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Background

This tumor predominately occurs in the head and neck.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/
Other Diagnostic Testing
 
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  
VARIANTS  
Histologic variants of fibrous papule.

Bansal C, Stewart D, Li A, Cockerell CJ.

Department of Dermatology, Division of Dermatopathology, University of Texas Southwestern Medical Center, Dallas, TX, USA.

J Cutan Pathol. 2005 Jul;32(6):424-8. Abstract quote  

Fibrous papules (FPs) are common benign lesions usually of the face. Most are readily recognizable histologically, although several variants exist that may not be as easily diagnosed.

These include hypercellular, clear cell, pigmented, pleomorphic, and inflammatory variants. A granular cell variant has also been described. We microscopically evaluated 212 FPs, and of those, 184 demonstrated features of one of the variants.

We conclude that variants of FP may be encountered not uncommonly. Dermatopathologists should be aware of these to avoid misdiagnosis.
CLEAR CELLS  
Fibrous papule with clear fibrocytes.

Rose C.
Am J Dermatopathol. 2000 Dec;22(6):563-4. Abstract quote  
GRANULAR CELLS  
Fibrous papule of the nose with granular cells: two cases.

Guitart J, Bergfeld WF, Tuthill RJ.

Department of Pathology, Cleveland Clinic Foundation, Ohio 44195.

J Cutan Pathol. 1991 Aug;18(4):284-7. Abstract quote

Two cases of fibrous papule of the nose (FPN) with granular cell features are presented and discussed. Both lesions have classic architectural features of FPN; however, the main stromal cells show large cytoplasmic granules of the type seen in granular cells.

We are uncertain of the significance of these findings. Our hypotheses include a perifollicular granular cell reaction and a granular degenerative change of local dermal dendrocytes.

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  
NKI/C3  
Clear Cell Fibrous Papule With NKI/C3 Expression: Clinical and Histologic Features in Six Cases.

Lee AN, Stein SL, Cohen LM.

From *Private Practice, Marlborough, Massachusetts; daggerDivision of Dermatology, University of Massachusetts Medical School, Worcester, Massachusetts; double daggerSection of Dermatology, University of Chicago, Chicago, Illinois; section signCohen Dermatopathology PC, Newton, Massachusetts; and parallelDepartment of Dermatology, Tufts University School of Medicine, Boston, Massachusetts.

Am J Dermatopathol. 2005 Aug;27(4):296-300. Abstract quote  

Fibrous papule of the nose is a common benign lesion of dermal fibroblast lineage. Two unusual variants have been described, namely, fibrous papule with granular cells and fibrous papule with clear fibrocytes.

We report a second case series (six cases) of clear cell fibrous papule to add to the first series of 9 cases. Clinical and histologic features in our cases are similar to those in the first series. All of our specimens were dome-shaped, 2- to 5-mm skin-colored to slightly erythematous papules on the faces of three male and three female adults ranging from 18 to 48 years of age. All but one lesion were on the nose. Clinical differential diagnoses included fibrous papule, verruca, basal cell carcinoma, and a variety of other neoplasms.

Histologically, dermal aggregates of clear cells with finely granular to vacuolated cytoplasm, and centrally located nuclei, were found. Most specimens also contained ectatic capillaries, and all showed evidence of irritation or trauma. Periodic acid-Schiff stain was negative in all specimens to which it was applied (5/6). Neural, melanocytic, and epithelial origins were eliminated by negative staining with S-100, Mart-1, cytokeratins, epithelial membrane antigen, and carcinoembryonic antigen, performed on some of the specimens. A mesenchymal nature was confirmed in one specimen staining strongly positive for vimentin. Five of six cases stained positively for CD68, and all five cases studied were strongly and diffusely positive for NKI/C3.

Factor XIIIa stain highlighted scattered dendritic cells within the lesion but was otherwise negative in all six cases studied. Recognition of this variant of fibrous papule is important to distinguish this benign lesion from other clear cell neoplasms.
S100  
Fibrous papule: an immunohistochemical study with an antibody to S-100 protein.

Spiegel J, Nadji M, Penneys NS.

J Am Acad Dermatol. 1983 Sep;9(3):360-2. Abstract quote  

We studied twenty biopsies taken from fibrous papules which were located in the central part of the face. The lesions, which were characteristic histologically, did not contain S-100 protein within the stellate cells in the papillary dermis, nor was this substance found in mesenchymal cells with some features of nevus cells.

In control studies, S-100 protein was found using an unlabeled antibody peroxidase-antiperoxidase technic within nevus cells composing junctional intradermal, and compound nevi, Spitz nevi, and halo nevi.

It therefore seems unlikely that fibrous papule represents a form of degenerated nevus as some investigators have proposed.
ELECTRON MICROSCOPY  
Fibrous papule of the face. An electron-microscopic study of two cases.

Santa Cruz DJ, Prioleau PG.

Am J Dermatopathol. 1979 Winter;1(4):349-52. Abstract quote  

Fibrous papules on the nose from two patients were examined by electron microscopy.

In each, the large characteristic dermal cells had ultrastructural features of fibroblasts, and there was no evidence of either melanocytic or Schwann cell differentiation.

Banded structures (long-spacing collagen) were present in one case.

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
PLEOMORPHIC FIBROMA  
Pleomorphic fibroma of the skin: a benign neoplasm with cytologic atypia. A clinicopathologic study of eight cases.

Kamino H, Lee JY, Berke A.

Department of Dermatology, New York University Medical Center 10016.
Am J Surg Pathol. 1989 Feb;13(2):107-13. Abstract quote  

A clinicopathologic study of eight examples of polypoid and dome-shaped cutaneous fibrous lesions with sparse cellularity but striking nuclear atypia and rare mitotic figures is presented.

Positive immunohistochemical staining for vimentin and actin supported the fibroblastic nature of these lesions. All eight cases were adults whose ages ranged from 33 to 67 years (mean 52 years). Five were women and three were men. Five lesions were located on extremities, two on the trunk, and one on the face and they measured from 4 to 16 mm in greatest dimension. The lesions were clinically followed from 4 months to 5 years. They all showed benign clinical behavior, with only one local recurrence in a lesion that had been incompletely removed. The nuclear atypia seen in these fibrous lesions may be similar to that which occurs in other benign mesenchymal neoplasms, such as pleomorphic lipoma, pleomorphic leiomyoma, ancient schwannoma, and variants of dermatofibroma with atypical cells.

We suggest that "pleomorphic fibroma" is an appropriate term for this lesion based on its histologic differentiation, cytologic atypia, and benign clinical course.
RHINOPHYMA  
Rhinophyma's fibrous variant. Histopathology and immunohistochemistry.

Tope WD, Sangueza OP.

Department of Dermatology, Oregon Health Sciences University, Portland.

Am J Dermatopathol. 1994 Jun;16(3):307-10. Abstract quote

Rhinophyma may present with either of two distinct histopathologic appearances. The most common shows histopathologic features of rosacea. The second pattern shows telangiectasia, diffuse dermal fibrosis with abundant mucin, and a virtual absence of pilosebaceous structures. These histopathologic features of the lesser-known fibrous variant of rhinophyma mimic those of fibrous papule of the nose.

We report an unusual case that histopathologically resembled the second variant. Immunohistochemically, the dermal fibroblasts in this case showed intense staining with Factor XIIIa in a pattern similar to that demonstrated in fibrous papules. S-100 protein failed to stain these dermal fibroblasts.

The presence of Factor XIIIa+ dermal fibroblasts may portend the evolution of fibrosis in rosacea and other inflammatory dermatoses.

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


Commonly Used Terms

Basic Principles of Disease
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Commonly Used Terms
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Diagnostic Process
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Surgical Pathology Report
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Special Stains
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Got Path?
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Last Updated September 7, 2005

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