J Cutan Pathol 2014: 41: 791–796 doi: 10.1111/cup.12378 John Wiley & Sons. Printed in Singapore
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Journal of Cutaneous Pathology
Systemic amyloidosis presenting with glans penis involvement Penile amyloidosis has been reported on many occasions in the literature, but all of these have been forms of primary cutaneous amyloidosis. Systemic amyloidosis presenting with a penile ulcer as the first manifestation has not previously been reported. We present two patients in whom an ulcer of the glans penis was the first complaint that led to a diagnosis of systemic amyloidosis. In both patients, lambda light chain type amyloid was showed immunohistochemically. Both patients presented with other manifestations of systemic amyloidosis, including nail dystrophy characterized by onycholisis, trachyonychia and onychoschizia.
Keywords: genital amyloidosis, penile amyloidosis, urethral amyloidosis Fernandez-Flores A, Castañón-González JA, Guerrero-Ramos B, Castro-Gaytan A, Saeb-Lima M. Systemic amyloidosis presenting with glans penis involvement. J Cutan Pathol 2014; 41: 791–796. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Reports of amyloidosis involving the glans penis are rare.1 – 13 All of these reports have so far described localized amyloidosis with no systemic involvement. In one case, the authors presented two localized amyloid lesions in the glans penis and one in the trachea, respectively,11 but no systemic amyloidosis was found on autopsy. Also, Lapan et al. presented a case of priapism in a man with systemic amyloidosis,14 but there was only involvement of the corpus cavernosum and of the corpus spongiosum without involvement of the glans. Herein, we report the first two cases in the literature (to the best of our knowledge) of systemic amyloidosis presenting with a penile ulcer as the first manifestation.
Angel Fernandez-Flores1 , Jorge A. Castañón-González2 , Brenda Guerrero-Ramos3 , Armando Castro-Gaytan4 and Marcela Saeb-Lima5 1
Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain, 2 Department of Internal Medicine, American British Cowdray Hospital, Mexico City, Mexico, 3 Department of Dermatology, Private Practice, Guadalajara, Mexico, 4 Department of Urology, American British Cowdray Hospital, Mexico City, Mexico, and 5 Dermatopathologist and Dermatologist, American British Cowdray Hospital, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico Marcela Saeb-Lima, MD, Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición “Salvador Zubirán”, Vasco de Quiroga 15, Sección XVI, Tlalpan, Mexico City, CP 14000, Mexico Tel./Fax: 01 55 548709000 ext. 2184 e-mail: [email protected] Accepted for publication July 29, 2014
Reports of patients Patient 1 A 70-year-old man came to the dermatology consultancy complaining of a painful penile ulcer. He had a history of type 2 diabetes mellitus, ischemic heart disease and subclinical hypothyroidism. Physical examination showed a penile ulcer measuring 3 cm in largest diameter (Fig. 1A). The patient also presented with fingernail dystrophy (Fig. 1B–D). Abdominal palpation revealed hepatosplenomegaly, confirmed by abdominal computed tomography that also showed ascitic effusion. A biopsy was obtained from the penile ulcer, as well as from the nail and from a minor
791
Fernandez-Flores et al. A
B
C
D
immunoglobulin chain (negative for kappa; Fig. 2F). Similar amyloid deposits were showed in the salivary glands of the lip biopsy (Fig. 3). The nail biopsy showed vascular wall deposits of amyloid in the onychodermis, which were more remarkable with immunohistochemical stains (Fig. 4). Serum and urine electrophoresis showed an M-spike in the gamma fraction with monoclonal immunoglobulin G (IgG) lambda and proteinuria (1.539 mg/dl) positive for monoclonal free lambda light chain. Bone marrow aspirate showed an increase in total lymphocyte count and 17% plasma cells. Bone marrow biopsy showed a kappa/lambda ratio of 1 : 2.
Fig. 1. Patient 1: the patient presented with a penile ulcer (A) and fingernail dystrophy (B and C). Dermoscopy of one of the nails showed transverse proximal leukonychia with trachyonychia and onychoschizia.
salivary gland of the lip. The penile biopsy showed an active ulcer with focal preservation of the glans epithelium (Fig. 2A,B). Underneath the ulcer, the deep dermal arteries showed marked thickening of the vessel walls because of amyloid hyaline deposits (Fig. 2C). The latter were positive with crystal violet (Fig. 2D) and with the immunohistochemical techniques for substance P (Fig. 2E) and lambda light
Patient 2 A 54-year-old man came to the dermatology consultancy complaining of a penile ulcer on the glans penis. Physical examination showed an ulcer on the glans measuring 2 cm in largest diameter (Fig. 5A). The patient also presented with onychodystrophy of all 10 nails (Fig. 5B) with onycholysis, trachyonychia, onychoschizia, thickened oral lips (Fig. 5C), macroglossia (Fig. 5D) and papules on both hands (Fig. 5E) and the preauricular areas (Fig. 5F). A biopsy of the penile ulcer was performed (Fig. 6A), showing an active chronic ulcer (Fig. 6B) underneath which dermal arteries with
A
B
C
D
E
F
Fig. 2. Penile biopsy from Patient 1. There was a chronic ulcer (A and B), underneath which thickened dermal arteries could be identified (C). The walls of the vessels were thickened because of hyaline amyloid deposits, which were marked with crystal violet stain (D), as well as with anti-P substance (E) and anti-lambda antibody (F).
792
Glans penis involvement in systemic amyloidosis A
B
C
D
Fig. 3. Biopsy of the salivary gland from Patient 1. The amyloid deposits were mainly located around the secretory part of the minor salivary glands (A and B), as well as in the walls of the blood vessels (C). Such deposits were positive for anti-lambda (D).
Fig. 4. Nail biopsy from the nailbed of Patient 1. Although the amyloid deposits were already identified with hematoxylin–eosin (A), immunohistochemical staining with anti-P substance (B) and anti-lambda (C) made the deposits more remarkable.
thick walls were noted (Fig. 6C,D). The deposits on the vessels were made of a hyaline substance, which was positive for crystal violet (Fig. 6E), Congo red (Fig. 6F), amyloid P and lambda light immunoglobulin chain (negative for kappa). In addition, biopsies of abdominal fat, labial mucosa and bone marrow were also
performed, showing deposits of amyloid in the subcutaneous abdominal tissue, around the minor salivary glands, and in the labial vessels. Bone marrow biopsy showed a CD138+ plasma cell myeloma with monoclonal expression of lambda immunoglobulin light chains.
793
Fernandez-Flores et al. A
E
B
C F
D
Fig. 5. Patient 2. The patient presented with a penile ulcer (A) and nail atrophy of all 10 nails (B). The oral lips appeared thickened (C) and he had macroglossia (D). Yellowish papules were identified on both hands (E) and on the preauricular areas (F).
A
D
B
Discussion Amyloidosis involving the glans penis is a rare condition.1 – 13 All cases described so far belong to primary amyloidosis (to the best of our knowledge; Table 1). Weitzner et al. reported a case with two localized amyloid lesions: one in the glans penis and one in the trachea.11 However, the patient died from sepsis and the autopsy did not demonstrate systemic involvement. Lapan et al. presented a case of priapism in a man with systemic amyloidosis involving the heart, liver, spleen, kidneys and small blood vessels in general.14 Nevertheless, in Lapan et al.’s case, there was involvement of the corpus cavernosum and corpus spongiosum with no involvement of the glans. Our two cases are the first in which systemic amyloidosis presented as a penile ulcer of the glans as the first manifestation. We have found 11 published cases of amyloidosis involving the penile glans (Table 1). All of them were associated with primary localized amyloidosis.2 – 4,6 – 8,11,13 There are three additional cases of penile amyloidosis involving not the glans but the shaft9,13 (one of these is Case 2 in the report by Merika et al.).3 Lesions varied from papules3 to plaques1 – 5,7,12 and nodules.3,6,8 In all cases, amyloid was investigated with Congo red, and in some cases, immunohistochemical studies were also used. Cytokeratins were only used in one study3 (with CK5 C
E
F
Fig. 6. Biopsy of the penile ulcer from Patient 2. Crystal violet staining (A). Chronic ulceration (B), underneath which the arteries show amyloid deposits (C and D, hematoxylin–eosin; E, crystal violet; F, Congo red.
794
Glans penis involvement in systemic amyloidosis Table 1. Cases of glans involvement by amyloidosis previously published in the literature Clinical presentation
Single/multiple lesions
Location of the amyloid
Balanic sulcus
Plaque
Single
No
Not specified
20
Glans
Plaque
Single
Dermis and hypodermis Dermis
No
Not specified
1970 1981
52 21
Nodule Nodule
Single Two lesions
Dermis Dermis
No No
Not specified Not specified
Srinivasan et al.8 Ritter et al.7
1992
32
Tip of the penis Tip of the penis and distal shaft of the penis Glans
Nodule
Single
Subcutis
No
AA
2003
67
Glans
Plaque and papules
Single plaque, multiple papules
Dermis and walls of the dermal vessels
Inconclusive
Dominguez Dominguez et al.6 Kawsar and Long5
2007
56
Glans
Nodule
Single
Not specified
Amyloid-P+, kappa−, lambda+ (weak), amyloid-A−, transthyretin− Yes, not specified
2007
54
Glans
Plaque
Single
No
Not specified
9
Muneer et al.4
2009
35
Ventral shaft and perimeatal area
Plaque
Two lesions
Amyloid-P+
Not specified
10
di Meo et al.2
2014
50
Glans
Plaque
Two lesions
Dermis and walls of the dermal vessels Dermis and walls of the dermal vessels Dermis and walls of the dermal vessels
Not specified
11
Merika et al.3 (Case 1)
2014
46
Glans and prepuce
Plaques and an hemorrhagic papule
Multiple
Amyloid-P+, kappa−, lambda−, amyloid-A−, transthyretin− Cytokeratin 5−
Case
Reference
Year
Age
1
Degos et al.1
1961
61
2
Friedmann and Duperrat12 Weitzner et al.11 Bodner et al.10
1967
3 4
5 6
7
8
Location
negativity). Two studies showed some degree of immunohistochemical positivity for immunoglobulin light chains (not otherwise specified6 or inconclusive7 ). In most cases, amyloid deposits were interspersed within the dermal connective tissue1 – 5,7,12,13 , as well as within the dermal vessels.1,2,4,5,7,13 In some cases, the deposits were also subcutaneous.1,8 Nail dystrophy has been reported in the literature as associated with systemic amyloidosis on several occasions.15 – 26 The most common clinical signs of amyloidosis are longitudinal striations of the nail plate, as well as increased nail brittleness and fragility.15 – 20 Other manifestations are partial anonychia,16 verrucous plaques,21 distal onycholysis,22 onychoschizia27
Dermis
Immunohistochemistry
Type of amyloid diagnosed
AL
Not specified
or subungual hemorrhage.22 In most cases, nails were not biopsied because the diagnosis was clinically obvious. Cases that were biopsied showed amyloid deposits in the dermis of the matrix18,23 , as well as in the walls of blood vessels.18 Vessel walls were the main location of amyloid deposits in the cases we studied. We have not found any previous dermoscopic description of systemic amyloidosis involving the nail. In a recent report, Chuang et al. described the dermoscopic features of 35 cases of primary cutaneous amyloidosis, but no involvement of the nail was seen in any of these cases.28 In conclusion, we present the first two cases in which systemic amyloidosis presented with glans penis involvement as the first manifestation.
References 1. Degos R, Cottenot F, Civatte J. Isolated amyloidosis of the glans penis. Bull Soc Fr Dermatol Syphiligr 1961; 68: 159. 2. di Meo N, Stinco G, De Marchi S, Trevisan G. Primary nodular amyloidosis of the glans penis. Br J Dermatol 2014. DOI: 10.1111/bjd.13078 [Epub ahead of print].
3. Merika EE, Darling M, Craig P, et al. Primary cutaneous amyloidosis of the glans penis. Two case reports and a review of the literature. Br J Dermatol 2014; 170: 730. 4. Muneer A, Ali I, Blick C, Turner G, Turner R. An unusual solitary lesion on the glans penis. Clin Exp Dermatol 2009; 34: 929.
5. Kawsar M, Long S. Localized amyloidosis of glans penis. Int J STD AIDS 2007; 18: 720. 6. Dominguez Dominguez M, Valero Puerta JA, Jimenez Leiro JF, Martinez Ruiz R,
795
Fernandez-Flores et al.
7.
8.
9.
10.
11.
12.
13.
14.
Medina PM. Primary localized amyloidosis of glans penis. A new case and review of the literature. Actas Urol Esp 2007; 31: 168. Ritter M, Nawab RA, Tannenbaum M, Hakky SI, Morgan MB. Localized amyloidosis of the glans penis: a case report and literature review. J Cutan Pathol 2003; 30: 37. Srinivasan V, Kehinde EO, Harris MD, Turner AG. Localized amyloid of the glans penis. Br J Surg 1992; 79: 1156. Leal SM, Novsam N, Zacks SI. Amyloidosis presenting as a penile mass. J Urol 1988; 140: 830. Bodner H, Retsky MI, Brown G. Primary amyloidosis of glans penis and urethra: resection and reconstruction. J Urol 1981; 125: 586. Weitzner S, Keen PE, Doughty WE. Primary localized amyloidosis of glans penis. Arch Dermatol 1970; 102: 463. Friedmann E, Duperrat MB. Isolated amyloidosis of the glans penis. Bull Soc Fr Dermatol Syphiligr 1967; 74: 313. Lim JH, Kim H. Localized amyloidosis presenting with a penile mass: a case report. Cases J 2009; 2: 160. Lapan DI, Graham AR, Bangert JL, Boyer JT, Conner WT. Amyloidosis presenting as priapism. Urology 1980; 15: 167.
796
15. Derrick EK, Price ML. Primary systemic amyloid with nail dystrophy. J R Soc Med 1995; 88: 290P. 16. Bedlow AJ, Sampson SA, Holden CA. Primary systemic amyloidosis of the hair and nails. Clin Exp Dermatol 1998; 23: 298. 17. Hoshino Y, Umezawa K, Machida M, et al. Nail dystrophy associated with AL amyloidosis. Intern Med 2007; 46: 525. 18. Prat C, Moreno A, Vinas M, Jucgla A. Nail dystrophy in primary systemic amyloidosis. J Eur Acad Dermatol Venereol 2008; 22: 107. 19. Pink AE, Stefanato CM, Breathnach SM. An unusual presentation of systemic AL amyloidosis: bullae, milia and nail dystrophy. Clin Exp Dermatol 2012; 37: 788. 20. Barja J, Pineyro F, Almagro M, et al. Systemic amyloidosis with an exceptional cutaneous presentation. Dermatol Online J 2013; 19: 11. 21. Tausend W, Neill M, Kelly B. Primary amyloidosis-induced nail dystrophy. Dermatol Online J 2014; 20: 21247. 22. Etienne M, Denizon N, Maillard H. Anomalies unguéales révélant une amylose systémique AL. Rev Med Interne 2014. DOI: 10.1016/j.revmed.2014.02.002 [Epub ahead of print].
23. Fanti PA, Tosti A, Morelli R, Galbiati G. Nail changes as the first sign of systemic amyloidosis. Dermatologica 1991; 183: 44. 24. Pineda MS, Herrero C, Palou J, Vilalta A, Mascaro JM. Nail alterations in systemic amyloidosis: report of one case, with histologic study. J Am Acad Dermatol 1988; 18: 1357. 25. Tidman MJ, Wells RS, MacDonald DM. Pachyonychia congenita with cutaneous amyloidosis and hyperpigmentation – a distinct variant. J Am Acad Dermatol 1987; 16: 935. 26. Breathnach SM, Wilkinson JD, Black MM. Systemic amyloidosis with an underlying lymphoproliferative disorder. Report of a case in which nail involvement was a presenting feature. Clin Exp Dermatol 1979; 4: 495. 27. Fujita Y, Tsuji-Abe Y, Sato-Matsumura KC, Akiyama M, Shimizu H. Nail dystrophy and blisters as sole manifestations in myeloma-associated amyloidosis. J Am Acad Dermatol 2006; 54: 712. 28. Chuang YY, Lee DD, et al. Characteristic dermoscopic features of primary cutaneous amyloidosis: a study of 35 cases. Br J Dermatol 2012; 167: 548.
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© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Journal of Cutaneous Pathology
Systemic amyloidosis presenting with glans penis involvement Penile amyloidosis has been reported on many occasions in the literature, but all of these have been forms of primary cutaneous amyloidosis. Systemic amyloidosis presenting with a penile ulcer as the first manifestation has not previously been reported. We present two patients in whom an ulcer of the glans penis was the first complaint that led to a diagnosis of systemic amyloidosis. In both patients, lambda light chain type amyloid was showed immunohistochemically. Both patients presented with other manifestations of systemic amyloidosis, including nail dystrophy characterized by onycholisis, trachyonychia and onychoschizia.
Keywords: genital amyloidosis, penile amyloidosis, urethral amyloidosis Fernandez-Flores A, Castañón-González JA, Guerrero-Ramos B, Castro-Gaytan A, Saeb-Lima M. Systemic amyloidosis presenting with glans penis involvement. J Cutan Pathol 2014; 41: 791–796. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Reports of amyloidosis involving the glans penis are rare.1 – 13 All of these reports have so far described localized amyloidosis with no systemic involvement. In one case, the authors presented two localized amyloid lesions in the glans penis and one in the trachea, respectively,11 but no systemic amyloidosis was found on autopsy. Also, Lapan et al. presented a case of priapism in a man with systemic amyloidosis,14 but there was only involvement of the corpus cavernosum and of the corpus spongiosum without involvement of the glans. Herein, we report the first two cases in the literature (to the best of our knowledge) of systemic amyloidosis presenting with a penile ulcer as the first manifestation.
Angel Fernandez-Flores1 , Jorge A. Castañón-González2 , Brenda Guerrero-Ramos3 , Armando Castro-Gaytan4 and Marcela Saeb-Lima5 1
Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain, 2 Department of Internal Medicine, American British Cowdray Hospital, Mexico City, Mexico, 3 Department of Dermatology, Private Practice, Guadalajara, Mexico, 4 Department of Urology, American British Cowdray Hospital, Mexico City, Mexico, and 5 Dermatopathologist and Dermatologist, American British Cowdray Hospital, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico Marcela Saeb-Lima, MD, Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición “Salvador Zubirán”, Vasco de Quiroga 15, Sección XVI, Tlalpan, Mexico City, CP 14000, Mexico Tel./Fax: 01 55 548709000 ext. 2184 e-mail: [email protected] Accepted for publication July 29, 2014
Reports of patients Patient 1 A 70-year-old man came to the dermatology consultancy complaining of a painful penile ulcer. He had a history of type 2 diabetes mellitus, ischemic heart disease and subclinical hypothyroidism. Physical examination showed a penile ulcer measuring 3 cm in largest diameter (Fig. 1A). The patient also presented with fingernail dystrophy (Fig. 1B–D). Abdominal palpation revealed hepatosplenomegaly, confirmed by abdominal computed tomography that also showed ascitic effusion. A biopsy was obtained from the penile ulcer, as well as from the nail and from a minor
791
Fernandez-Flores et al. A
B
C
D
immunoglobulin chain (negative for kappa; Fig. 2F). Similar amyloid deposits were showed in the salivary glands of the lip biopsy (Fig. 3). The nail biopsy showed vascular wall deposits of amyloid in the onychodermis, which were more remarkable with immunohistochemical stains (Fig. 4). Serum and urine electrophoresis showed an M-spike in the gamma fraction with monoclonal immunoglobulin G (IgG) lambda and proteinuria (1.539 mg/dl) positive for monoclonal free lambda light chain. Bone marrow aspirate showed an increase in total lymphocyte count and 17% plasma cells. Bone marrow biopsy showed a kappa/lambda ratio of 1 : 2.
Fig. 1. Patient 1: the patient presented with a penile ulcer (A) and fingernail dystrophy (B and C). Dermoscopy of one of the nails showed transverse proximal leukonychia with trachyonychia and onychoschizia.
salivary gland of the lip. The penile biopsy showed an active ulcer with focal preservation of the glans epithelium (Fig. 2A,B). Underneath the ulcer, the deep dermal arteries showed marked thickening of the vessel walls because of amyloid hyaline deposits (Fig. 2C). The latter were positive with crystal violet (Fig. 2D) and with the immunohistochemical techniques for substance P (Fig. 2E) and lambda light
Patient 2 A 54-year-old man came to the dermatology consultancy complaining of a penile ulcer on the glans penis. Physical examination showed an ulcer on the glans measuring 2 cm in largest diameter (Fig. 5A). The patient also presented with onychodystrophy of all 10 nails (Fig. 5B) with onycholysis, trachyonychia, onychoschizia, thickened oral lips (Fig. 5C), macroglossia (Fig. 5D) and papules on both hands (Fig. 5E) and the preauricular areas (Fig. 5F). A biopsy of the penile ulcer was performed (Fig. 6A), showing an active chronic ulcer (Fig. 6B) underneath which dermal arteries with
A
B
C
D
E
F
Fig. 2. Penile biopsy from Patient 1. There was a chronic ulcer (A and B), underneath which thickened dermal arteries could be identified (C). The walls of the vessels were thickened because of hyaline amyloid deposits, which were marked with crystal violet stain (D), as well as with anti-P substance (E) and anti-lambda antibody (F).
792
Glans penis involvement in systemic amyloidosis A
B
C
D
Fig. 3. Biopsy of the salivary gland from Patient 1. The amyloid deposits were mainly located around the secretory part of the minor salivary glands (A and B), as well as in the walls of the blood vessels (C). Such deposits were positive for anti-lambda (D).
Fig. 4. Nail biopsy from the nailbed of Patient 1. Although the amyloid deposits were already identified with hematoxylin–eosin (A), immunohistochemical staining with anti-P substance (B) and anti-lambda (C) made the deposits more remarkable.
thick walls were noted (Fig. 6C,D). The deposits on the vessels were made of a hyaline substance, which was positive for crystal violet (Fig. 6E), Congo red (Fig. 6F), amyloid P and lambda light immunoglobulin chain (negative for kappa). In addition, biopsies of abdominal fat, labial mucosa and bone marrow were also
performed, showing deposits of amyloid in the subcutaneous abdominal tissue, around the minor salivary glands, and in the labial vessels. Bone marrow biopsy showed a CD138+ plasma cell myeloma with monoclonal expression of lambda immunoglobulin light chains.
793
Fernandez-Flores et al. A
E
B
C F
D
Fig. 5. Patient 2. The patient presented with a penile ulcer (A) and nail atrophy of all 10 nails (B). The oral lips appeared thickened (C) and he had macroglossia (D). Yellowish papules were identified on both hands (E) and on the preauricular areas (F).
A
D
B
Discussion Amyloidosis involving the glans penis is a rare condition.1 – 13 All cases described so far belong to primary amyloidosis (to the best of our knowledge; Table 1). Weitzner et al. reported a case with two localized amyloid lesions: one in the glans penis and one in the trachea.11 However, the patient died from sepsis and the autopsy did not demonstrate systemic involvement. Lapan et al. presented a case of priapism in a man with systemic amyloidosis involving the heart, liver, spleen, kidneys and small blood vessels in general.14 Nevertheless, in Lapan et al.’s case, there was involvement of the corpus cavernosum and corpus spongiosum with no involvement of the glans. Our two cases are the first in which systemic amyloidosis presented as a penile ulcer of the glans as the first manifestation. We have found 11 published cases of amyloidosis involving the penile glans (Table 1). All of them were associated with primary localized amyloidosis.2 – 4,6 – 8,11,13 There are three additional cases of penile amyloidosis involving not the glans but the shaft9,13 (one of these is Case 2 in the report by Merika et al.).3 Lesions varied from papules3 to plaques1 – 5,7,12 and nodules.3,6,8 In all cases, amyloid was investigated with Congo red, and in some cases, immunohistochemical studies were also used. Cytokeratins were only used in one study3 (with CK5 C
E
F
Fig. 6. Biopsy of the penile ulcer from Patient 2. Crystal violet staining (A). Chronic ulceration (B), underneath which the arteries show amyloid deposits (C and D, hematoxylin–eosin; E, crystal violet; F, Congo red.
794
Glans penis involvement in systemic amyloidosis Table 1. Cases of glans involvement by amyloidosis previously published in the literature Clinical presentation
Single/multiple lesions
Location of the amyloid
Balanic sulcus
Plaque
Single
No
Not specified
20
Glans
Plaque
Single
Dermis and hypodermis Dermis
No
Not specified
1970 1981
52 21
Nodule Nodule
Single Two lesions
Dermis Dermis
No No
Not specified Not specified
Srinivasan et al.8 Ritter et al.7
1992
32
Tip of the penis Tip of the penis and distal shaft of the penis Glans
Nodule
Single
Subcutis
No
AA
2003
67
Glans
Plaque and papules
Single plaque, multiple papules
Dermis and walls of the dermal vessels
Inconclusive
Dominguez Dominguez et al.6 Kawsar and Long5
2007
56
Glans
Nodule
Single
Not specified
Amyloid-P+, kappa−, lambda+ (weak), amyloid-A−, transthyretin− Yes, not specified
2007
54
Glans
Plaque
Single
No
Not specified
9
Muneer et al.4
2009
35
Ventral shaft and perimeatal area
Plaque
Two lesions
Amyloid-P+
Not specified
10
di Meo et al.2
2014
50
Glans
Plaque
Two lesions
Dermis and walls of the dermal vessels Dermis and walls of the dermal vessels Dermis and walls of the dermal vessels
Not specified
11
Merika et al.3 (Case 1)
2014
46
Glans and prepuce
Plaques and an hemorrhagic papule
Multiple
Amyloid-P+, kappa−, lambda−, amyloid-A−, transthyretin− Cytokeratin 5−
Case
Reference
Year
Age
1
Degos et al.1
1961
61
2
Friedmann and Duperrat12 Weitzner et al.11 Bodner et al.10
1967
3 4
5 6
7
8
Location
negativity). Two studies showed some degree of immunohistochemical positivity for immunoglobulin light chains (not otherwise specified6 or inconclusive7 ). In most cases, amyloid deposits were interspersed within the dermal connective tissue1 – 5,7,12,13 , as well as within the dermal vessels.1,2,4,5,7,13 In some cases, the deposits were also subcutaneous.1,8 Nail dystrophy has been reported in the literature as associated with systemic amyloidosis on several occasions.15 – 26 The most common clinical signs of amyloidosis are longitudinal striations of the nail plate, as well as increased nail brittleness and fragility.15 – 20 Other manifestations are partial anonychia,16 verrucous plaques,21 distal onycholysis,22 onychoschizia27
Dermis
Immunohistochemistry
Type of amyloid diagnosed
AL
Not specified
or subungual hemorrhage.22 In most cases, nails were not biopsied because the diagnosis was clinically obvious. Cases that were biopsied showed amyloid deposits in the dermis of the matrix18,23 , as well as in the walls of blood vessels.18 Vessel walls were the main location of amyloid deposits in the cases we studied. We have not found any previous dermoscopic description of systemic amyloidosis involving the nail. In a recent report, Chuang et al. described the dermoscopic features of 35 cases of primary cutaneous amyloidosis, but no involvement of the nail was seen in any of these cases.28 In conclusion, we present the first two cases in which systemic amyloidosis presented with glans penis involvement as the first manifestation.
References 1. Degos R, Cottenot F, Civatte J. Isolated amyloidosis of the glans penis. Bull Soc Fr Dermatol Syphiligr 1961; 68: 159. 2. di Meo N, Stinco G, De Marchi S, Trevisan G. Primary nodular amyloidosis of the glans penis. Br J Dermatol 2014. DOI: 10.1111/bjd.13078 [Epub ahead of print].
3. Merika EE, Darling M, Craig P, et al. Primary cutaneous amyloidosis of the glans penis. Two case reports and a review of the literature. Br J Dermatol 2014; 170: 730. 4. Muneer A, Ali I, Blick C, Turner G, Turner R. An unusual solitary lesion on the glans penis. Clin Exp Dermatol 2009; 34: 929.
5. Kawsar M, Long S. Localized amyloidosis of glans penis. Int J STD AIDS 2007; 18: 720. 6. Dominguez Dominguez M, Valero Puerta JA, Jimenez Leiro JF, Martinez Ruiz R,
795
Fernandez-Flores et al.
7.
8.
9.
10.
11.
12.
13.
14.
Medina PM. Primary localized amyloidosis of glans penis. A new case and review of the literature. Actas Urol Esp 2007; 31: 168. Ritter M, Nawab RA, Tannenbaum M, Hakky SI, Morgan MB. Localized amyloidosis of the glans penis: a case report and literature review. J Cutan Pathol 2003; 30: 37. Srinivasan V, Kehinde EO, Harris MD, Turner AG. Localized amyloid of the glans penis. Br J Surg 1992; 79: 1156. Leal SM, Novsam N, Zacks SI. Amyloidosis presenting as a penile mass. J Urol 1988; 140: 830. Bodner H, Retsky MI, Brown G. Primary amyloidosis of glans penis and urethra: resection and reconstruction. J Urol 1981; 125: 586. Weitzner S, Keen PE, Doughty WE. Primary localized amyloidosis of glans penis. Arch Dermatol 1970; 102: 463. Friedmann E, Duperrat MB. Isolated amyloidosis of the glans penis. Bull Soc Fr Dermatol Syphiligr 1967; 74: 313. Lim JH, Kim H. Localized amyloidosis presenting with a penile mass: a case report. Cases J 2009; 2: 160. Lapan DI, Graham AR, Bangert JL, Boyer JT, Conner WT. Amyloidosis presenting as priapism. Urology 1980; 15: 167.
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