Correspondence
a case of primary lymphocutaneous N. vinacea in an immunosuppressed patient.3 Treatment with minocycline and trimethoprim–sulfamethoxazole failed, despite documented in vitro sensitivity of the organism to both agents.3 The patient eventually required surgical excision and a combination of meropenem and trimethoprim– sulfamethoxazole to clear the infections.3 Finally, cutaneous N. vinacea was described in an immunocompetent patient, who presented with a tender, purulent, erythematous plaque on his forearm.2 After failing treatment with oral cefditoren and topical potassium permanganate, the patient improved on amoxicillin–clavulanate.2 All of the patients had a history of outdoor exposure, emphasizing that Nocardia spp. are common isolates in soil.2 These cases highlight several key points in the diagnosis and management of primary cutaneous N. vinacea infection. A high level of suspicion for atypical infections must be maintained, and the diagnostic laboratory must be informed when Nocardia is considered, as it has specific culture and identification requirements. In addition, even in cases of proven in vitro sensitivity to particular antibiotics, N. vinacea may demonstrate in vivo resistance to those antibiotics, as demonstrated above. Appropriate treatment requires close follow-up and interdisciplinary management to achieve resolution. R. E. Chikowski, A. M. Sprigle, S. M. Krishna, and J. R. Nunley Department of Dermatology, Virginia Commonwealth University, Richmond, VA, USA E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 13 December 2013
References 1 Yu X, Han F, Wu J et al. Nocardia infection in kidney transplant recipients: case report and analysis of 66 published cases. Transpl Infect Dis 2011; 13: 385–91. 2 Kim MS, Choi H, Choi KC, Shin BS. Primary cutaneous nocardiosis due to Nocardia vinacea: first case in an immunocompetent patient. Clin Exp Dermatol 2011; 36: 812–14. 3 Iwasawa MT, Togawa Y, Kamada N et al. Lymphocutaneous type of nocardiosis caused by Nocardia vinacea in a patient with polymyositis. Mycopathologia 2011; 172: 47–53. 4 Luong MS, Bret C, Godreuil S et al. Pyoderma vegetans in a renal transplant recipient: first case of human infection with Nocardia vinacea. Transplantation 2009; 87: 1898– 9. 5 Kageyama A, Yazawa K, Nishimura K, Mikami Y. Nocardia anaemiae sp. nov. isolated from an immunocompromised patient and the first isolation report of Nocardia vinacea from humans. Nihon Ishinkin Gakkai Zasshi 2005; 46: 21–6.
536
Clinical and Experimental Dermatology (2014) 39, pp533–541
Eosinophilic pustular folliculitis associated with zary syndrome Se doi: 10.1111/ced.12315 Eosinophilic pustular folliculitis (EPF) is characterized clinically by intensely pruritic follicular papules and pustules arranged in arcuate plaques. Histopathologically, a noninfectious eosinophilic infiltration of the hair follicles is a crucial finding. EPF is divided into three clinical subtypes: classic, infantile, and immunosuppression-associated. The immunosuppression-associated type is further subdivided into two types: HIV-associated and malignancyassociated. In the era of HIV, many cases of the former type have been reported, whereas cases of malignancyassociated EPF are rarely reported. We report a case of EPF associated with Sezary syndrome (SS), and discuss possible mechanisms these might have in common. A 42-year-old Japanese man presented with a 3-month history of generalized erythroderma with lymphadenopathy (Fig. 1a). Histology of a biopsy specimen showed infiltration of atypical lymphocytes into the upper dermis with epidermotropism. Tumour cells were positive for CD3, CD4 and CD5. Laboratory tests showed a leucocyte count of 8.1 9 109/L (normal range: 3.5–9.2 9 109/L) with 24% atypical lymphocytes (absolute Sezary cell count of 1944 cells/mm3). Serum lactate dehydrogenase level was 400 IU/L (normal range: 125–237 IU/L) and soluble interleukin-2 receptor level was 1535 U/mL (normal range: 167–497 U/mL). Antibody against human T-cell leukaemia virus type I gave negative results. The same rearranged bands of T cell receptor-c were detected in samples of peripheral blood and lesional skin. The patient was diagnosed with SS (T4N1M0B2; stage IVA1).1,2 Because none of standard treatments for SS such as extracorporeal photochemotherapy, interferon-a, denileukin diftitox or chlorambucil were available in our country, the patient was treated with topical steroid and narrowband ultraviolet B phototherapy. Erythroderma was improved about 1 year after the start of UV therapy. At this point, the patient developed pruritic reddish follicular papules on both cheeks (Fig. 1b). Leucocyte count was 5.5 9 109/L with 1.5% atypical lymphocytes and 9.5% eosinophils. Histopathology showed massive infiltration of eosinophils and lymphocytes around hair follicles (Fig. 2). No atypical lymphocytes were detected. The final diagnosis was EPF associated with SS. The pruritic papules responded well to the addition of oral indomethacin (75 mg per day). SS is a rare type of cutaneous T-cell lymphoma (CTCL) characterized by generalized erythroderma, lymphadenopathy, and presence of > 1000 per mm3 circulating atypical T cells, so-called Sezary cells. Infiltration of Sezary cells into the skin can clinically present as multiple papules. However, in our patient, the erythroderma had cleared by the time the pruritic papules appeared. The
ª 2014 British Association of Dermatologists
Correspondence
(a)
Figure 2 Massive infiltration of eosinophils and lymphocytes
around the hair follicles (haematoxylin and eosin, original magnification 9400).
(b)
Infiltration of M2 macrophages expressing CD163 has been reported. Interestingly, SS is also regarded as a Th2type disease, frequently accompanied by eosinophilia and high serum levels of IgE.4 We have reported previously that CCL26 expression is increased in both the sera and lesional skin of patients with CTCL. Moreover, CD163positive macrophages infiltrate into lesional skin of CTCL.5 Thus, a Th2-dominant environment could be the common underlying pathology of EPF and SS. M. Sugaya, H. Suga, T. Miyagaki, H. Fujita and S. Sato Department of Dermatology, Faculty of Medicine, University of Tokyo 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 8 December 2013
References
Figure 1 (a) Erythroderma with severe pruritus; (b) pruritic fol-
licular papules on the right cheek.
lack of atypical lymphocytes and presence of numerous eosinophils in the histology specimen, along with the good response to indomethacin, supported the diagnosis of EPF. EPF is often accompanied by peripheral blood eosinophilia and raised serum IgE levels, and a T-helper (Th)2 cytokine-dominant condition has been postulated as the underlying mechanism. CCL26, a CC chemokine, also called eotaxin-3, is reported to be important in EPF.3
ª 2014 British Association of Dermatologists
1 Vonderheid EC, Bernengo MG, Burg G et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol 2002; 46: 95–106. 2 Olsen E, Vonderheid E, Pimpinelli N et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood 2007; 110: 1713–22. 3 Yokobayashi H, Sugaya M, Miyagaki T et al. Analysis of serum chemokine levels in patients with HIV-associated eosinophilic folliculitis. J Eur Acad Dermatol Venereol 2013; 27: e212–16.
Clinical and Experimental Dermatology (2014) 39, pp533–541
537
Correspondence
4 Dummer R, Heald PW, Nestle FO et al. Sezary syndrome T-cell clones display T-helper 2 cytokines and express the accessory factor-1 (interferon-gamma receptor betachain). Blood 1996; 88: 1383–9. 5 Sugaya M, Miyagaki T, Ohmatsu H et al. Association of the numbers of CD163(+) cells in lesional skin and serum levels of soluble CD163 with disease progression of cutaneous T cell lymphoma. J Dermatol Sci 2012; 68: 45–51.
Ultrasound B-mode and elastographic findings of angiomatoid fibrous histiocytoma doi: 10.1111/ced.12314 Angiomatoid fibrous histiocytoma (AFH) is a rare softtissue tumour of low- to intermediate-grade malignancy.1 The first case of this tumour was described in 1979, and the tumour was called ‘angiomatoid malignant fibrous
(a)
(d)
(b)
(e)
(c)
(f)
Figure 1 (a) a subcutaneous nodule, 10 mm in diameter, on the scalp. (b) Ultrasonography (B-mode) showed a cystic hypoechoic area
on the scalp (arrows), while colour Doppler ultrasonography revealed hypervascularity at the periphery of the cystic area (inset). (c) Ultrasound elastography showed a butterfly-shaped area with high elasticity, appearing as red at the centre of the cystic lesion (arrowheads). (d) Haemorrhagic and cystic spaces were visible in the lesion; (e) the periphery of the lesion was partly hyalinized, forming a pseudocapsule, and the haemorrhagic cystic spaces were lined with flattened tumour cells; (f) the tumour cells with nuclear atypia were composed of spindle-shape to ovoid-shaped cells arranged in sheets, whorls and short fascicle patterns Haematoxylin and eosin; original magnification (a) 920; (b) 9100; (c) 9400.
538
Clinical and Experimental Dermatology (2014) 39, pp533–541
ª 2014 British Association of Dermatologists
a case of primary lymphocutaneous N. vinacea in an immunosuppressed patient.3 Treatment with minocycline and trimethoprim–sulfamethoxazole failed, despite documented in vitro sensitivity of the organism to both agents.3 The patient eventually required surgical excision and a combination of meropenem and trimethoprim– sulfamethoxazole to clear the infections.3 Finally, cutaneous N. vinacea was described in an immunocompetent patient, who presented with a tender, purulent, erythematous plaque on his forearm.2 After failing treatment with oral cefditoren and topical potassium permanganate, the patient improved on amoxicillin–clavulanate.2 All of the patients had a history of outdoor exposure, emphasizing that Nocardia spp. are common isolates in soil.2 These cases highlight several key points in the diagnosis and management of primary cutaneous N. vinacea infection. A high level of suspicion for atypical infections must be maintained, and the diagnostic laboratory must be informed when Nocardia is considered, as it has specific culture and identification requirements. In addition, even in cases of proven in vitro sensitivity to particular antibiotics, N. vinacea may demonstrate in vivo resistance to those antibiotics, as demonstrated above. Appropriate treatment requires close follow-up and interdisciplinary management to achieve resolution. R. E. Chikowski, A. M. Sprigle, S. M. Krishna, and J. R. Nunley Department of Dermatology, Virginia Commonwealth University, Richmond, VA, USA E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 13 December 2013
References 1 Yu X, Han F, Wu J et al. Nocardia infection in kidney transplant recipients: case report and analysis of 66 published cases. Transpl Infect Dis 2011; 13: 385–91. 2 Kim MS, Choi H, Choi KC, Shin BS. Primary cutaneous nocardiosis due to Nocardia vinacea: first case in an immunocompetent patient. Clin Exp Dermatol 2011; 36: 812–14. 3 Iwasawa MT, Togawa Y, Kamada N et al. Lymphocutaneous type of nocardiosis caused by Nocardia vinacea in a patient with polymyositis. Mycopathologia 2011; 172: 47–53. 4 Luong MS, Bret C, Godreuil S et al. Pyoderma vegetans in a renal transplant recipient: first case of human infection with Nocardia vinacea. Transplantation 2009; 87: 1898– 9. 5 Kageyama A, Yazawa K, Nishimura K, Mikami Y. Nocardia anaemiae sp. nov. isolated from an immunocompromised patient and the first isolation report of Nocardia vinacea from humans. Nihon Ishinkin Gakkai Zasshi 2005; 46: 21–6.
536
Clinical and Experimental Dermatology (2014) 39, pp533–541
Eosinophilic pustular folliculitis associated with zary syndrome Se doi: 10.1111/ced.12315 Eosinophilic pustular folliculitis (EPF) is characterized clinically by intensely pruritic follicular papules and pustules arranged in arcuate plaques. Histopathologically, a noninfectious eosinophilic infiltration of the hair follicles is a crucial finding. EPF is divided into three clinical subtypes: classic, infantile, and immunosuppression-associated. The immunosuppression-associated type is further subdivided into two types: HIV-associated and malignancyassociated. In the era of HIV, many cases of the former type have been reported, whereas cases of malignancyassociated EPF are rarely reported. We report a case of EPF associated with Sezary syndrome (SS), and discuss possible mechanisms these might have in common. A 42-year-old Japanese man presented with a 3-month history of generalized erythroderma with lymphadenopathy (Fig. 1a). Histology of a biopsy specimen showed infiltration of atypical lymphocytes into the upper dermis with epidermotropism. Tumour cells were positive for CD3, CD4 and CD5. Laboratory tests showed a leucocyte count of 8.1 9 109/L (normal range: 3.5–9.2 9 109/L) with 24% atypical lymphocytes (absolute Sezary cell count of 1944 cells/mm3). Serum lactate dehydrogenase level was 400 IU/L (normal range: 125–237 IU/L) and soluble interleukin-2 receptor level was 1535 U/mL (normal range: 167–497 U/mL). Antibody against human T-cell leukaemia virus type I gave negative results. The same rearranged bands of T cell receptor-c were detected in samples of peripheral blood and lesional skin. The patient was diagnosed with SS (T4N1M0B2; stage IVA1).1,2 Because none of standard treatments for SS such as extracorporeal photochemotherapy, interferon-a, denileukin diftitox or chlorambucil were available in our country, the patient was treated with topical steroid and narrowband ultraviolet B phototherapy. Erythroderma was improved about 1 year after the start of UV therapy. At this point, the patient developed pruritic reddish follicular papules on both cheeks (Fig. 1b). Leucocyte count was 5.5 9 109/L with 1.5% atypical lymphocytes and 9.5% eosinophils. Histopathology showed massive infiltration of eosinophils and lymphocytes around hair follicles (Fig. 2). No atypical lymphocytes were detected. The final diagnosis was EPF associated with SS. The pruritic papules responded well to the addition of oral indomethacin (75 mg per day). SS is a rare type of cutaneous T-cell lymphoma (CTCL) characterized by generalized erythroderma, lymphadenopathy, and presence of > 1000 per mm3 circulating atypical T cells, so-called Sezary cells. Infiltration of Sezary cells into the skin can clinically present as multiple papules. However, in our patient, the erythroderma had cleared by the time the pruritic papules appeared. The
ª 2014 British Association of Dermatologists
Correspondence
(a)
Figure 2 Massive infiltration of eosinophils and lymphocytes
around the hair follicles (haematoxylin and eosin, original magnification 9400).
(b)
Infiltration of M2 macrophages expressing CD163 has been reported. Interestingly, SS is also regarded as a Th2type disease, frequently accompanied by eosinophilia and high serum levels of IgE.4 We have reported previously that CCL26 expression is increased in both the sera and lesional skin of patients with CTCL. Moreover, CD163positive macrophages infiltrate into lesional skin of CTCL.5 Thus, a Th2-dominant environment could be the common underlying pathology of EPF and SS. M. Sugaya, H. Suga, T. Miyagaki, H. Fujita and S. Sato Department of Dermatology, Faculty of Medicine, University of Tokyo 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 8 December 2013
References
Figure 1 (a) Erythroderma with severe pruritus; (b) pruritic fol-
licular papules on the right cheek.
lack of atypical lymphocytes and presence of numerous eosinophils in the histology specimen, along with the good response to indomethacin, supported the diagnosis of EPF. EPF is often accompanied by peripheral blood eosinophilia and raised serum IgE levels, and a T-helper (Th)2 cytokine-dominant condition has been postulated as the underlying mechanism. CCL26, a CC chemokine, also called eotaxin-3, is reported to be important in EPF.3
ª 2014 British Association of Dermatologists
1 Vonderheid EC, Bernengo MG, Burg G et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol 2002; 46: 95–106. 2 Olsen E, Vonderheid E, Pimpinelli N et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood 2007; 110: 1713–22. 3 Yokobayashi H, Sugaya M, Miyagaki T et al. Analysis of serum chemokine levels in patients with HIV-associated eosinophilic folliculitis. J Eur Acad Dermatol Venereol 2013; 27: e212–16.
Clinical and Experimental Dermatology (2014) 39, pp533–541
537
Correspondence
4 Dummer R, Heald PW, Nestle FO et al. Sezary syndrome T-cell clones display T-helper 2 cytokines and express the accessory factor-1 (interferon-gamma receptor betachain). Blood 1996; 88: 1383–9. 5 Sugaya M, Miyagaki T, Ohmatsu H et al. Association of the numbers of CD163(+) cells in lesional skin and serum levels of soluble CD163 with disease progression of cutaneous T cell lymphoma. J Dermatol Sci 2012; 68: 45–51.
Ultrasound B-mode and elastographic findings of angiomatoid fibrous histiocytoma doi: 10.1111/ced.12314 Angiomatoid fibrous histiocytoma (AFH) is a rare softtissue tumour of low- to intermediate-grade malignancy.1 The first case of this tumour was described in 1979, and the tumour was called ‘angiomatoid malignant fibrous
(a)
(d)
(b)
(e)
(c)
(f)
Figure 1 (a) a subcutaneous nodule, 10 mm in diameter, on the scalp. (b) Ultrasonography (B-mode) showed a cystic hypoechoic area
on the scalp (arrows), while colour Doppler ultrasonography revealed hypervascularity at the periphery of the cystic area (inset). (c) Ultrasound elastography showed a butterfly-shaped area with high elasticity, appearing as red at the centre of the cystic lesion (arrowheads). (d) Haemorrhagic and cystic spaces were visible in the lesion; (e) the periphery of the lesion was partly hyalinized, forming a pseudocapsule, and the haemorrhagic cystic spaces were lined with flattened tumour cells; (f) the tumour cells with nuclear atypia were composed of spindle-shape to ovoid-shaped cells arranged in sheets, whorls and short fascicle patterns Haematoxylin and eosin; original magnification (a) 920; (b) 9100; (c) 9400.
538
Clinical and Experimental Dermatology (2014) 39, pp533–541
ª 2014 British Association of Dermatologists
