Letters to the editor
712
Eosinophilic pustular folliculitis in a patient with mycosis fungoides Dear Editor Eosinophilic pustular folliculitis (EPF), or Ofuji’s disease, is a rare dermatosis clinically characterized by recurrent and pruritic eruptions consisting of follicular papules and sterile pustules associated with eosinophilia.1 Histologically, eosinophils and lymphocytes surround and infiltrate the follicle, forming intrafollicular eosinophilic micropustules. The aetiology of EPF is still uncertain, but associations with acquired immunodeficiency syndrome (AIDS),2 cutaneous T-cell lymphoma (CTCL), anaplastic large cell lymphoma (ALCL),3,4 acute myelogenous leukaemia (AML), B-cell chronic lymphatic leukaemia and acute lymphoblastic leukaemia (ALL)3 have been reported. In addition, EPF has been shown to be associated with bone marrow transplantation (BMT).3 Here, we describe a case of EPF in a patient with mycosis fungoides (MF) who had received an unrelated BMT (uBMT). A 36-year-old female with MF (T3N2M0B0, stage IIb), which developed around 16 years previously, came to our hospital with pruritic eruptions on her face. Twenty-one months before, she had been treated with uBMT for skin tumours associated with MF, which had appeared 4 years before, resulting in partial remission. The eruptions first developed around the eyebrows, and gradually spread over her face in 7 days. Clinically, firm reddish papules of up to 5 mm in diameter were scattered over her face (Fig. 1). On her left popliteal fossa and right shoulder, dark red tumours of up to 4 mm in diameter were noted, which had been histologically diagnosed as recurrent lesions of MF. Laboratory investigations showed increased eosinophils (480 cells/L, 10.7% of total leucocytes). A skin biopsy from a papule on her right submandibular facial eruption showed dense infiltration of eosinophils, lymphocytes and neutrophils around hair follicles, and many eosinophils were infiltrated in the hair follicles (Fig. 2a,b). Mucin was also detected within the follicles (Fig. 2c). Immunohistochemically, atypical mononuclear cells positive for CD3, CD4 and CD5 and negative for CD7 were also observed (Fig. 2d,e). From the clinical and histological findings, the eruption was diagnosed as EPF associated with MF. Topical diflucortolone valerate showed no effect, and oral indomethacin (100 mg/day) was started. New papules stopped appearing and the pruritus disappeared within 7 days after the treatment, and peripheral eosinophils were decreased (297 cells/L, 6.6% of total leucocytes) after 4 weeks. However, after 6 weeks, the treatment was discontinued due to adverse drug eruptions, and the skin eruptions recurred. To the best of our knowledge, this is the first case of EPF that developed in a patient with MF. Interestingly, immunohis-
JEADV 2016, 30, 683–719
Figure 1 Clinical features of the eruptions on the face. Erythematous follicular papules coalescing into erythema are seen.
tochemical studies have revealed that MF-associated atypical lymphoma cells are also involved in regional hair follicles of EPF. In addition, deposits of mucin have also been observed within the hair follicles, as found in follicular mucinosis, which is known to develop in patients with MF.4 These findings suggest that MF is involved in the development of EPF. In addition, uBMT therapy may be associated with the development of EPF, since EPF that developed after BMT has been reported.4 However, all previous cases were patients who had received autoBMT but not uBMT; therefore, the aetiological role of uBMT in the development of EPF is still undetermined. In summary, here we reported a case of EPF that was associated with MF, in which oral indomethacin was effective, as is found in classical cases of the disease.
Funding sources None. S. Takashima,1 W. Nishie,1,* Y. Morita,1 R. Osawa,1 H. Iwata,1 Y. Fujita,1 K. Fujimoto,2 H. Shimizu1 Departments of 1Dermatology, 2Haematology, Hokkaido University Graduate School of Medicine, Sapporo, Japan *Correspondence: W. Nishie, E-mail: [email protected]
© 2015 European Academy of Dermatology and Venereology
Letters to the editor
(a)
713
(b)
(d)
(c)
(e)
Figure 2 Histopathologic findings of the skin biopsy specimen. Eosinophilic infiltration extends into the hair follicles (a, b) [hematoxylin-eosin staining: (a) 9100; (b) 9400]. Mucin was also detected within the follicular epidermis (c) (Alcian-blue staining: 9400). Atypical mononuclear cells infiltrating in the follicular epithelium are CD4-positive (d) and CD7-negative (e) [hematoxylin-eosin staining: (d, e) 9100].
References 1 Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987; 174: 53–56. 2 Rosenthal D, LeBoit PE, Klumpp L et al. Human immunodeficiency virus-associated eosinophilic folliculitis. A unique dermatosis associated with advanced human immunodeficiency virus infection. Arch Dermatol 1991; 127: 206–209. 3 Ota M, Shimizu T, Hashino S et al. Eosinophilic folliculitis in a patient after allogeneic bone marrow transplantation: case report and review of the literature. Am J Hematol 2004; 76: 295–296. 4 Fujiyama T, Tokura Y. Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis. J Dermatol 2013; 40: 419–423. DOI: 10.1111/jdv.13009
JEADV 2016, 30, 683–719
Severe diclofenac photoallergy in a patient treated with vemurafenib Vemurafenib has significantly improved overall survival and disease-free survival of patients with BRAF V600 mutation metastatic melanoma.1 However, this small-molecule inhibitor of BRAF is associated with skin toxicity. The most frequent adverse skin events include UVA photosensitivity, keratoacanthomas, skin papillomas and hand–foot skin reaction.2,3 A 61-year-old woman, skin phototype II, who had presented 4 years previously with a superficial spreading melanoma with a Breslow thickness of 1.6 mm on her back, was
© 2015 European Academy of Dermatology and Venereology
712
Eosinophilic pustular folliculitis in a patient with mycosis fungoides Dear Editor Eosinophilic pustular folliculitis (EPF), or Ofuji’s disease, is a rare dermatosis clinically characterized by recurrent and pruritic eruptions consisting of follicular papules and sterile pustules associated with eosinophilia.1 Histologically, eosinophils and lymphocytes surround and infiltrate the follicle, forming intrafollicular eosinophilic micropustules. The aetiology of EPF is still uncertain, but associations with acquired immunodeficiency syndrome (AIDS),2 cutaneous T-cell lymphoma (CTCL), anaplastic large cell lymphoma (ALCL),3,4 acute myelogenous leukaemia (AML), B-cell chronic lymphatic leukaemia and acute lymphoblastic leukaemia (ALL)3 have been reported. In addition, EPF has been shown to be associated with bone marrow transplantation (BMT).3 Here, we describe a case of EPF in a patient with mycosis fungoides (MF) who had received an unrelated BMT (uBMT). A 36-year-old female with MF (T3N2M0B0, stage IIb), which developed around 16 years previously, came to our hospital with pruritic eruptions on her face. Twenty-one months before, she had been treated with uBMT for skin tumours associated with MF, which had appeared 4 years before, resulting in partial remission. The eruptions first developed around the eyebrows, and gradually spread over her face in 7 days. Clinically, firm reddish papules of up to 5 mm in diameter were scattered over her face (Fig. 1). On her left popliteal fossa and right shoulder, dark red tumours of up to 4 mm in diameter were noted, which had been histologically diagnosed as recurrent lesions of MF. Laboratory investigations showed increased eosinophils (480 cells/L, 10.7% of total leucocytes). A skin biopsy from a papule on her right submandibular facial eruption showed dense infiltration of eosinophils, lymphocytes and neutrophils around hair follicles, and many eosinophils were infiltrated in the hair follicles (Fig. 2a,b). Mucin was also detected within the follicles (Fig. 2c). Immunohistochemically, atypical mononuclear cells positive for CD3, CD4 and CD5 and negative for CD7 were also observed (Fig. 2d,e). From the clinical and histological findings, the eruption was diagnosed as EPF associated with MF. Topical diflucortolone valerate showed no effect, and oral indomethacin (100 mg/day) was started. New papules stopped appearing and the pruritus disappeared within 7 days after the treatment, and peripheral eosinophils were decreased (297 cells/L, 6.6% of total leucocytes) after 4 weeks. However, after 6 weeks, the treatment was discontinued due to adverse drug eruptions, and the skin eruptions recurred. To the best of our knowledge, this is the first case of EPF that developed in a patient with MF. Interestingly, immunohis-
JEADV 2016, 30, 683–719
Figure 1 Clinical features of the eruptions on the face. Erythematous follicular papules coalescing into erythema are seen.
tochemical studies have revealed that MF-associated atypical lymphoma cells are also involved in regional hair follicles of EPF. In addition, deposits of mucin have also been observed within the hair follicles, as found in follicular mucinosis, which is known to develop in patients with MF.4 These findings suggest that MF is involved in the development of EPF. In addition, uBMT therapy may be associated with the development of EPF, since EPF that developed after BMT has been reported.4 However, all previous cases were patients who had received autoBMT but not uBMT; therefore, the aetiological role of uBMT in the development of EPF is still undetermined. In summary, here we reported a case of EPF that was associated with MF, in which oral indomethacin was effective, as is found in classical cases of the disease.
Funding sources None. S. Takashima,1 W. Nishie,1,* Y. Morita,1 R. Osawa,1 H. Iwata,1 Y. Fujita,1 K. Fujimoto,2 H. Shimizu1 Departments of 1Dermatology, 2Haematology, Hokkaido University Graduate School of Medicine, Sapporo, Japan *Correspondence: W. Nishie, E-mail: [email protected]
© 2015 European Academy of Dermatology and Venereology
Letters to the editor
(a)
713
(b)
(d)
(c)
(e)
Figure 2 Histopathologic findings of the skin biopsy specimen. Eosinophilic infiltration extends into the hair follicles (a, b) [hematoxylin-eosin staining: (a) 9100; (b) 9400]. Mucin was also detected within the follicular epidermis (c) (Alcian-blue staining: 9400). Atypical mononuclear cells infiltrating in the follicular epithelium are CD4-positive (d) and CD7-negative (e) [hematoxylin-eosin staining: (d, e) 9100].
References 1 Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987; 174: 53–56. 2 Rosenthal D, LeBoit PE, Klumpp L et al. Human immunodeficiency virus-associated eosinophilic folliculitis. A unique dermatosis associated with advanced human immunodeficiency virus infection. Arch Dermatol 1991; 127: 206–209. 3 Ota M, Shimizu T, Hashino S et al. Eosinophilic folliculitis in a patient after allogeneic bone marrow transplantation: case report and review of the literature. Am J Hematol 2004; 76: 295–296. 4 Fujiyama T, Tokura Y. Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis. J Dermatol 2013; 40: 419–423. DOI: 10.1111/jdv.13009
JEADV 2016, 30, 683–719
Severe diclofenac photoallergy in a patient treated with vemurafenib Vemurafenib has significantly improved overall survival and disease-free survival of patients with BRAF V600 mutation metastatic melanoma.1 However, this small-molecule inhibitor of BRAF is associated with skin toxicity. The most frequent adverse skin events include UVA photosensitivity, keratoacanthomas, skin papillomas and hand–foot skin reaction.2,3 A 61-year-old woman, skin phototype II, who had presented 4 years previously with a superficial spreading melanoma with a Breslow thickness of 1.6 mm on her back, was
© 2015 European Academy of Dermatology and Venereology
