Correspondence
The patient was diagnosed with isolated AL amyloidosis without associated hematologic neoplasia. The cutaneous clinical presentation indicated the presence of diffuse plane xanthoma-like lesions; however, histopathologic examination led to the differential diagnosis of AL amyloidosis. Hematologic neoplasia can be associated with AL amyloidosis or diffuse plane xanthomatosis, necessitating a detailed clinical investigation.2,6 Amyloid elastosis characteristically presents with plane xanthoma-like lesions. Cases of AL amyloidosis with such lesions, which have been reported to date, correspond to this variant. However, degenerative changes in the elastic fibers and the deposition of amyloid material around the fibers, typical of amyloid elastosis, were not observed in the present case.5,7 The coexistence of AL amyloidosis with normolipemic diffuse plane xanthomatosis in a patient with monoclonal gammopathy has been previously reported.8 However, in the present case, the absence of lipid deposits on histopathologic examination indicated that AL amyloidosis was not associated with diffuse plane xanthomatosis. Thus, AL amyloidosis should be considered in the differential diagnosis of patients presenting with xanthomatous lesions. Bruno Sima˜o dos Santos1, MD Antonio Diniz1, MD Roge´rio Estrella1, MD Mayra Rochael2, MD PhD 1 Dermatology and 2Pathology, Hospital Universitário Antonio Pedro – UFF Rua Marques de Paraná
Plaque-type blue nevus on the palm
Editor, A 34-year-old man presented with an asymptomatic, large, blue macula on the right palm, which had been present since the subject was two years old. No history of trauma, burn, drug use, or previous skin disease in the hand was reported. The lesion had grown proportionally in line with the patient. Over the previous few years, the lesion had increased a little in size but had shown no changes in texture or color. The plaque had no pulse, and nuclear magnetic resonance imaging ruled out the presence of vascular disease. No other pigmentary changes or lesions were noted, and the subject’s general health was otherwise normal. Physical examination revealed a blue-to-black plaque on the right palm composed of multiple plaques, papules, and nodules irregularly demarcated, and with maximum dimensions of 8 · 6 cm (Fig. 1). Dermoscopy showed a lattice-like, homogeneous, blue dermal pattern (Fig. 2). A biopsy specimen from a papular lesion was obtained and stained with hematoxylin and eosin. The biopsy ª 2013 The International Society of Dermatology
1405
303 - Centro, Niterói Rio de Janeiro 24033-900 Brazil E-mail: [email protected]
References 1 Varez-Ruiz SB, García-Río I, Daudén E. Systemic amyloidoses. Actas Dermosifiliogr 2005; 96: 69–82. 2 Kumar S, Gertz MA. Cutaneous and systemic amyloidoses. Int J Dermatol 2002; 41: 133–134. 3 Lópes L, González K, Navarrete G, et al. Multiple myeloma and systemic amyloidosis. Int J Dermatol 2008; 47: 165–167. 4 Courivaud-Canonne D, Meyns B, Vandaele A, et al. Cutaneous lesions with a primary systemic amyloidosis. Ann Dermatol Venereol 2004; 131: 492–493. 5 Vecchietti G, Masouyé I, Salomon D, et al. An unusual form of primary systemic amyloidosis: amyloid elastosis: report of a case treated by hematopoietic cell transplantation. Br J Dermatol 2003; 148: 154–159. 6 Kourou K, Suga Y, Muramatsu S, et al. A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy. J Dermatol 2006; 33: 64–67. 7 Bocquier B, D’Incan M, Joubert J, et al. Amyloid elastosis: a new case studied extensively by electron microscopy and immunohistochemistry. Br J Dermatol 2008; 158: 858–860. 8 Buezo GF, Porras JI, Fraga J, et al. Coexistence of diffuse plane normolipemic xanthoma and amyloidosis in a patient with monoclonal gammopathy. Br J Dermatol 1996; 135: 460–462.
showed an acanthotic epidermis and the presence in the reticular dermis of clumps of dendritic and spindle-shaped melanocytic cells mixed with irregularly arranged collagen bundles (Fig. 3). No cytologic atypia or mitotic activity were observed. The benign-appearing melanocytes extended into the subcutaneous tissues surrounding nerves, vessels, and pilosebaceous apparatus in a plexiform pattern (Fig. 3), surrounding the adipocites (Fig. 4). Immunohistochemistry with Melan A stain revealed melanocytes distributed in a plexiform manner and involving the adipose tissue (Fig. 5). A plaque-type common blue nevus was diagnosed. The plaque-type blue nevus (BN) is a rare variant of BN, of which only a few reports appear in the English language literature.1,2 There are three types of BN: common; cellular; and the least common, plaque-type BN. All of these belong to the dermal melanocytoses (DM), which are characterized by the presence of melanocytes in the dermis.1 Clinically, these are bluish plaques composed of multiple macules, papules, and nodules, which range in size from 1 to 25 cm in diameter and have a predilection for the trunk. To our knowledge, the present report International Journal of Dermatology 2013, 52, 1398–1461
1405
1406
Correspondence
Figure 1 Clinical examination showed a large, bluish plaque
on the right palm, irregularly demarcated and composed of multiple macules, papules and nodules
Figure 3 Histopathology showed an acanthotic epidermis and proliferation of clumps of dendritic and spindle-shaped melanocytic cells in the mid to reticular dermis, mixed with irregularly arranged collagen bundles. (Hematoxylin and eosin stain; original magnification ·40.) Inset: the benign-appearing melanocytes extended into the subcutaneous tissues surrounding nerves, vessels and pilosebaceous apparatus in a plexiform pattern (H&E stain; ·original magnification 100)
Figure 2 Dermoscopy showed a blue, lattice-like, homoge-
neous pattern
represents the first case of plaque-type BN involving the palm to be published. This type of BN may also be seen as a blue patch without palpable lesions.3–5 Histopathologically, this plaque is composed of islands of melanin-rich dendritic melanocytes intermingled throughout the collagen bundles in the reticular dermis in a manner similar to that in common BN, although it may sometimes show features consistent with cellular BN.3,4,6 In addition, in plaque-type BN, benign-appearing melanocytes involve the subcutaneous tissue and surrounding hair follicles, nerves, and vessels, without cytologic atypia and preserving the architecture of the involved structures.3,4,6 Sometimes this perifollicular, perineural, and perivascular involvement of the benign melanocytes shows a plexiform or reticular pattern, as in our case. Plaque-type BN may International Journal of Dermatology 2013, 52, 1398–1461
Figure 4 Numerous melanocytes surround the adipocites without dissecting or infiltrating the adipose lobule. (H&E stain; ·200)
be congenital or may appear in infancy or adult life. None of the cases of plaque-type BN reported in the literature demonstrated malignant transformation.4 However, given the small number of cases reported, it is difficult to establish any definitive conclusions on the potential risk for malignant transformation and the best treatment. Most authors recommend that, because the lesion is usually asymptomatic, involves a large area, and has uncertain malignant potential, close observation and follow-up are prudent, and any new lesions that appear should be biopª 2013 The International Society of Dermatology
Correspondence
Ramo´n Garcı´a, MD Jose´ L Sa´nchez, PhD Vı´ctor Alegre, PhD Department of Dermatology General Hospital of Valencia and Department of Dermatology Instituto Valenciano de Oncología (Valencian Institute of Oncology) Valencia Spain E-mail: [email protected]
References Figure 5 Immunohistochemistry was positive for melanocytes
distributed in a plexiform manner and invading the adipose tissue. (Melan A stain; ·40)
sied.1,3,6 Other authors agree that surgical excision should be performed if the size of the lesion permits it.3,4 In our patient, because of the size and location of the lesion, we elected to maintain close follow-up and observation every six months (we have already completed the first year of follow-up under this schedule) and to biopsy any new lesions that might arise. Our patient has shown no clinical or dermoscopic changes in 12 months of follow-up. Anna Agusti-Mejias, MD Francesc Messeguer, MD
Lower-dose thalidomide therapy effectively treats cutaneous lupus erythematosus but is limited by neuropathic toxicity
Editor, Thalidomide has been used to treat refractory cutaneous lupus erythematosus (CLE),1,2 but neuropathic side effects at previously studied doses limit its use. It inhibits inflammatory cytokine synthesis and prevents ultraviolet B-induced keratinocyte apoptosis, both of which are thought to contribute to its efficacy in CLE.1 We assessed the response of CLE to a low starting dose of thalidomide of 50 mg/d. From July 2007 to August 2009, five patients with CLE seen at the Brigham and Women’s Hospital Department of Dermatology were treated with thalidomide. Two patients had discoid lupus erythematosus, and three had subacute CLE. The patients’ medical records were reviewed retrospectively for data on demographics, diagnosis, baseline cutaneous disease, and treatment response. Because of thalidomide’s teratogenic
ª 2013 The International Society of Dermatology
1 Pittman JL, Fisher BK. Plaque-type blue nevus. Arch Dermatol 1976; 112: 1127–1128. 2 Ishibashi A, Kimura K, Kukita A. Plaque-type blue nevus combined with lentigo (nevus spilus). J Cutan Pathol 1990; 17: 241–245. 3 Busam KJ, Woodruff JM, Erlandson RA, et al. Large plaque-type blue nevus with subcutaneous cellular nodules. Am J Surg Pathol 2000; 24: 92–99. 4 Wen SY. Plaque-type blue nevus. Review and an unusual case. Acta Derm Venereol 1997; 77: 458–459. 5 Hendricks WM. Eruptive blue nevi. J Am Acad Dermatol 1981; 4: 50–53. 6 Skowron F, Balme B. Large plaque-type blue nevus with subcutaneous cellular nodules. Clin Exp Dermatol 2009; 34: 782–784.
effects, all patients adhered to the System for Thalidomide Education and Prescribing Safety program.2 Cutaneous disease response was classified as complete (>75% improvement), partial (25–75% improvement), or no response (
The patient was diagnosed with isolated AL amyloidosis without associated hematologic neoplasia. The cutaneous clinical presentation indicated the presence of diffuse plane xanthoma-like lesions; however, histopathologic examination led to the differential diagnosis of AL amyloidosis. Hematologic neoplasia can be associated with AL amyloidosis or diffuse plane xanthomatosis, necessitating a detailed clinical investigation.2,6 Amyloid elastosis characteristically presents with plane xanthoma-like lesions. Cases of AL amyloidosis with such lesions, which have been reported to date, correspond to this variant. However, degenerative changes in the elastic fibers and the deposition of amyloid material around the fibers, typical of amyloid elastosis, were not observed in the present case.5,7 The coexistence of AL amyloidosis with normolipemic diffuse plane xanthomatosis in a patient with monoclonal gammopathy has been previously reported.8 However, in the present case, the absence of lipid deposits on histopathologic examination indicated that AL amyloidosis was not associated with diffuse plane xanthomatosis. Thus, AL amyloidosis should be considered in the differential diagnosis of patients presenting with xanthomatous lesions. Bruno Sima˜o dos Santos1, MD Antonio Diniz1, MD Roge´rio Estrella1, MD Mayra Rochael2, MD PhD 1 Dermatology and 2Pathology, Hospital Universitário Antonio Pedro – UFF Rua Marques de Paraná
Plaque-type blue nevus on the palm
Editor, A 34-year-old man presented with an asymptomatic, large, blue macula on the right palm, which had been present since the subject was two years old. No history of trauma, burn, drug use, or previous skin disease in the hand was reported. The lesion had grown proportionally in line with the patient. Over the previous few years, the lesion had increased a little in size but had shown no changes in texture or color. The plaque had no pulse, and nuclear magnetic resonance imaging ruled out the presence of vascular disease. No other pigmentary changes or lesions were noted, and the subject’s general health was otherwise normal. Physical examination revealed a blue-to-black plaque on the right palm composed of multiple plaques, papules, and nodules irregularly demarcated, and with maximum dimensions of 8 · 6 cm (Fig. 1). Dermoscopy showed a lattice-like, homogeneous, blue dermal pattern (Fig. 2). A biopsy specimen from a papular lesion was obtained and stained with hematoxylin and eosin. The biopsy ª 2013 The International Society of Dermatology
1405
303 - Centro, Niterói Rio de Janeiro 24033-900 Brazil E-mail: [email protected]
References 1 Varez-Ruiz SB, García-Río I, Daudén E. Systemic amyloidoses. Actas Dermosifiliogr 2005; 96: 69–82. 2 Kumar S, Gertz MA. Cutaneous and systemic amyloidoses. Int J Dermatol 2002; 41: 133–134. 3 Lópes L, González K, Navarrete G, et al. Multiple myeloma and systemic amyloidosis. Int J Dermatol 2008; 47: 165–167. 4 Courivaud-Canonne D, Meyns B, Vandaele A, et al. Cutaneous lesions with a primary systemic amyloidosis. Ann Dermatol Venereol 2004; 131: 492–493. 5 Vecchietti G, Masouyé I, Salomon D, et al. An unusual form of primary systemic amyloidosis: amyloid elastosis: report of a case treated by hematopoietic cell transplantation. Br J Dermatol 2003; 148: 154–159. 6 Kourou K, Suga Y, Muramatsu S, et al. A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy. J Dermatol 2006; 33: 64–67. 7 Bocquier B, D’Incan M, Joubert J, et al. Amyloid elastosis: a new case studied extensively by electron microscopy and immunohistochemistry. Br J Dermatol 2008; 158: 858–860. 8 Buezo GF, Porras JI, Fraga J, et al. Coexistence of diffuse plane normolipemic xanthoma and amyloidosis in a patient with monoclonal gammopathy. Br J Dermatol 1996; 135: 460–462.
showed an acanthotic epidermis and the presence in the reticular dermis of clumps of dendritic and spindle-shaped melanocytic cells mixed with irregularly arranged collagen bundles (Fig. 3). No cytologic atypia or mitotic activity were observed. The benign-appearing melanocytes extended into the subcutaneous tissues surrounding nerves, vessels, and pilosebaceous apparatus in a plexiform pattern (Fig. 3), surrounding the adipocites (Fig. 4). Immunohistochemistry with Melan A stain revealed melanocytes distributed in a plexiform manner and involving the adipose tissue (Fig. 5). A plaque-type common blue nevus was diagnosed. The plaque-type blue nevus (BN) is a rare variant of BN, of which only a few reports appear in the English language literature.1,2 There are three types of BN: common; cellular; and the least common, plaque-type BN. All of these belong to the dermal melanocytoses (DM), which are characterized by the presence of melanocytes in the dermis.1 Clinically, these are bluish plaques composed of multiple macules, papules, and nodules, which range in size from 1 to 25 cm in diameter and have a predilection for the trunk. To our knowledge, the present report International Journal of Dermatology 2013, 52, 1398–1461
1405
1406
Correspondence
Figure 1 Clinical examination showed a large, bluish plaque
on the right palm, irregularly demarcated and composed of multiple macules, papules and nodules
Figure 3 Histopathology showed an acanthotic epidermis and proliferation of clumps of dendritic and spindle-shaped melanocytic cells in the mid to reticular dermis, mixed with irregularly arranged collagen bundles. (Hematoxylin and eosin stain; original magnification ·40.) Inset: the benign-appearing melanocytes extended into the subcutaneous tissues surrounding nerves, vessels and pilosebaceous apparatus in a plexiform pattern (H&E stain; ·original magnification 100)
Figure 2 Dermoscopy showed a blue, lattice-like, homoge-
neous pattern
represents the first case of plaque-type BN involving the palm to be published. This type of BN may also be seen as a blue patch without palpable lesions.3–5 Histopathologically, this plaque is composed of islands of melanin-rich dendritic melanocytes intermingled throughout the collagen bundles in the reticular dermis in a manner similar to that in common BN, although it may sometimes show features consistent with cellular BN.3,4,6 In addition, in plaque-type BN, benign-appearing melanocytes involve the subcutaneous tissue and surrounding hair follicles, nerves, and vessels, without cytologic atypia and preserving the architecture of the involved structures.3,4,6 Sometimes this perifollicular, perineural, and perivascular involvement of the benign melanocytes shows a plexiform or reticular pattern, as in our case. Plaque-type BN may International Journal of Dermatology 2013, 52, 1398–1461
Figure 4 Numerous melanocytes surround the adipocites without dissecting or infiltrating the adipose lobule. (H&E stain; ·200)
be congenital or may appear in infancy or adult life. None of the cases of plaque-type BN reported in the literature demonstrated malignant transformation.4 However, given the small number of cases reported, it is difficult to establish any definitive conclusions on the potential risk for malignant transformation and the best treatment. Most authors recommend that, because the lesion is usually asymptomatic, involves a large area, and has uncertain malignant potential, close observation and follow-up are prudent, and any new lesions that appear should be biopª 2013 The International Society of Dermatology
Correspondence
Ramo´n Garcı´a, MD Jose´ L Sa´nchez, PhD Vı´ctor Alegre, PhD Department of Dermatology General Hospital of Valencia and Department of Dermatology Instituto Valenciano de Oncología (Valencian Institute of Oncology) Valencia Spain E-mail: [email protected]
References Figure 5 Immunohistochemistry was positive for melanocytes
distributed in a plexiform manner and invading the adipose tissue. (Melan A stain; ·40)
sied.1,3,6 Other authors agree that surgical excision should be performed if the size of the lesion permits it.3,4 In our patient, because of the size and location of the lesion, we elected to maintain close follow-up and observation every six months (we have already completed the first year of follow-up under this schedule) and to biopsy any new lesions that might arise. Our patient has shown no clinical or dermoscopic changes in 12 months of follow-up. Anna Agusti-Mejias, MD Francesc Messeguer, MD
Lower-dose thalidomide therapy effectively treats cutaneous lupus erythematosus but is limited by neuropathic toxicity
Editor, Thalidomide has been used to treat refractory cutaneous lupus erythematosus (CLE),1,2 but neuropathic side effects at previously studied doses limit its use. It inhibits inflammatory cytokine synthesis and prevents ultraviolet B-induced keratinocyte apoptosis, both of which are thought to contribute to its efficacy in CLE.1 We assessed the response of CLE to a low starting dose of thalidomide of 50 mg/d. From July 2007 to August 2009, five patients with CLE seen at the Brigham and Women’s Hospital Department of Dermatology were treated with thalidomide. Two patients had discoid lupus erythematosus, and three had subacute CLE. The patients’ medical records were reviewed retrospectively for data on demographics, diagnosis, baseline cutaneous disease, and treatment response. Because of thalidomide’s teratogenic
ª 2013 The International Society of Dermatology
1 Pittman JL, Fisher BK. Plaque-type blue nevus. Arch Dermatol 1976; 112: 1127–1128. 2 Ishibashi A, Kimura K, Kukita A. Plaque-type blue nevus combined with lentigo (nevus spilus). J Cutan Pathol 1990; 17: 241–245. 3 Busam KJ, Woodruff JM, Erlandson RA, et al. Large plaque-type blue nevus with subcutaneous cellular nodules. Am J Surg Pathol 2000; 24: 92–99. 4 Wen SY. Plaque-type blue nevus. Review and an unusual case. Acta Derm Venereol 1997; 77: 458–459. 5 Hendricks WM. Eruptive blue nevi. J Am Acad Dermatol 1981; 4: 50–53. 6 Skowron F, Balme B. Large plaque-type blue nevus with subcutaneous cellular nodules. Clin Exp Dermatol 2009; 34: 782–784.
effects, all patients adhered to the System for Thalidomide Education and Prescribing Safety program.2 Cutaneous disease response was classified as complete (>75% improvement), partial (25–75% improvement), or no response (
