- Email: [email protected]
Papular amyloidosis limited to the ears
1078 Letters
J AM ACAD DERMATOL JUNE 2010
Histopathologic study of the lesion showed a diffuse interstitial infiltrate with strands of neoplastic cells involving the entire dermis and extending into subcutaneous tissue with an overlying ulcerated epidermis (Fig 2, A). Cytologically, the infiltrate was composed of large cells with central to eccentrically placed, mildly pleomorphic nuclei with prominent nucleoli, and granular eosinophilic cytoplasm, consistent with plasmablasts (Fig 2, B and C ). The cells strongly expressed CD79a, CD138 (Fig 2, D), and epithelial membrane antigen by immunohistochemistry, and were negative for S-100 and Melan-A. There was monoclonal expression of kappa light chains and IgA. This was consistent with the patient’s serum electrophoresis results, supporting the neoplastic nature of the dermal plasma cells. The diagnosis of a cutaneous involvement of MM was established and the patient was referred to the oncology department. The patient died 6 months later. The largest series of cutaneous involvement in MM published by Requena et al3 in 2005 establishes the main characteristics of this rare phenomenon. It occurs most frequently in middle age to older males (range, 36-81 years) with IgG and IgA MM. Typically, skin lesions appear as multiple, violaceous, smooth papules and/or nodules located on the trunk, abdomen and, in lesser frequency, on the scalp, face, neck, and extremities. Ulceration and secondary infection are exceptional findings. Differential diagnosis in the reported case included those entities which could be present as ulcerated toe lesions, including acral melanoma, pyogenic granuloma, Kaposi sarcoma, and traumatic ulcer. Histopathologic analysis shows two patterns— nodular and diffuse interstitial types—with the former being the most prevalent. The neoplastic dermal infiltrate is often composed of immature plasmablasts that lack the typical cytologic features of plasma cells; imunohistochemical confirmation is therefore required to establish the diagnosis. Cutaneous involvement by plasma cell neoplasms usually heralds a poor prognosis regardless of the therapy used.2,3 In summary, we describe a well documented case of cutaneous MM mimicking an acral melanoma. Clinicopathologic correlation was essential to come to the correct diagnosis in this unusual myeloma presentation. The authors would like to thank Nicole Balmer, MD, of the Department of Pathology at Wake Forest University Baptist Medical Center, Winston-Salem, NC.
Antonio Martorell, MD,a Fernando Millan-Parrilla, MD,b and Enrique Gimeno-Carpio, MDb
Departments of Dermatology, Instituto Valenciano de Oncologı´aa and Hospital Arnau de Vilanova,b Valencia, Spain. Funding sources: None. Conflicts of interest: None declared. Correspondence to: Antonio Martorell, MD, Department of Dermatology, Instituto Valenciano Oncologı´a, San Jose de la Montan ˜ a St, 14, 46008, Valencia, Spain. E-mail: [email protected] REFERENCES 1. Patterson JW, Parson JM, White RM, Fitzpatrick JE, Kohout-Dutz E. Cutaneous involvement of multiple myeloma and extramedullary plasmacytoma. J Am Acad Dermatol 1988;19(5 pt 2):879-90. 2. Requena L, Kutzner H, Palmedo G, Calonje E, Requena C, Pe´rez G, et al. Cutaneous involvement in multiple myeloma: a clinicopathologic, immunohistochemical, and cytogenetic study of 8 cases. Arch Dermatol 2003;139:475-86. 3. Requena L. Specific cutaneous involvement in patients with multiple myeloma. A clinicopathological, immunohistochemical and cytogenetic study of 40 cases [in Spanish]. Actas Dermosifiliogr 2005;96:424-40. doi:10.1016/j.jaad.2009.07.012
Papular amyloidosis limited to the ears To the Editor: A 42-year-old woman presented with a greater than 10-year history of asymptomatic brown papules involving her external ears. The lesions had slowly increased in size and number. She denied antecedent trauma and other skin disease. The physical examination revealed multiple 1- to 2mm smooth, firm, brown papules involving the conchal bowls, crura of the antihelices, and external auditory canals bilaterally (Fig 1). There was no obstruction of the canals on otoscopic examination. The remainder of the full skin examination was normal. A histopathologic examination of the biopsy specimen revealed hyperkeratosis, elongation of the rete ridges, and collections of amorphous, faintly eosinophilic material within expanded dermal papillae (Fig 2). There were scattered melanophages within the superficial dermis. The material stained positively with Congo red and periodic acideSchiff stains; this is a characteristic of amyloids. The amyloidoses are a diverse group of disorders that have in common the deposition of various proteins with similar staining characteristics. The cutaneous amyloidoses may be classified as primary, secondary, or associated with systemic amyloidosis. Auricular amyloidosis is a rare form of primary cutaneous amyloidosis. The primary cutaneous amyloidoses include lichen, macular, and nodular types. Macular and
Letters 1079
J AM ACAD DERMATOL VOLUME 62, NUMBER 6
Fig 1. Multiple waxy brown papules involving the conchal bowls, crura of the antihelices, and external auditory canals bilaterally.
lesions elsewhere. Their patients had similar histopathology to ours, with nodular deposits of amyloid in the papillary dermis. Immunoperoxidase staining performed on one of their cases showed positive staining with the antikeratin antibody EKH4, confirming the keratinocyte origin of the amyloid. Previous authors had reported similar papules of the external ear under the title ‘‘collagenous papules of the external ear.’’2,3 Secondary localized cutaneous amyloidosis refers to the presence of amyloid in cutaneous neoplasms, such as seborrheic keratosis and basal cell carcinoma. The amyloid is usually keratinocyte-derived (AK). Cutaneous involvement is common in primary systemic amyloidosis, manifesting as waxy papules and plaques, often found on the face, scalp, neck, hands, and flexures. Hemorrhage into the lesions is common.4 The amyloid is derived from immunoglobulin light chains (AL) of the delta or kappa type. The lesions of amyloidosis of the auricular concha are asymptomatic, but may be of cosmetic concern to the patient. Because of the localized distribution and superficial pathology, treatment modalities such as curettage or excision may be considered. Ann G. Neff, MD,a Jill Buckthal McCuin, MD,b and Diya F. Mutasim, MDa Department of Dermatology,a University of Cincinnati College of Medicine, Cincinnati, Ohio, and Newnan Dermatology at Cowles Clinic,b Greensboro, Georgia Funding sources: None. Conflicts of interest: None declared.
Fig 2. Histologic examination of a biopsy specimen reveals hyperkeratosis, elongation of the rete ridges, and nodules of eosinophilic material that fill and expand dermal papillae. (Hematoxylineeosin stain.)
Correspondence to: Diya F. Mutasim, MD, Department of Dermatology, University of Cincinnati, 231 Albert Sabin Way, P.O. Box 670592, Cincinnati, OH 45267-0592 E-mail: [email protected]
lichen amyloidosis result from amyloid in the tips of the dermal papillae. The amyloid protein is derived from keratinocytes, and is referred to as amyloid K (AK) protein. Nodular amyloidosis presents with one or few waxy plaques or nodules, with diffuse infiltration of the dermis and subcutis by immunoglobulin light chainederived material, referred to as amyloid light chain (AL). Plasma cells, which may be monoclonal, are usually present and are the source of the amyloid deposition. Amyloidosis of the auricular concha is an uncommon variant of primary cutaneous amyloidosis, and was first reported by Hicks et al1 in 1988. The authors described four patients with papules of the auricle, acquired in adulthood, and unassociated with
REFERENCES 1. Hicks BC, Weber PJ, Hashimoto K, Ito K, Koreman DM. Primary cutaneous amyloidosis of the auricular concha. J Am Acad Dermatol 1988;18:19-25. 2. Sanchez JL. Collagenous papules on the aural conchae. Am J Dermatopathol 1983;5:231-3. 3. Dupre A, Lassere J, Bonafe JL, Viraben R, Gorguet B. Collagenous papules of the external ear. Micropapular hyalinosis of the external ear with transepithelial elimination. Relation to primary localized cutaneous amyloidosis [in French]. Ann Dermatol Venereol 1984;111:913-7. 4. Ratz JL, Bailin PL. Cutaneous amyloidosis. A case report of the tumefactive variant and a review of the spectrum of clinical presentations. J Am Acad Dermatol 1981;4:21-6. doi:10.1016/j.jaad.2009.07.014
J AM ACAD DERMATOL JUNE 2010
Histopathologic study of the lesion showed a diffuse interstitial infiltrate with strands of neoplastic cells involving the entire dermis and extending into subcutaneous tissue with an overlying ulcerated epidermis (Fig 2, A). Cytologically, the infiltrate was composed of large cells with central to eccentrically placed, mildly pleomorphic nuclei with prominent nucleoli, and granular eosinophilic cytoplasm, consistent with plasmablasts (Fig 2, B and C ). The cells strongly expressed CD79a, CD138 (Fig 2, D), and epithelial membrane antigen by immunohistochemistry, and were negative for S-100 and Melan-A. There was monoclonal expression of kappa light chains and IgA. This was consistent with the patient’s serum electrophoresis results, supporting the neoplastic nature of the dermal plasma cells. The diagnosis of a cutaneous involvement of MM was established and the patient was referred to the oncology department. The patient died 6 months later. The largest series of cutaneous involvement in MM published by Requena et al3 in 2005 establishes the main characteristics of this rare phenomenon. It occurs most frequently in middle age to older males (range, 36-81 years) with IgG and IgA MM. Typically, skin lesions appear as multiple, violaceous, smooth papules and/or nodules located on the trunk, abdomen and, in lesser frequency, on the scalp, face, neck, and extremities. Ulceration and secondary infection are exceptional findings. Differential diagnosis in the reported case included those entities which could be present as ulcerated toe lesions, including acral melanoma, pyogenic granuloma, Kaposi sarcoma, and traumatic ulcer. Histopathologic analysis shows two patterns— nodular and diffuse interstitial types—with the former being the most prevalent. The neoplastic dermal infiltrate is often composed of immature plasmablasts that lack the typical cytologic features of plasma cells; imunohistochemical confirmation is therefore required to establish the diagnosis. Cutaneous involvement by plasma cell neoplasms usually heralds a poor prognosis regardless of the therapy used.2,3 In summary, we describe a well documented case of cutaneous MM mimicking an acral melanoma. Clinicopathologic correlation was essential to come to the correct diagnosis in this unusual myeloma presentation. The authors would like to thank Nicole Balmer, MD, of the Department of Pathology at Wake Forest University Baptist Medical Center, Winston-Salem, NC.
Antonio Martorell, MD,a Fernando Millan-Parrilla, MD,b and Enrique Gimeno-Carpio, MDb
Departments of Dermatology, Instituto Valenciano de Oncologı´aa and Hospital Arnau de Vilanova,b Valencia, Spain. Funding sources: None. Conflicts of interest: None declared. Correspondence to: Antonio Martorell, MD, Department of Dermatology, Instituto Valenciano Oncologı´a, San Jose de la Montan ˜ a St, 14, 46008, Valencia, Spain. E-mail: [email protected] REFERENCES 1. Patterson JW, Parson JM, White RM, Fitzpatrick JE, Kohout-Dutz E. Cutaneous involvement of multiple myeloma and extramedullary plasmacytoma. J Am Acad Dermatol 1988;19(5 pt 2):879-90. 2. Requena L, Kutzner H, Palmedo G, Calonje E, Requena C, Pe´rez G, et al. Cutaneous involvement in multiple myeloma: a clinicopathologic, immunohistochemical, and cytogenetic study of 8 cases. Arch Dermatol 2003;139:475-86. 3. Requena L. Specific cutaneous involvement in patients with multiple myeloma. A clinicopathological, immunohistochemical and cytogenetic study of 40 cases [in Spanish]. Actas Dermosifiliogr 2005;96:424-40. doi:10.1016/j.jaad.2009.07.012
Papular amyloidosis limited to the ears To the Editor: A 42-year-old woman presented with a greater than 10-year history of asymptomatic brown papules involving her external ears. The lesions had slowly increased in size and number. She denied antecedent trauma and other skin disease. The physical examination revealed multiple 1- to 2mm smooth, firm, brown papules involving the conchal bowls, crura of the antihelices, and external auditory canals bilaterally (Fig 1). There was no obstruction of the canals on otoscopic examination. The remainder of the full skin examination was normal. A histopathologic examination of the biopsy specimen revealed hyperkeratosis, elongation of the rete ridges, and collections of amorphous, faintly eosinophilic material within expanded dermal papillae (Fig 2). There were scattered melanophages within the superficial dermis. The material stained positively with Congo red and periodic acideSchiff stains; this is a characteristic of amyloids. The amyloidoses are a diverse group of disorders that have in common the deposition of various proteins with similar staining characteristics. The cutaneous amyloidoses may be classified as primary, secondary, or associated with systemic amyloidosis. Auricular amyloidosis is a rare form of primary cutaneous amyloidosis. The primary cutaneous amyloidoses include lichen, macular, and nodular types. Macular and
Letters 1079
J AM ACAD DERMATOL VOLUME 62, NUMBER 6
Fig 1. Multiple waxy brown papules involving the conchal bowls, crura of the antihelices, and external auditory canals bilaterally.
lesions elsewhere. Their patients had similar histopathology to ours, with nodular deposits of amyloid in the papillary dermis. Immunoperoxidase staining performed on one of their cases showed positive staining with the antikeratin antibody EKH4, confirming the keratinocyte origin of the amyloid. Previous authors had reported similar papules of the external ear under the title ‘‘collagenous papules of the external ear.’’2,3 Secondary localized cutaneous amyloidosis refers to the presence of amyloid in cutaneous neoplasms, such as seborrheic keratosis and basal cell carcinoma. The amyloid is usually keratinocyte-derived (AK). Cutaneous involvement is common in primary systemic amyloidosis, manifesting as waxy papules and plaques, often found on the face, scalp, neck, hands, and flexures. Hemorrhage into the lesions is common.4 The amyloid is derived from immunoglobulin light chains (AL) of the delta or kappa type. The lesions of amyloidosis of the auricular concha are asymptomatic, but may be of cosmetic concern to the patient. Because of the localized distribution and superficial pathology, treatment modalities such as curettage or excision may be considered. Ann G. Neff, MD,a Jill Buckthal McCuin, MD,b and Diya F. Mutasim, MDa Department of Dermatology,a University of Cincinnati College of Medicine, Cincinnati, Ohio, and Newnan Dermatology at Cowles Clinic,b Greensboro, Georgia Funding sources: None. Conflicts of interest: None declared.
Fig 2. Histologic examination of a biopsy specimen reveals hyperkeratosis, elongation of the rete ridges, and nodules of eosinophilic material that fill and expand dermal papillae. (Hematoxylineeosin stain.)
Correspondence to: Diya F. Mutasim, MD, Department of Dermatology, University of Cincinnati, 231 Albert Sabin Way, P.O. Box 670592, Cincinnati, OH 45267-0592 E-mail: [email protected]
lichen amyloidosis result from amyloid in the tips of the dermal papillae. The amyloid protein is derived from keratinocytes, and is referred to as amyloid K (AK) protein. Nodular amyloidosis presents with one or few waxy plaques or nodules, with diffuse infiltration of the dermis and subcutis by immunoglobulin light chainederived material, referred to as amyloid light chain (AL). Plasma cells, which may be monoclonal, are usually present and are the source of the amyloid deposition. Amyloidosis of the auricular concha is an uncommon variant of primary cutaneous amyloidosis, and was first reported by Hicks et al1 in 1988. The authors described four patients with papules of the auricle, acquired in adulthood, and unassociated with
REFERENCES 1. Hicks BC, Weber PJ, Hashimoto K, Ito K, Koreman DM. Primary cutaneous amyloidosis of the auricular concha. J Am Acad Dermatol 1988;18:19-25. 2. Sanchez JL. Collagenous papules on the aural conchae. Am J Dermatopathol 1983;5:231-3. 3. Dupre A, Lassere J, Bonafe JL, Viraben R, Gorguet B. Collagenous papules of the external ear. Micropapular hyalinosis of the external ear with transepithelial elimination. Relation to primary localized cutaneous amyloidosis [in French]. Ann Dermatol Venereol 1984;111:913-7. 4. Ratz JL, Bailin PL. Cutaneous amyloidosis. A case report of the tumefactive variant and a review of the spectrum of clinical presentations. J Am Acad Dermatol 1981;4:21-6. doi:10.1016/j.jaad.2009.07.014