DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

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Monday, May 24, 2010

Answer of Dermatopathology Case 41

Fibrous Papule of the Face (Fibrous Papule of the Nose)

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Solitary Oral Fibromas of the Tongue Show Similar Morphologic Features to Fibrous Papule of the Face: A Study of 31 Cases. Am J Dermatopathol. 2010 Apr 24.

The morphologic similarities between fibrous papules of the face and multiple oral fibromas were mentioned long ago, mainly in the context of phakomatoses. Both lesions have been considered to be different types of angiofibromas. Nonetheless, this interpretation is not accepted by all authors. We tried to investigate if solitary oral fibromas also share morphologic features with fibrous papules of the face. For this purpose, we designed a retrospective study retrieving 31 fibromas of the tongue from 30 different patients and studied a control group of 20 fibrous papules of the face from 20 different patients. From the oral fibromas, 15 lesions presented as dome shape, whereas 16 lesions presented as polypoid. Lesion size varied between 2 and 7 mm. In all the lesions, we found a common pattern: a fibrous and collagenized stroma, with prominent vessels. Inflammatory infiltrate was mild. Also, we specifically searched for 4 morphologic features: dilated blood vessels, concentric perivascular fibrosis, multinucleated cells, and mast cells. Multinucleated cells and dilated blood vessels were a common feature. On the contrary, concentric perivascular fibrosis was not a prominent feature. Thick collagen bundles (keloid like) were also occasionally found. Mitoses were rare. These findings were similar to the ones described in fibrous papules of the face, which were also present in our control group of 20 fibrous papules. Therefore, we conclude that the sporadic type of fibroma shares many morphologic features with the fibrous papules of the face. This would be similar to the morphologic relation, already described, between fibrous papules of the face and oral fibromas of phakomatoses.

Fibrous papule of the face with granular cells. Dermatology. 2008;216(1):56-9.

Fibrous papule of the face is a common benign lesion located most often on the nose. It presents usually as a single small, firm, skin-coloured papule and is often misdiagnosed as melanocytic naevus, wart or small nodular basal cell carcinoma. Histopathologically, the lesions are characterized by involvement of the upper dermis by a fibrovascular proliferation and scattered triangular or stellate, often multinucleated cells. Uncommon histopathologic variants of fibrous papule of the face include hypercellular, clear-cell, pleomorphic, pigmented, inflammatory and granular-cell types. We present here a patient with the syndrome of familial cancer and fibrous papule of the face with granular cells. The granules stained strongly with PAS stain, as well as with CD68 and NKI/C3 immunostains, whereas S-100 protein resulted negative. In our patient the mutations in the 2 most often affected DNA mismatch repair genes of Muir-Torre syndrome were not found, therefore the origin of the familial cancer syndrome remains unknown. Probably the occurrence of the granular-cell fibrous papule of the face was coincidental.

Clear cell fibrous papule with NKI/C3 expression: clinical and histologic features in six cases. Am J Dermatopathol. 2005 Aug;27(4):296-300.

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.

Histologic variants of fibrous papule. J Cutan Pathol. 2005 Jul;32(6):424-8.

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.