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Solitary Papule on the Nose
Author(s)
Qiong Wu, MD
Daniel C. Skipper, DO
Dirk M. Elston, MD

The Diagnosis: Sclerosing Perineurioma 

Sclerosing perineurioma, first described in 1997 by Fetsch and Miettinen,1 is a subtype of perineurioma with a strong predilection for the fingers and palms of young adults. Rare cases involving extra-acral sites including the forearm, elbow, axilla, back, neck, lower leg, thigh, knee, lips, nose, and mouth have been reported.2-4 Perineurioma is a relatively uncommon and benign peripheral nerve sheath tumor with exclusive perineurial differentiation.5 Perineurioma is divided into intraneural and extraneural types; the latter are further subclassified into soft tissue, sclerosing, reticular, and plexiform types. Other rare forms include the sclerosing, Pacinian corpuscle-like perineurioma, lipomatous perineurioma, perineurioma with xanthomatous areas, and perineurioma with granular cells.6,7  

Clinically, sclerosing perineurioma usually presents as a solitary lesion; however, rare cases of multiple lesions have been reported.8 Our patient presented with a solitary papule on the nose. Histopathologically, sclerosing perineurioma demonstrates slender spindle cells in a whorled growth pattern (onion skin) embedded in a hyalinized, lamellar, and dense collagenous stroma with intervening cleftlike spaces. Immunohistochemically, the spindle cells of our case stained positive for epithelial membrane antigen (quiz images). Other positive immunostains for perineurioma include claudin-1 and glucose transporter 1 (GLUT1). Perineurioma lacks expression of S-100 but can express CD34.2 As a benign tumor, the prognosis of sclerosing perineurioma is excellent. Complete local excision is considered curative.1  

Angiofibroma, also known as fibrous papule, is a common and benign lesion located primarily on or in close proximity to the nose.9 Angiofibromas can be associated with genodermatoses such as tuberous sclerosis, multiple endocrine neoplasia type 1, or Birt-Hogg-Dubé syndrome. When angiofibromas involve the penis, they are called pearly penile papules. Ungual angiofibroma, also known as Koenen tumor, occurs underneath the nail.10-12 Both facial angiofibromas (>3) and ungual angiofibromas (>2) are independent major criteria for tuberous sclerosis.13 Clinically, angiofibroma presents as a small, dome-shaped, pink papule arising on the lower portion of the nose or nearby area of the central face. Histopathologically, angiofibromas classically demonstrate increased dilated vessels and fibrosis in the dermis. Stellate, plump, spindle-shaped, and multinucleated cells can be seen in the collagenous stroma. The collagen fibers around hair follicles are arranged concentrically, resulting in an onion skin-like appearance. The epidermal rete ridges can be effaced (Figure 1). Increased numbers of single-unit melanocytes along the dermoepidermal junction can be seen in some cases. Immunohistochemically, a variable number of spindled and multinucleated cells in the dermis stain with factor XIIIa. There are at least 7 histologic variants of angiofibroma including hypercellular, pigmented, inflammatory, pleomorphic, clear cell, granular cell, and epithelioid.9,14 

Figure 1. Fibrous papule. Increased dilated vessels and fibrosis in the dermis with an onion skin–like appearance around hair follicles. Scattered stellate, spindled, and multinucleated cells can be seen (H&E, original magnification ×100).


Desmoplastic nevus (DN) is a benign melanocytic neoplasm characterized by predominantly spindle-shaped nevus cells embedded within a fibrotic stroma. Although it can resemble a Spitz nevus, it is recognized as a distinct entity.15-17 Clinically, DN presents as a small and flesh-colored, erythematous or slightly pigmented papule or nodule that usually occurs on the arms and legs of young adults. Histopathologically, DN demonstrates a dermal-based proliferation of spindled melanocytes embedded in a dense collagenous stroma with sparse or absent melanin deposition. The collagen bundles often show artifactual clefts and onion skin-like accentuation around vessels. Melanocytes may be epithelioid (Figure 2).16 Immunohistochemically, DN expresses melanocytic markers such as S-100, Melan-A, and human melanoma black 45, but epithelial membrane antigen is negative. Human melanoma black 45 demonstrates maturation with stronger staining in superficial portions of the lesion and diminution of staining with increasing dermal depth.18 Many other melanocytic tumors share histologic similarities to DN including blue nevus and desmoplastic melanoma.17,19,20  

Figure 2. Desmoplastic nevus. A proliferation of spindled and epithelioid melanocytes embedded in a dense collagenous stroma. Clefts within sclerotic collagen bundles can be seen (H&E, original magnification ×100).

Palisaded encapsulated neuroma, also referred to as solitary circumscribed neuroma, was first described by Reed et al21 in 1972. It is a benign and solitary, firm, dome-shaped, flesh-colored papule that occurs in middle-aged adults, predominately near mucocutaneous junctions of the face. Other locations include the oral mucosa, eyelid, nasal fossa, shoulder, arm, hand, foot, and glans penis.22,23 Histopathologically, palisaded encapsulated neuroma demonstrates a solitary, well-circumscribed, partially encapsulated, intradermal nodule composed of interweaving fascicles of spindle cells with prominent clefts (Figure 3). Rarely, palisaded encapsulated neuroma may have a plexiform or multinodular architecture.24 Immunohistochemically, tumor cells stain positively for S-100 protein, type IV collagen, and vimentin. The capsule, composed of perineural cells, stains positive for epithelial membrane antigen. A neurofilament stain will highlight axons within the tumor.24,25 Currently, palisaded encapsulated neuroma does not have a well-established link to known neurocutaneous or inherited syndromes. Excision is curative with a low risk of recurrence.26 

Figure 3. Palisaded encapsulated neuroma. A partially encapsulated nodule composed of interweaving fascicles of spindle cells with prominent clefts can be seen (H&E, original magnification ×100).

Sclerotic fibromas (SFs) were first reported by Weary et al27 as multiple tumors involving the tongues of patients with Cowden syndrome. Sporadic or solitary SFs of the skin in patients without Cowden syndrome have been reported, and both multiple and solitary SFs present with similar pathologic changes.28-30 Clinically, the solitary variant manifests as a well-demarcated, flesh-colored to erythematous, waxy papule or nodule with no site or sex predilection.30,31 Histologically, SF demonstrates a well-demarcated, nonencapsulated dermal nodule composed of hypocellular and sclerotic collagen bundles with scattered spindled cells and prominent clefts resembling Vincent van Gogh's Starry Night or plywood (Figure 4). Immunohistochemically, the spindled cells strongly express CD34. Factor XIIIa and markers of melanocytic, neural, and muscular differentiation are negative. When rendering a diagnosis in a patient with multiple SFs, a comment regarding the possibility of Cowden syndrome should be mentioned.32 

Figure 4. Sclerotic fibroma. Sclerotic collagen bundles with scattered spindled cells and clefts resembling Vincent van Gogh’s Starry Night (H&E, original magnification ×100).

References
  1. Fetsch JF, Miettinen M. Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults. Am J Surg Pathol. 1997;21:1433-1442. 
  2. Fox MD, Gleason BC, Thomas AB, et al. Extra-acral cutaneous/soft tissue sclerosing perineurioma: an under-recognized entity in the differential of CD34-positive cutaneous neoplasms. J Cutan Pathol. 2010;37:1053-1056. 
  3. Erstine EM, Ko JS, Rubin BP, et al. Broadening the anatomic landscape of sclerosing perineurioma: a series of 5 cases in nonacral sites. Am J Dermatopathol. 2017;39:679-681. 
  4. Senghore N, Cunliffe D, Watt-Smith S, et al. Extraneural perineurioma of the face: an unusual cutaneous presentation of an uncommon tumour. Br J Oral Maxillofac Surg. 2001;39:315-319. 
  5. Lazarus SS, Trombetta LD. Ultrastructural identification of a benign perineurial cell tumor. Cancer. 1978;41:1823-1829. 
  6. Macarenco RS, Cury-Martins J. Extra-acral cutaneous sclerosing perineurioma with CD34 fingerprint pattern. J Cutan Pathol. 2017;44:388-392. 
  7. Santos-Briz A, Godoy E, Canueto J, et al. Cutaneous intraneural perineurioma: a case report. Am J Dermatopathol. 2013;35:E45-E48. 
  8. Rubin AI, Yassaee M, Johnson W, et al. Multiple cutaneous sclerosing perineuriomas: an extensive presentation with involvement of the bilateral upper extremities. J Cutan Pathol. 2009;36(suppl 1):60-65. 
  9. Damman J, Biswas A. Fibrous papule: a histopathologic review. Am J Dermatopathol. 2018;40:551-560. 
  10. Macri A, Tanner LS. Cutaneous angiofibroma. StatPearls. https://www.statpearls.com/kb/viewarticle/17566/. Updated January 24, 2019. Accessed October 21, 2019.  
  11. Darling TN, Skarulis MC, Steinberg SM, et al. Multiple facial angiofibromas and collagenomas in patients with multiple endocrine neoplasia type 1. Arch Dermatol. 1997;133:853-857. 
  12. Schaffer JV, Gohara MA, McNiff JM, et al. Multiple facial angiofibromas: a cutaneous manifestation of Birt-Hogg-Dube syndrome. J Am Acad Dermatol. 2005;53:S108-S111. 
  13. Northrup H, Krueger DA; International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol. 2013;49:243-254. 
  14. Bansal C, Stewart D, Li A, et al. Histologic variants of fibrous papule. J Cutan Pathol. 2005;32:424-428. 
  15. Harris GR, Shea CR, Horenstein MG, et al. Desmoplastic (sclerotic) nevus: an underrecognized entity that resembles dermatofibroma and desmoplastic melanoma. Am J Surg Pathol. 1999;23:786-794. 
  16. Ferrara G, Brasiello M, Annese P, et al. Desmoplastic nevus: clinicopathologic keynotes. Am J Dermatopathol. 2009;31:718-722. 
  17. Sherrill AM, Crespo G, Prakash AV, et al. Desmoplastic nevus: an entity distinct from Spitz nevus and blue nevus. Am J Dermatopathol. 2011;33:35-39. 
  18. Kucher C, Zhang PJ, Pasha T, et al. Expression of Melan-A and Ki-67 in desmoplastic melanoma and desmoplastic nevi. Am J Dermatopathol. 2004;26:452-457. 
  19. Sidiropoulos M, Sholl LM, Obregon R, et al. Desmoplastic nevus of chronically sun-damaged skin: an entity to be distinguished from desmoplastic melanoma. Am J Dermatopathol. 2014;36:629-634. 
  20. Kiuru M, Patel RM, Busam KJ. Desmoplastic melanocytic nevi with lymphocytic aggregates. J Cutan Pathol. 2012;39:940-944. 
  21. Reed RJ, Fine RM, Meltzer HD. Palisaded, encapsulated neuromas of the skin. Arch Dermatol. 1972;106:865-870. 
  22. Newman MD, Milgraum S. Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. Dermatol Online J. 2008;14:12. 
  23. Beutler B, Cohen PR. Palisaded encapsulated neuroma of the trunk: a case report and review of palisaded encapsulated neuroma. Cureus. 2016;8:E726. 
  24. Jokinen CH, Ragsdale BD, Argenyi ZB. Expanding the clinicopathologic spectrum of palisaded encapsulated neuroma. J Cutan Pathol. 2010;37:43-48. 
  25. Argenyi ZB. Immunohistochemical characterization of palisaded, encapsulated neuroma. J Cutan Pathol. 1990;17:329-335. 
  26. Batra J, Ramesh V, Molpariya A, et al. Palisaded encapsulated neuroma: an unusual presentation. Indian Dermatol Online J. 2018;9:262-264. 
  27.  Weary PE, Gorlin RJ, Gentry WC Jr, et al. Multiple hamartoma syndrome (Cowden's disease). Arch Dermatol. 1972;106:682-690. 
  28. Mahmood MN, Salama ME, Chaffins M, et al. Solitary sclerotic fibroma of skin: a possible link with pleomorphic fibroma with immunophenotypic expression for O13 (CD99) and CD34. J Cutan Pathol. 2003;30:631-636. 
  29. Nakashima K, Yamada N, Adachi K, et al. Solitary sclerotic fibroma of the skin: morphological characterization of the 'plywood-like pattern'. J Cutan Pathol. 2008;35(suppl 1):74-79. 
  30. Rapini RP, Golitz LE. Sclerotic fibromas of the skin. J Am Acad Dermatol. 1989;20:266-271. 
  31. Abbas O, Ghosn S, Bahhady R, et al. Solitary sclerotic fibroma on the scalp of a young girl: reactive sclerosis pattern? J Dermatol. 2010;37:575-577. 
  32. Hanft VN, Shea CR, McNutt NS, et al. Expression of CD34 in sclerotic ("plywood") fibromas. Am J Dermatopathol. 2000;22:17-21.
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Author and Disclosure Information

Dr. Wu is from the Department of Dermatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, China. Drs. Wu and Elston are from the Department of Dermatology and Dermatologic Surgery, and Dr. Skipper is from the Department of Pathology and Laboratory Medicine, all at the Medical University of South Carolina, Charleston.

The authors report no conflict of interest.

Correspondence: Dirk M. Elston, MD, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, 135 Rutledge Ave, MSC 578, Charleston, SC 29425 ([email protected]).

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Cutis - 104(5)
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Cutis
Topics
Dermatopathology
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268, 292-294
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Author(s)
Qiong Wu, MD
Daniel C. Skipper, DO
Dirk M. Elston, MD
Author(s)
Qiong Wu, MD
Daniel C. Skipper, DO
Dirk M. Elston, MD
Author and Disclosure Information

Dr. Wu is from the Department of Dermatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, China. Drs. Wu and Elston are from the Department of Dermatology and Dermatologic Surgery, and Dr. Skipper is from the Department of Pathology and Laboratory Medicine, all at the Medical University of South Carolina, Charleston.

The authors report no conflict of interest.

Correspondence: Dirk M. Elston, MD, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, 135 Rutledge Ave, MSC 578, Charleston, SC 29425 ([email protected]).

Author and Disclosure Information

Dr. Wu is from the Department of Dermatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, China. Drs. Wu and Elston are from the Department of Dermatology and Dermatologic Surgery, and Dr. Skipper is from the Department of Pathology and Laboratory Medicine, all at the Medical University of South Carolina, Charleston.

The authors report no conflict of interest.

Correspondence: Dirk M. Elston, MD, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, 135 Rutledge Ave, MSC 578, Charleston, SC 29425 ([email protected]).

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The Diagnosis: Sclerosing Perineurioma 

Sclerosing perineurioma, first described in 1997 by Fetsch and Miettinen,1 is a subtype of perineurioma with a strong predilection for the fingers and palms of young adults. Rare cases involving extra-acral sites including the forearm, elbow, axilla, back, neck, lower leg, thigh, knee, lips, nose, and mouth have been reported.2-4 Perineurioma is a relatively uncommon and benign peripheral nerve sheath tumor with exclusive perineurial differentiation.5 Perineurioma is divided into intraneural and extraneural types; the latter are further subclassified into soft tissue, sclerosing, reticular, and plexiform types. Other rare forms include the sclerosing, Pacinian corpuscle-like perineurioma, lipomatous perineurioma, perineurioma with xanthomatous areas, and perineurioma with granular cells.6,7  

Clinically, sclerosing perineurioma usually presents as a solitary lesion; however, rare cases of multiple lesions have been reported.8 Our patient presented with a solitary papule on the nose. Histopathologically, sclerosing perineurioma demonstrates slender spindle cells in a whorled growth pattern (onion skin) embedded in a hyalinized, lamellar, and dense collagenous stroma with intervening cleftlike spaces. Immunohistochemically, the spindle cells of our case stained positive for epithelial membrane antigen (quiz images). Other positive immunostains for perineurioma include claudin-1 and glucose transporter 1 (GLUT1). Perineurioma lacks expression of S-100 but can express CD34.2 As a benign tumor, the prognosis of sclerosing perineurioma is excellent. Complete local excision is considered curative.1  

Angiofibroma, also known as fibrous papule, is a common and benign lesion located primarily on or in close proximity to the nose.9 Angiofibromas can be associated with genodermatoses such as tuberous sclerosis, multiple endocrine neoplasia type 1, or Birt-Hogg-Dubé syndrome. When angiofibromas involve the penis, they are called pearly penile papules. Ungual angiofibroma, also known as Koenen tumor, occurs underneath the nail.10-12 Both facial angiofibromas (>3) and ungual angiofibromas (>2) are independent major criteria for tuberous sclerosis.13 Clinically, angiofibroma presents as a small, dome-shaped, pink papule arising on the lower portion of the nose or nearby area of the central face. Histopathologically, angiofibromas classically demonstrate increased dilated vessels and fibrosis in the dermis. Stellate, plump, spindle-shaped, and multinucleated cells can be seen in the collagenous stroma. The collagen fibers around hair follicles are arranged concentrically, resulting in an onion skin-like appearance. The epidermal rete ridges can be effaced (Figure 1). Increased numbers of single-unit melanocytes along the dermoepidermal junction can be seen in some cases. Immunohistochemically, a variable number of spindled and multinucleated cells in the dermis stain with factor XIIIa. There are at least 7 histologic variants of angiofibroma including hypercellular, pigmented, inflammatory, pleomorphic, clear cell, granular cell, and epithelioid.9,14 

Figure 1. Fibrous papule. Increased dilated vessels and fibrosis in the dermis with an onion skin–like appearance around hair follicles. Scattered stellate, spindled, and multinucleated cells can be seen (H&E, original magnification ×100).


Desmoplastic nevus (DN) is a benign melanocytic neoplasm characterized by predominantly spindle-shaped nevus cells embedded within a fibrotic stroma. Although it can resemble a Spitz nevus, it is recognized as a distinct entity.15-17 Clinically, DN presents as a small and flesh-colored, erythematous or slightly pigmented papule or nodule that usually occurs on the arms and legs of young adults. Histopathologically, DN demonstrates a dermal-based proliferation of spindled melanocytes embedded in a dense collagenous stroma with sparse or absent melanin deposition. The collagen bundles often show artifactual clefts and onion skin-like accentuation around vessels. Melanocytes may be epithelioid (Figure 2).16 Immunohistochemically, DN expresses melanocytic markers such as S-100, Melan-A, and human melanoma black 45, but epithelial membrane antigen is negative. Human melanoma black 45 demonstrates maturation with stronger staining in superficial portions of the lesion and diminution of staining with increasing dermal depth.18 Many other melanocytic tumors share histologic similarities to DN including blue nevus and desmoplastic melanoma.17,19,20  

Figure 2. Desmoplastic nevus. A proliferation of spindled and epithelioid melanocytes embedded in a dense collagenous stroma. Clefts within sclerotic collagen bundles can be seen (H&E, original magnification ×100).

Palisaded encapsulated neuroma, also referred to as solitary circumscribed neuroma, was first described by Reed et al21 in 1972. It is a benign and solitary, firm, dome-shaped, flesh-colored papule that occurs in middle-aged adults, predominately near mucocutaneous junctions of the face. Other locations include the oral mucosa, eyelid, nasal fossa, shoulder, arm, hand, foot, and glans penis.22,23 Histopathologically, palisaded encapsulated neuroma demonstrates a solitary, well-circumscribed, partially encapsulated, intradermal nodule composed of interweaving fascicles of spindle cells with prominent clefts (Figure 3). Rarely, palisaded encapsulated neuroma may have a plexiform or multinodular architecture.24 Immunohistochemically, tumor cells stain positively for S-100 protein, type IV collagen, and vimentin. The capsule, composed of perineural cells, stains positive for epithelial membrane antigen. A neurofilament stain will highlight axons within the tumor.24,25 Currently, palisaded encapsulated neuroma does not have a well-established link to known neurocutaneous or inherited syndromes. Excision is curative with a low risk of recurrence.26 

Figure 3. Palisaded encapsulated neuroma. A partially encapsulated nodule composed of interweaving fascicles of spindle cells with prominent clefts can be seen (H&E, original magnification ×100).

Sclerotic fibromas (SFs) were first reported by Weary et al27 as multiple tumors involving the tongues of patients with Cowden syndrome. Sporadic or solitary SFs of the skin in patients without Cowden syndrome have been reported, and both multiple and solitary SFs present with similar pathologic changes.28-30 Clinically, the solitary variant manifests as a well-demarcated, flesh-colored to erythematous, waxy papule or nodule with no site or sex predilection.30,31 Histologically, SF demonstrates a well-demarcated, nonencapsulated dermal nodule composed of hypocellular and sclerotic collagen bundles with scattered spindled cells and prominent clefts resembling Vincent van Gogh's Starry Night or plywood (Figure 4). Immunohistochemically, the spindled cells strongly express CD34. Factor XIIIa and markers of melanocytic, neural, and muscular differentiation are negative. When rendering a diagnosis in a patient with multiple SFs, a comment regarding the possibility of Cowden syndrome should be mentioned.32 

Figure 4. Sclerotic fibroma. Sclerotic collagen bundles with scattered spindled cells and clefts resembling Vincent van Gogh’s Starry Night (H&E, original magnification ×100).

The Diagnosis: Sclerosing Perineurioma 

Sclerosing perineurioma, first described in 1997 by Fetsch and Miettinen,1 is a subtype of perineurioma with a strong predilection for the fingers and palms of young adults. Rare cases involving extra-acral sites including the forearm, elbow, axilla, back, neck, lower leg, thigh, knee, lips, nose, and mouth have been reported.2-4 Perineurioma is a relatively uncommon and benign peripheral nerve sheath tumor with exclusive perineurial differentiation.5 Perineurioma is divided into intraneural and extraneural types; the latter are further subclassified into soft tissue, sclerosing, reticular, and plexiform types. Other rare forms include the sclerosing, Pacinian corpuscle-like perineurioma, lipomatous perineurioma, perineurioma with xanthomatous areas, and perineurioma with granular cells.6,7  

Clinically, sclerosing perineurioma usually presents as a solitary lesion; however, rare cases of multiple lesions have been reported.8 Our patient presented with a solitary papule on the nose. Histopathologically, sclerosing perineurioma demonstrates slender spindle cells in a whorled growth pattern (onion skin) embedded in a hyalinized, lamellar, and dense collagenous stroma with intervening cleftlike spaces. Immunohistochemically, the spindle cells of our case stained positive for epithelial membrane antigen (quiz images). Other positive immunostains for perineurioma include claudin-1 and glucose transporter 1 (GLUT1). Perineurioma lacks expression of S-100 but can express CD34.2 As a benign tumor, the prognosis of sclerosing perineurioma is excellent. Complete local excision is considered curative.1  

Angiofibroma, also known as fibrous papule, is a common and benign lesion located primarily on or in close proximity to the nose.9 Angiofibromas can be associated with genodermatoses such as tuberous sclerosis, multiple endocrine neoplasia type 1, or Birt-Hogg-Dubé syndrome. When angiofibromas involve the penis, they are called pearly penile papules. Ungual angiofibroma, also known as Koenen tumor, occurs underneath the nail.10-12 Both facial angiofibromas (>3) and ungual angiofibromas (>2) are independent major criteria for tuberous sclerosis.13 Clinically, angiofibroma presents as a small, dome-shaped, pink papule arising on the lower portion of the nose or nearby area of the central face. Histopathologically, angiofibromas classically demonstrate increased dilated vessels and fibrosis in the dermis. Stellate, plump, spindle-shaped, and multinucleated cells can be seen in the collagenous stroma. The collagen fibers around hair follicles are arranged concentrically, resulting in an onion skin-like appearance. The epidermal rete ridges can be effaced (Figure 1). Increased numbers of single-unit melanocytes along the dermoepidermal junction can be seen in some cases. Immunohistochemically, a variable number of spindled and multinucleated cells in the dermis stain with factor XIIIa. There are at least 7 histologic variants of angiofibroma including hypercellular, pigmented, inflammatory, pleomorphic, clear cell, granular cell, and epithelioid.9,14 

Figure 1. Fibrous papule. Increased dilated vessels and fibrosis in the dermis with an onion skin–like appearance around hair follicles. Scattered stellate, spindled, and multinucleated cells can be seen (H&E, original magnification ×100).


Desmoplastic nevus (DN) is a benign melanocytic neoplasm characterized by predominantly spindle-shaped nevus cells embedded within a fibrotic stroma. Although it can resemble a Spitz nevus, it is recognized as a distinct entity.15-17 Clinically, DN presents as a small and flesh-colored, erythematous or slightly pigmented papule or nodule that usually occurs on the arms and legs of young adults. Histopathologically, DN demonstrates a dermal-based proliferation of spindled melanocytes embedded in a dense collagenous stroma with sparse or absent melanin deposition. The collagen bundles often show artifactual clefts and onion skin-like accentuation around vessels. Melanocytes may be epithelioid (Figure 2).16 Immunohistochemically, DN expresses melanocytic markers such as S-100, Melan-A, and human melanoma black 45, but epithelial membrane antigen is negative. Human melanoma black 45 demonstrates maturation with stronger staining in superficial portions of the lesion and diminution of staining with increasing dermal depth.18 Many other melanocytic tumors share histologic similarities to DN including blue nevus and desmoplastic melanoma.17,19,20  

Figure 2. Desmoplastic nevus. A proliferation of spindled and epithelioid melanocytes embedded in a dense collagenous stroma. Clefts within sclerotic collagen bundles can be seen (H&E, original magnification ×100).

Palisaded encapsulated neuroma, also referred to as solitary circumscribed neuroma, was first described by Reed et al21 in 1972. It is a benign and solitary, firm, dome-shaped, flesh-colored papule that occurs in middle-aged adults, predominately near mucocutaneous junctions of the face. Other locations include the oral mucosa, eyelid, nasal fossa, shoulder, arm, hand, foot, and glans penis.22,23 Histopathologically, palisaded encapsulated neuroma demonstrates a solitary, well-circumscribed, partially encapsulated, intradermal nodule composed of interweaving fascicles of spindle cells with prominent clefts (Figure 3). Rarely, palisaded encapsulated neuroma may have a plexiform or multinodular architecture.24 Immunohistochemically, tumor cells stain positively for S-100 protein, type IV collagen, and vimentin. The capsule, composed of perineural cells, stains positive for epithelial membrane antigen. A neurofilament stain will highlight axons within the tumor.24,25 Currently, palisaded encapsulated neuroma does not have a well-established link to known neurocutaneous or inherited syndromes. Excision is curative with a low risk of recurrence.26 

Figure 3. Palisaded encapsulated neuroma. A partially encapsulated nodule composed of interweaving fascicles of spindle cells with prominent clefts can be seen (H&E, original magnification ×100).

Sclerotic fibromas (SFs) were first reported by Weary et al27 as multiple tumors involving the tongues of patients with Cowden syndrome. Sporadic or solitary SFs of the skin in patients without Cowden syndrome have been reported, and both multiple and solitary SFs present with similar pathologic changes.28-30 Clinically, the solitary variant manifests as a well-demarcated, flesh-colored to erythematous, waxy papule or nodule with no site or sex predilection.30,31 Histologically, SF demonstrates a well-demarcated, nonencapsulated dermal nodule composed of hypocellular and sclerotic collagen bundles with scattered spindled cells and prominent clefts resembling Vincent van Gogh's Starry Night or plywood (Figure 4). Immunohistochemically, the spindled cells strongly express CD34. Factor XIIIa and markers of melanocytic, neural, and muscular differentiation are negative. When rendering a diagnosis in a patient with multiple SFs, a comment regarding the possibility of Cowden syndrome should be mentioned.32 

Figure 4. Sclerotic fibroma. Sclerotic collagen bundles with scattered spindled cells and clefts resembling Vincent van Gogh’s Starry Night (H&E, original magnification ×100).

References
  1. Fetsch JF, Miettinen M. Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults. Am J Surg Pathol. 1997;21:1433-1442. 
  2. Fox MD, Gleason BC, Thomas AB, et al. Extra-acral cutaneous/soft tissue sclerosing perineurioma: an under-recognized entity in the differential of CD34-positive cutaneous neoplasms. J Cutan Pathol. 2010;37:1053-1056. 
  3. Erstine EM, Ko JS, Rubin BP, et al. Broadening the anatomic landscape of sclerosing perineurioma: a series of 5 cases in nonacral sites. Am J Dermatopathol. 2017;39:679-681. 
  4. Senghore N, Cunliffe D, Watt-Smith S, et al. Extraneural perineurioma of the face: an unusual cutaneous presentation of an uncommon tumour. Br J Oral Maxillofac Surg. 2001;39:315-319. 
  5. Lazarus SS, Trombetta LD. Ultrastructural identification of a benign perineurial cell tumor. Cancer. 1978;41:1823-1829. 
  6. Macarenco RS, Cury-Martins J. Extra-acral cutaneous sclerosing perineurioma with CD34 fingerprint pattern. J Cutan Pathol. 2017;44:388-392. 
  7. Santos-Briz A, Godoy E, Canueto J, et al. Cutaneous intraneural perineurioma: a case report. Am J Dermatopathol. 2013;35:E45-E48. 
  8. Rubin AI, Yassaee M, Johnson W, et al. Multiple cutaneous sclerosing perineuriomas: an extensive presentation with involvement of the bilateral upper extremities. J Cutan Pathol. 2009;36(suppl 1):60-65. 
  9. Damman J, Biswas A. Fibrous papule: a histopathologic review. Am J Dermatopathol. 2018;40:551-560. 
  10. Macri A, Tanner LS. Cutaneous angiofibroma. StatPearls. https://www.statpearls.com/kb/viewarticle/17566/. Updated January 24, 2019. Accessed October 21, 2019.  
  11. Darling TN, Skarulis MC, Steinberg SM, et al. Multiple facial angiofibromas and collagenomas in patients with multiple endocrine neoplasia type 1. Arch Dermatol. 1997;133:853-857. 
  12. Schaffer JV, Gohara MA, McNiff JM, et al. Multiple facial angiofibromas: a cutaneous manifestation of Birt-Hogg-Dube syndrome. J Am Acad Dermatol. 2005;53:S108-S111. 
  13. Northrup H, Krueger DA; International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol. 2013;49:243-254. 
  14. Bansal C, Stewart D, Li A, et al. Histologic variants of fibrous papule. J Cutan Pathol. 2005;32:424-428. 
  15. Harris GR, Shea CR, Horenstein MG, et al. Desmoplastic (sclerotic) nevus: an underrecognized entity that resembles dermatofibroma and desmoplastic melanoma. Am J Surg Pathol. 1999;23:786-794. 
  16. Ferrara G, Brasiello M, Annese P, et al. Desmoplastic nevus: clinicopathologic keynotes. Am J Dermatopathol. 2009;31:718-722. 
  17. Sherrill AM, Crespo G, Prakash AV, et al. Desmoplastic nevus: an entity distinct from Spitz nevus and blue nevus. Am J Dermatopathol. 2011;33:35-39. 
  18. Kucher C, Zhang PJ, Pasha T, et al. Expression of Melan-A and Ki-67 in desmoplastic melanoma and desmoplastic nevi. Am J Dermatopathol. 2004;26:452-457. 
  19. Sidiropoulos M, Sholl LM, Obregon R, et al. Desmoplastic nevus of chronically sun-damaged skin: an entity to be distinguished from desmoplastic melanoma. Am J Dermatopathol. 2014;36:629-634. 
  20. Kiuru M, Patel RM, Busam KJ. Desmoplastic melanocytic nevi with lymphocytic aggregates. J Cutan Pathol. 2012;39:940-944. 
  21. Reed RJ, Fine RM, Meltzer HD. Palisaded, encapsulated neuromas of the skin. Arch Dermatol. 1972;106:865-870. 
  22. Newman MD, Milgraum S. Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. Dermatol Online J. 2008;14:12. 
  23. Beutler B, Cohen PR. Palisaded encapsulated neuroma of the trunk: a case report and review of palisaded encapsulated neuroma. Cureus. 2016;8:E726. 
  24. Jokinen CH, Ragsdale BD, Argenyi ZB. Expanding the clinicopathologic spectrum of palisaded encapsulated neuroma. J Cutan Pathol. 2010;37:43-48. 
  25. Argenyi ZB. Immunohistochemical characterization of palisaded, encapsulated neuroma. J Cutan Pathol. 1990;17:329-335. 
  26. Batra J, Ramesh V, Molpariya A, et al. Palisaded encapsulated neuroma: an unusual presentation. Indian Dermatol Online J. 2018;9:262-264. 
  27.  Weary PE, Gorlin RJ, Gentry WC Jr, et al. Multiple hamartoma syndrome (Cowden's disease). Arch Dermatol. 1972;106:682-690. 
  28. Mahmood MN, Salama ME, Chaffins M, et al. Solitary sclerotic fibroma of skin: a possible link with pleomorphic fibroma with immunophenotypic expression for O13 (CD99) and CD34. J Cutan Pathol. 2003;30:631-636. 
  29. Nakashima K, Yamada N, Adachi K, et al. Solitary sclerotic fibroma of the skin: morphological characterization of the 'plywood-like pattern'. J Cutan Pathol. 2008;35(suppl 1):74-79. 
  30. Rapini RP, Golitz LE. Sclerotic fibromas of the skin. J Am Acad Dermatol. 1989;20:266-271. 
  31. Abbas O, Ghosn S, Bahhady R, et al. Solitary sclerotic fibroma on the scalp of a young girl: reactive sclerosis pattern? J Dermatol. 2010;37:575-577. 
  32. Hanft VN, Shea CR, McNutt NS, et al. Expression of CD34 in sclerotic ("plywood") fibromas. Am J Dermatopathol. 2000;22:17-21.
References
  1. Fetsch JF, Miettinen M. Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults. Am J Surg Pathol. 1997;21:1433-1442. 
  2. Fox MD, Gleason BC, Thomas AB, et al. Extra-acral cutaneous/soft tissue sclerosing perineurioma: an under-recognized entity in the differential of CD34-positive cutaneous neoplasms. J Cutan Pathol. 2010;37:1053-1056. 
  3. Erstine EM, Ko JS, Rubin BP, et al. Broadening the anatomic landscape of sclerosing perineurioma: a series of 5 cases in nonacral sites. Am J Dermatopathol. 2017;39:679-681. 
  4. Senghore N, Cunliffe D, Watt-Smith S, et al. Extraneural perineurioma of the face: an unusual cutaneous presentation of an uncommon tumour. Br J Oral Maxillofac Surg. 2001;39:315-319. 
  5. Lazarus SS, Trombetta LD. Ultrastructural identification of a benign perineurial cell tumor. Cancer. 1978;41:1823-1829. 
  6. Macarenco RS, Cury-Martins J. Extra-acral cutaneous sclerosing perineurioma with CD34 fingerprint pattern. J Cutan Pathol. 2017;44:388-392. 
  7. Santos-Briz A, Godoy E, Canueto J, et al. Cutaneous intraneural perineurioma: a case report. Am J Dermatopathol. 2013;35:E45-E48. 
  8. Rubin AI, Yassaee M, Johnson W, et al. Multiple cutaneous sclerosing perineuriomas: an extensive presentation with involvement of the bilateral upper extremities. J Cutan Pathol. 2009;36(suppl 1):60-65. 
  9. Damman J, Biswas A. Fibrous papule: a histopathologic review. Am J Dermatopathol. 2018;40:551-560. 
  10. Macri A, Tanner LS. Cutaneous angiofibroma. StatPearls. https://www.statpearls.com/kb/viewarticle/17566/. Updated January 24, 2019. Accessed October 21, 2019.  
  11. Darling TN, Skarulis MC, Steinberg SM, et al. Multiple facial angiofibromas and collagenomas in patients with multiple endocrine neoplasia type 1. Arch Dermatol. 1997;133:853-857. 
  12. Schaffer JV, Gohara MA, McNiff JM, et al. Multiple facial angiofibromas: a cutaneous manifestation of Birt-Hogg-Dube syndrome. J Am Acad Dermatol. 2005;53:S108-S111. 
  13. Northrup H, Krueger DA; International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol. 2013;49:243-254. 
  14. Bansal C, Stewart D, Li A, et al. Histologic variants of fibrous papule. J Cutan Pathol. 2005;32:424-428. 
  15. Harris GR, Shea CR, Horenstein MG, et al. Desmoplastic (sclerotic) nevus: an underrecognized entity that resembles dermatofibroma and desmoplastic melanoma. Am J Surg Pathol. 1999;23:786-794. 
  16. Ferrara G, Brasiello M, Annese P, et al. Desmoplastic nevus: clinicopathologic keynotes. Am J Dermatopathol. 2009;31:718-722. 
  17. Sherrill AM, Crespo G, Prakash AV, et al. Desmoplastic nevus: an entity distinct from Spitz nevus and blue nevus. Am J Dermatopathol. 2011;33:35-39. 
  18. Kucher C, Zhang PJ, Pasha T, et al. Expression of Melan-A and Ki-67 in desmoplastic melanoma and desmoplastic nevi. Am J Dermatopathol. 2004;26:452-457. 
  19. Sidiropoulos M, Sholl LM, Obregon R, et al. Desmoplastic nevus of chronically sun-damaged skin: an entity to be distinguished from desmoplastic melanoma. Am J Dermatopathol. 2014;36:629-634. 
  20. Kiuru M, Patel RM, Busam KJ. Desmoplastic melanocytic nevi with lymphocytic aggregates. J Cutan Pathol. 2012;39:940-944. 
  21. Reed RJ, Fine RM, Meltzer HD. Palisaded, encapsulated neuromas of the skin. Arch Dermatol. 1972;106:865-870. 
  22. Newman MD, Milgraum S. Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. Dermatol Online J. 2008;14:12. 
  23. Beutler B, Cohen PR. Palisaded encapsulated neuroma of the trunk: a case report and review of palisaded encapsulated neuroma. Cureus. 2016;8:E726. 
  24. Jokinen CH, Ragsdale BD, Argenyi ZB. Expanding the clinicopathologic spectrum of palisaded encapsulated neuroma. J Cutan Pathol. 2010;37:43-48. 
  25. Argenyi ZB. Immunohistochemical characterization of palisaded, encapsulated neuroma. J Cutan Pathol. 1990;17:329-335. 
  26. Batra J, Ramesh V, Molpariya A, et al. Palisaded encapsulated neuroma: an unusual presentation. Indian Dermatol Online J. 2018;9:262-264. 
  27.  Weary PE, Gorlin RJ, Gentry WC Jr, et al. Multiple hamartoma syndrome (Cowden's disease). Arch Dermatol. 1972;106:682-690. 
  28. Mahmood MN, Salama ME, Chaffins M, et al. Solitary sclerotic fibroma of skin: a possible link with pleomorphic fibroma with immunophenotypic expression for O13 (CD99) and CD34. J Cutan Pathol. 2003;30:631-636. 
  29. Nakashima K, Yamada N, Adachi K, et al. Solitary sclerotic fibroma of the skin: morphological characterization of the 'plywood-like pattern'. J Cutan Pathol. 2008;35(suppl 1):74-79. 
  30. Rapini RP, Golitz LE. Sclerotic fibromas of the skin. J Am Acad Dermatol. 1989;20:266-271. 
  31. Abbas O, Ghosn S, Bahhady R, et al. Solitary sclerotic fibroma on the scalp of a young girl: reactive sclerosis pattern? J Dermatol. 2010;37:575-577. 
  32. Hanft VN, Shea CR, McNutt NS, et al. Expression of CD34 in sclerotic ("plywood") fibromas. Am J Dermatopathol. 2000;22:17-21.
Issue
Cutis - 104(5)
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Cutis - 104(5)
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268, 292-294
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268, 292-294
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Solitary Papule on the Nose
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Solitary Papule on the Nose
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H&E, original magnification ×20.

H&E, original magnification ×100 (epithelial membrane antigen, original magnification ×200 [inset]).

A 25-year-old man presented with a flesh-colored papule on the left side of the nose of 2 years' duration. 

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