Multiple angiofibromas are commonly found in patients with tuberous sclerosis complex. We report a rare presentation of multiple congenital fibrous papules occurring only on the lips with no syndromic associations.

BACKGROUND: Angiofibromas are common benign skin lesions that are diagnosed clinically and histopathologically. Fibrous papule (FP) is one of the clinical types of angiofibromas located primarily on the nose.
METHODS: The cases with the confirmed diagnosis of angiofibroma were enrolled. Demographic features of the patients and clinical characteristics of the lesions were extracted. Moreover, histopathological features of the available slides were microscopically reviewed.
RESULTS: From 83 cases of cutaneous angiofibromas, 57 (69%) had lesions consistent with FP. The mean age at diagnosis was 37.7 ± 16.3 years and the most common site of involvement was the nose (53% of cases). Regarding the histopathological evaluation of 71 slides, most cases represented vascular changes (97%) and dermal fibroplasia (87%). Melanin incontinence (41%), acanthosis (31%), and lichenoid features (25%) were other common histopathological findings. Moreover, a sparse inflammatory cell infiltrate was present in 68% of cases. The most prevalent inflammatory cells were lymphocytes (61%) and histiocytes (34%).
CONCLUSIONS: This study presented the histopathological findings of a series of cutaneous angiofibromas. Dermatopathologists should be aware of these features, especially those that are less common, to better diagnose and differentiate them from malignant neoplasms.

BACKGROUND: Signs of inflammation including epidermal interface changes, spongiosis, and dermal inflammation as well as pagetoid dyskeratosis are rarely described in fibrous papule (FP). We aimed to describe the inflammatory parameters, the rate of pagetoid dyskeratosis, along with CD163 immunohistochemical staining as an adjunctive diagnostic tool in FP.
METHODS: Histopathology samples of all biopsy-proven FP cases were retrieved from archives and investigated for inflammatory parameters, presence of pagetoid dyskeratosis, as well as CD163, CD10, and CD34 immunostaining pattern of dermal spindle/stellate or multinucleate cells (graded from 0 to 4).
RESULTS: Thirty-two cases of FP were identified. A high rate of inflammatory parameters including interface changes (20/32), spongiosis (31/32), and dermal lymphocytic inflammation (31/32) were detected. Pagetoid dyskeratosis was identified in eight out of 32 cases (25%). A grade 4 staining revealing a strong dendritic pattern was confirmed in all FP cases with CD163 immunohistochemistry including atypical variants such as granular FP, compared with CD10 (11/32) and CD34 (3/32).
CONCLUSIONS: The dendritic cellular proliferation in FP may represent an inflammatory response to various stimuli; pagetoid dyskeratosis is a relatively common and underrecognized epidermal feature and CD163 immunostaining may be used as an adjunctive diagnostic tool in unusual histopathological subtypes.

Scleromyxedema (SMX) is an inflammatory condition of unknown etiology strongly associated with monoclonal gammopathy. Classical histopathology of SMX is characterized with the triad of diffuse mucin deposits, increased amount of collagen, and presence of stellate fibroblasts. Herein, we report an unusual histopathological variant of SMX in a 41-year-old female with lesions of the nose histopathologically mimicking cellular angiofibromas. The dome-shaped papules were characterized by increased collagen bundles and fascicles of spindle cells. Widened vessels were seen at the periphery of the proliferation. Cells expressed CD68. Factor XIIIa was expressed only by dendritic cells. The mucin was highlighted with colloidal iron. In sum, we draw attention to this unusual variant of SMX, which should be suspected in a setting of multiple \"angiofibromas/fibrous papules\" on the face with presence of mucin.

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Since the initial description of the granular cell tumor in 1926, numerous other neoplasms, both benign and malignant, have been described to exhibit granular cell change. In most cases, diagnosis remains straightforward via recognition of retained histopathological morphology of the archetypal tumor, despite the presence of focal granular appearance. However, tumors with granular cell differentiation can present a diagnostic challenge either by mimicking alternative diagnoses, or by failing to exhibit architectural clues of the primary entity, thus requiring an immunohistochemical work-up. In light of this, it is important to be aware of the various entities that have been reported to exhibit granular cell morphology. In this review such tumors are discussed along with pertinent clinical and histopathological features.

Facial angiofibromas are benign tumors presenting as firm, dome-shaped, flesh-colored to pink papules, typically on the nose and adjoining central face. Clinically and dermoscopically they can mimic melanocytic nevi or basal cell carcinomas (BCC). Reflectance confocal microscopy (RCM) is a noninvasive imaging tool that is useful in diagnosing melanocytic and non-melanocytic facial lesions. To date no studies have described the RCM features of facial angiofibromas. Herein, we present two cases of facial angiofibromas that were imaged with RCM and revealed tumor island-like structures that mimicked BCC, leading to skin biopsy.

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Dendritic myxofibrolipoma is a newly described benign soft tissue tumor that could be easily mistaken for sarcoma. It develops primarily in the subcutis or muscular fascia of the head and neck, shoulders, etc. Histologically, the tumor is characterized by an admixture of mature adipose tissue, spindle and stellate cells, and abundant myxoid stroma with prominent collagenization. These neoplasms typically show positive immunoreactivity for CD-34, vimentin and Bcl-2. Herein, we described a rare case presenting with a papule on the nasal tip in a 69-year old patient. Histopathology and immunohistochemical staining confirmed the diagnosis. In short, it brings the attention of clinicians to the importance of proper identification and characterization of this tumor.

BACKGROUND: Fibrous papules of the face are frequent benign lesions seen in the nasal and perinasal region. Their clinical aspect is indistinct and the histological signs are sometimes mild or possibly misleading in the case of atypical forms. We carried out a retrospective study of 283 fibrous papules diagnosed at our histology laboratory. The goal of this study was to characterize this type of frequent but occasionally unrecognized lesion.
METHODS: We performed a retrospective study of fibrous papules of the face diagnosed in the dermatopathology laboratory of our dermatology centre between January 2002 and December 2011. The study concerned the clinical information noted in the examination request and the morphological abnormalities seen at optical microscopy. An immunohistological study of factor XIIIa was performed in selected cases.
RESULTS: The fibrous papules of the face came from 129 men and 154 women aged between 18 and 90 years (mean: 46 years). Two hundred and thirty-seven (83.7%) lesions were taken from the nasal region and none were taken from anywhere other than the face. The clinically mentioned diagnoses varied. A diagnosis of fibrous papule of the face was stated in 42% of cases, and the main differential diagnoses were nevus (stated in 34% of cases) and basal cell carcinoma (stated in 14% of cases). The fibrous papules were classic in 85.5% of cases. We observed 6 variants of fibrous papule: hypercellular, inflammatory, pleomorphic, pigmented, clear-cell and granular-cell types. Immunohistochemistry of factor XIIIa was positive in all cases except clear-cell fibrous papules.
CONCLUSIONS: This study shows that despite their frequency, these lesions often go unrecognized, since the hypothesis of a fibrous papule of the face was mentioned in fewer than 50% of cases at the time of biopsy. Diagnosis is often made by the histopathologist, who may be misled by some rare types. The principal differential diagnoses are nevus and basal cell carcinoma, thus warranting methodical histological analysis of all pieces.