Letters to the editor
825
nocardiosis, leishmaniasis, tularaemia and other deep fungal or bacterial infections. Diagnosis in these cases is usually established on histopathology, including special stains and culture. However, histology in our patient revealed malignant mesenchymal tumour which was subsequently confirmed on IHC. To the best of our knowledge, this type of presentation of MPNST has not been reported in literature and we suggest the inclusion of MPNST in the differential diagnosis of multiple linear nodules presenting in a sporotrichoid distribution so that an early recognition of this condition can prevent the morbidity and mortality ultimately associated with dissemination. U. Khanna,1,* T. K. Dhali,1 P. D’Souza,1 O. Kaur2 1
Department of Dermatology, Venereology and Leprology, ESIPGIMSR Basaidarpur, New Delhi, India, 2 Department of Pathology, ESIPGIMSR Basaidarpur, New Delhi, India *Correspondence: U. Khanna. E-mail: [email protected]
References 1 Strauss M, Grey L. Malignant tumors of the peripheral nerves. In Weiss SW, Goldblum JR, eds. Enzinger and Weiss’s Soft Tissue Tumors, 4th edn. Mosby, St. Louis, 2001: 903–944. 2 Tucker T, Wolkenstein P, Revuz J et al. Association between benign and malignant peripheral nerve sheath tumors in NF1. Neurology 2005; 65: 205–211. 3 Friedrich RE, Kluwe L, Funsterer C et al. Malignant peripheral nerve sheath tumors in neurofibromatosis type 1 (NF1): diagnostic findings on magnetic resonance images and mutation analysis of the NF1 gene. Anticancer Res 2005; 25: 1699–1702. 4 Evans DG, Baser ME, McGaughran J et al. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 2002; 39: 311. 5 Ducatman BS, Scheithauer B, Piepgras DG, Reiman HM. Malignant peripheral nerve sheath tumors in childhood. J Neurooncol 1984; 2: 241–248. 6 Minovi A, Basten O, Hunter B et al. Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck 2007; 29: 439–445. DOI: 10.1111/jdv.12428
Primary cutaneous follicle centre lymphoma presenting as diffuse facial erythema Editor A 59-year-old woman with a history of allergic contact dermatitis and Hashimoto thyroiditis developed a diffuse and ill-defined macular erythema of her forehead that progressed to her scalp and superior cheeks over the course of 6 months (Fig. 1). In addition to, there were multiple discrete 6–8 mm erythematous non-scaling papules coalescing into a plaque on the left inferior
JEADV 2015, 29, 818–832
Figure 1 (above) Ill-defined macular erythema of the scalp, forehead and superior cheeks.
mandible, and she also noted thinning of the hair of the frontal scalp associated with diffuse macular erythema. Patchy perifollicular erythema with minimal perifollicular hyperkeratosis was also noted on the scalp, along with subtle violaceous induration on the vertex and mid-frontal scalp without nodularity, ulceration or infiltrative plaques. A biopsy of the scalp showed an atypical B-cell infiltrate suspicious for follicular lymphoma. She was referred to the Cutaneous Lymphoma Clinic at the Huntsman Cancer Institute for definitive diagnosis and staging. Four additional staging biopsies were performed, one from each of the areas of concern (left scalp vertex, mid-frontal scalp, forehead and left mandible). All showed similar findings of a dense, nodular and focally diffuse lymphocyte-predominant infiltrate, with medium-to-large atypical CD20 positive B cells that coexpressed Bcl-6, were negative for Bcl-2 (not shown) and were arranged in aberrantly formed follicles, consistent with the diagnosis of follicle centre lymphoma (Fig. 2). Although a B cell- predominant lymphoid hyperplasia secondary to medications was considered, a review of the patient’s medications revealed no culprit medications classically associated with pseudolymphomas.1 Review of systems was negative for ‘B’ symptoms. Lymph node examination was unremarkable except for a small 0.5-cm soft, fluctuant, non-fixed lymph node on the left superior lateral neck. Routine laboratory tests including a CBC, CMP and LDH were unremarkable. An ANA was negative. Viral hepatitis serologies were negative. Contrast-enhanced computed tomographic scans of the chest and pelvis showed mildly enlarged right hilar nodes and multiple scattered mesenteric and retroperitoneal lymph nodes that were non-pathologic by size.
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
826
(a)
(b)
As a result, she was diagnosed with stage IE (T2c, N0, M0) primary cutaneous follicle centre lymphoma (PCFCL). While local radiotherapy is typically a first-line treatment,1 due to diffuse involvement of the face and scalp, our patient was treated with a 4-week course of systemic anti-CD20 (rituximab) and achieved resolution of her symptoms and facial erythema. Multifocal disease and non-specific diffuse macular erythema of the face and scalp is a previously unreported presentation of PCFCL. PCFCL characteristically presents as a solitary or grouped non-scaling erythematous papule(s), infiltrative plaque(s) or tumour(s) localized on the forehead, scalp, temples, neck, trunk or upper extremities.2–5 This patient’s presentation of diffuse erythema along with subtle perifollicular scaling might lead the clinician to first consider inflammatory cutaneous disease such as lupus erythematosus, rosacea or lichen planopilaris. This report serves to add to the expanding body of knowledge on the spectrum of clinical presentations of PCFCL, of which dermatologists should be aware, so that skin biopsies may be performed to establish a timely diagnosis, and guide appropriate treatment and follow-up. C. Wada,1 M.J. Glenn,2 M. Hyde,3 D.L. Powell,4 R.R. Miles,5 K. Duffy,3,4 S.R. Florell,4 D.A. Wada3,4,* 1
(c)
John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, 2 Division of Hematology and Hematologic Malignancies/Oncology, Department of Internal Medicine, Huntsman Cancer Institute, 3 Department of Oncological Dermatology, Huntsman Cancer Institute, 4 Department of Dermatology, University of Utah School of Medicine, 5 Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT, USA *Correspondence: D.A. Wada. E-mail: [email protected]
References
Figure 2 (right) (a) Dense, nodular lymphocyte-predominant infiltrate (H&E stain, 409), (b) Medium-to-large atypical CD20 positive B cells arranged in aberrantly formed follicles (CD20 stain, 2009), which (c) co-expressed Bcl-6 (Bcl-6 stain, 2009).
JEADV 2015, 29, 818–832
1 Albrech J, Fine LA, Piette W. Drug-associated lymphoma and pseudolymphoma: recognition and management. Dermatol Clin 2007; 25: 233–244. 2 Gulia A, Saggini A, Wiesner T et al. Clinicopathologic features of early lesions of primary cutaneous follicle center lymphoma, diffuse type: implications for early diagnosis and treatment. J Am Acad Dermatol 2011; 65: 991–1000. 3 Willemze R, Jaffe ES, Burg G et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768–3785. 4 Barzilai A, Feuerman H, Quaglino P et al. Cutaneous B-cell neoplasms mimicking granulomatous rosacea or rhinophyma. Arch Dermatol 2012; 148: 824–831. 5 Massone C, Fink-Puches R, Laimer M, Rutten A, Vale E, Cerroni L. Miliary and agminated-type primary cutaneous follicle center lymphoma: report of 18 cases. J Am Acad Dermatol 2011; 65: 749–755. DOI: 10.1111/jdv.12430
© 2014 European Academy of Dermatology and Venereology
825
nocardiosis, leishmaniasis, tularaemia and other deep fungal or bacterial infections. Diagnosis in these cases is usually established on histopathology, including special stains and culture. However, histology in our patient revealed malignant mesenchymal tumour which was subsequently confirmed on IHC. To the best of our knowledge, this type of presentation of MPNST has not been reported in literature and we suggest the inclusion of MPNST in the differential diagnosis of multiple linear nodules presenting in a sporotrichoid distribution so that an early recognition of this condition can prevent the morbidity and mortality ultimately associated with dissemination. U. Khanna,1,* T. K. Dhali,1 P. D’Souza,1 O. Kaur2 1
Department of Dermatology, Venereology and Leprology, ESIPGIMSR Basaidarpur, New Delhi, India, 2 Department of Pathology, ESIPGIMSR Basaidarpur, New Delhi, India *Correspondence: U. Khanna. E-mail: [email protected]
References 1 Strauss M, Grey L. Malignant tumors of the peripheral nerves. In Weiss SW, Goldblum JR, eds. Enzinger and Weiss’s Soft Tissue Tumors, 4th edn. Mosby, St. Louis, 2001: 903–944. 2 Tucker T, Wolkenstein P, Revuz J et al. Association between benign and malignant peripheral nerve sheath tumors in NF1. Neurology 2005; 65: 205–211. 3 Friedrich RE, Kluwe L, Funsterer C et al. Malignant peripheral nerve sheath tumors in neurofibromatosis type 1 (NF1): diagnostic findings on magnetic resonance images and mutation analysis of the NF1 gene. Anticancer Res 2005; 25: 1699–1702. 4 Evans DG, Baser ME, McGaughran J et al. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 2002; 39: 311. 5 Ducatman BS, Scheithauer B, Piepgras DG, Reiman HM. Malignant peripheral nerve sheath tumors in childhood. J Neurooncol 1984; 2: 241–248. 6 Minovi A, Basten O, Hunter B et al. Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck 2007; 29: 439–445. DOI: 10.1111/jdv.12428
Primary cutaneous follicle centre lymphoma presenting as diffuse facial erythema Editor A 59-year-old woman with a history of allergic contact dermatitis and Hashimoto thyroiditis developed a diffuse and ill-defined macular erythema of her forehead that progressed to her scalp and superior cheeks over the course of 6 months (Fig. 1). In addition to, there were multiple discrete 6–8 mm erythematous non-scaling papules coalescing into a plaque on the left inferior
JEADV 2015, 29, 818–832
Figure 1 (above) Ill-defined macular erythema of the scalp, forehead and superior cheeks.
mandible, and she also noted thinning of the hair of the frontal scalp associated with diffuse macular erythema. Patchy perifollicular erythema with minimal perifollicular hyperkeratosis was also noted on the scalp, along with subtle violaceous induration on the vertex and mid-frontal scalp without nodularity, ulceration or infiltrative plaques. A biopsy of the scalp showed an atypical B-cell infiltrate suspicious for follicular lymphoma. She was referred to the Cutaneous Lymphoma Clinic at the Huntsman Cancer Institute for definitive diagnosis and staging. Four additional staging biopsies were performed, one from each of the areas of concern (left scalp vertex, mid-frontal scalp, forehead and left mandible). All showed similar findings of a dense, nodular and focally diffuse lymphocyte-predominant infiltrate, with medium-to-large atypical CD20 positive B cells that coexpressed Bcl-6, were negative for Bcl-2 (not shown) and were arranged in aberrantly formed follicles, consistent with the diagnosis of follicle centre lymphoma (Fig. 2). Although a B cell- predominant lymphoid hyperplasia secondary to medications was considered, a review of the patient’s medications revealed no culprit medications classically associated with pseudolymphomas.1 Review of systems was negative for ‘B’ symptoms. Lymph node examination was unremarkable except for a small 0.5-cm soft, fluctuant, non-fixed lymph node on the left superior lateral neck. Routine laboratory tests including a CBC, CMP and LDH were unremarkable. An ANA was negative. Viral hepatitis serologies were negative. Contrast-enhanced computed tomographic scans of the chest and pelvis showed mildly enlarged right hilar nodes and multiple scattered mesenteric and retroperitoneal lymph nodes that were non-pathologic by size.
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
826
(a)
(b)
As a result, she was diagnosed with stage IE (T2c, N0, M0) primary cutaneous follicle centre lymphoma (PCFCL). While local radiotherapy is typically a first-line treatment,1 due to diffuse involvement of the face and scalp, our patient was treated with a 4-week course of systemic anti-CD20 (rituximab) and achieved resolution of her symptoms and facial erythema. Multifocal disease and non-specific diffuse macular erythema of the face and scalp is a previously unreported presentation of PCFCL. PCFCL characteristically presents as a solitary or grouped non-scaling erythematous papule(s), infiltrative plaque(s) or tumour(s) localized on the forehead, scalp, temples, neck, trunk or upper extremities.2–5 This patient’s presentation of diffuse erythema along with subtle perifollicular scaling might lead the clinician to first consider inflammatory cutaneous disease such as lupus erythematosus, rosacea or lichen planopilaris. This report serves to add to the expanding body of knowledge on the spectrum of clinical presentations of PCFCL, of which dermatologists should be aware, so that skin biopsies may be performed to establish a timely diagnosis, and guide appropriate treatment and follow-up. C. Wada,1 M.J. Glenn,2 M. Hyde,3 D.L. Powell,4 R.R. Miles,5 K. Duffy,3,4 S.R. Florell,4 D.A. Wada3,4,* 1
(c)
John A. Burns School of Medicine, University of Hawaii, Honolulu, HI, 2 Division of Hematology and Hematologic Malignancies/Oncology, Department of Internal Medicine, Huntsman Cancer Institute, 3 Department of Oncological Dermatology, Huntsman Cancer Institute, 4 Department of Dermatology, University of Utah School of Medicine, 5 Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT, USA *Correspondence: D.A. Wada. E-mail: [email protected]
References
Figure 2 (right) (a) Dense, nodular lymphocyte-predominant infiltrate (H&E stain, 409), (b) Medium-to-large atypical CD20 positive B cells arranged in aberrantly formed follicles (CD20 stain, 2009), which (c) co-expressed Bcl-6 (Bcl-6 stain, 2009).
JEADV 2015, 29, 818–832
1 Albrech J, Fine LA, Piette W. Drug-associated lymphoma and pseudolymphoma: recognition and management. Dermatol Clin 2007; 25: 233–244. 2 Gulia A, Saggini A, Wiesner T et al. Clinicopathologic features of early lesions of primary cutaneous follicle center lymphoma, diffuse type: implications for early diagnosis and treatment. J Am Acad Dermatol 2011; 65: 991–1000. 3 Willemze R, Jaffe ES, Burg G et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768–3785. 4 Barzilai A, Feuerman H, Quaglino P et al. Cutaneous B-cell neoplasms mimicking granulomatous rosacea or rhinophyma. Arch Dermatol 2012; 148: 824–831. 5 Massone C, Fink-Puches R, Laimer M, Rutten A, Vale E, Cerroni L. Miliary and agminated-type primary cutaneous follicle center lymphoma: report of 18 cases. J Am Acad Dermatol 2011; 65: 749–755. DOI: 10.1111/jdv.12430
© 2014 European Academy of Dermatology and Venereology
