare idiopathic inflammatory disease characterized by sterile pruritic follicular papules and pustules located on the face, trunk and extremitites . The pathogenesis of the EPF is stil unrevealed. Classical type of EPF is described mainly in the Japanese .
Several antigenic stimulations in EPF may provoke multifactorial immune reaction towards follicles [1, 2, 8]. Especially IS-EPF may occur after immune dysfunction or an augmentation of the immune system associated hypersensitivity towards different types of Malessezia . Detection of a fewfungal hyphae and spores may support this opinion. Fungi may also have contributed the event by superinfection after an immune dysfunction. IL-4 and IL-5 have vital roles in changes of cytokin production in the pathogenesis of the EPF. These cytokines are responsible from the proliferation and activation of intercellular adhesion molecules (ICAM1) and eosinophils. ICAM-1 might be responsible from enhanced chemotaxis of the eosinophils . Th-2 immune response impaired for the aberrant follicular antigen might be associated with the EPF pathogenesis. In the HIV associated EPF the cytokine production shifts in favor of Th-2 associateed cytokines with disease progression. During AIDS treatment Th-2 produced by IL-5 and IFN-γ, cytokines associated with eosinophilia, provides amelioration in EPF by improving cell function [1, 9]. Changes in the cytokin levels in PV might have a role in EPF progression.
IS- EPF is mostly related to HIV, whereas it can be seen in hematologic and lyphoproliferative diseases like lyphoma, leukemia and polycytemia vera. It’s observed in 25-50% of patients with eosinophilia. IS-EPF is distinguished from the classical type by pruritus and its atypical appearance [3, 8, 10]. Kimoto had reported a rush in PV patients characterized by multiple erythematous papules and infiltrative plaques with reduction and increase in pruritic symptoms. Lesions appear as pustules in HIV associated EPF. Clinical appearance of EPF associated with immunologic and hematologic anomalies is different than the classical type . Our patient manifested crusted papular and pustular erupsions in his face and neck on a background of erythromelalgia, but on normal skin in the trunk. Lesions in the soles and palms of our patient had coalesced to form bullous pustules.
Frequently serial sections must be taken to derive adequate histologic information from a biopsy in IS-EPF. Hisyopathology of EPF includes eosinophilic infiltration around and in the hair follicle, with probable follicular destruction and privacular infiltrate containing lymphoctes and neutrophils . Interstital inflammatory infiltrate predominated by eosinophils in the papillary dermis and around the hair follicle beneath a slightly acanthotic epidermis were observed in one of the biopsies taken from the face and back of our patient. In adults sebaceous glands and the outer root sheath are invaded by infiltration. Hence the disease is typically manifested on seborrheic areas, which are the face, back and the extensor surfaces of the arms . Folliculitis and seborrheic dermatitis caused by bacterial and fungal infections especially in immunocompromised patients should be kept in mind in the differential diagnosis .
Nonsteroidal anti-inflammatory drugs, specifically indometacin, is the first choice for systemic treatment. Since the pathogenesis of the Ofuji disease is not fully understood, the treatment is usually empirical. Successful treatments have been reported with oral and topical corticosteroids, dapsone, minocycline, interferon, isotretinoin, cyclosporine, colchicine, sulfamethoxazole or UVB . There are reports suggesting spontaneous, within months or years, remission . EPF might follow a serious course and persistent to therapy in the immunocompromised and patients with hematopoietic disorders . EPF did not respond to topical drugs and indometacin in our patient. He didn't approve of usage of any other drugs than low dose corticosteroids. To our knowledge, this report is the second EPF case concomittant with PV in the English literature.
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