Perifollicular Fibroma Research Articles

What is in a name-Perifollicular fibroma or fibrofolliculoma?

So far, confusion exists regarding the question of whether hereditary perifollicular fibromas and fibrofolliculomas can be distinguished from each other. Here, histopathological arguments are presented to clarify this terminological problem. In 1977, Birt et al. described a large kindred affected with hereditary multiple "fibrofolliculomas," which they thought were "a hitherto unrecognized pilar hamartoma," but they never claimed the fibrofolliculomas were part of a syndrome. A careful microscopic comparison shows, however, that the tumors are clinically and histopathologically identical to perifollicular fibromas, as first described by Burnier and Rejšek in 1925. Their familial occurrence was discovered in 1971 by Civatte and Le Tréguilly. Before 1977, the term "perifollicular fibroma" was used for these skin tumors. By contrast, Hornstein and Knickenberg described in 1975 perifollicular fibromas as a cutaneous marker of a syndrome characterized by a predisposition to colon cancer and pneumothorax. Later, two French groups erroneously proposed the term "Birt-Hogg-Dubé syndrome" to describe the co-occurrence of fibrofolliculomas, trichodiscomas, and acrochordons, which was contrary to what Birt et al. had in mind. Hence, today, we should discriminate between the hereditary nonsyndromic perifollicular fibromas, as documented by Civatte and Le Tréguilly and later by Birt et al., and the syndromic perifollicular fibromas, as delineated by Hornstein and Knickenberg.

Nevus Lipomatosus Cutaneous Superficialis with a Histopathological Appearance Resembling Sclerotic Fibroma: A Rare Case Report

Background: Nevus lipomatosus cutaneous superficialis (NLCS) of Hoffmann–Zurhelle is a benign idiopathic hamartoma. There are two types of NLCS, multiple and solitary. They are found in the abdomen, lower back, buttocks, hips, upper posterior thighs, and pelvis. The diagnosis can be evaluated with a typical histopathological of mature fat cells in the dermis, with 10%–50% of the dermis. Case Report: We reported a case of NLCS with clinical papules and multiple nodules on the buttocks since the age of 6 years with a history of lipoma removal. The dermoscopic examination was conducted to confirm the diagnosis. The histopathological examination showed a dominant sclerotic fibroma with two sessions of biopsy and a few mature fats on the dermis after deeper cuts paraffin block. Cryotherapy with an open spray method is treatment of choice in this patient. Discussion: The appearance of the dermis in NLCS can be normal or an increase in collagen. Interestingly, collagen has sclerosis partially and resembles sclerotic fibroma never been reported. NLCS increases the amount of collagen; however, collagen as sclerosis remains obscure. The features of NLCS histopathological with other morphological abnormalities in the dermis have been reported, such as NLCS with perifollicular fibroma (PF) features. The sclerotic fibroma features are other morphological abnormalities in NLCS, as reported in the PF.

Hornstein-Knickenberg syndrome vs. Birt-Hogg-Dubé syndrome: a critical review of an unjustified designation.

The disorder that is presently called 'Birt-Hogg-Dubé syndrome' was in reality delineated in 1975 by Otto P. Hornstein and Monika Knickenberg from Erlangen (Germany) who emphasized that the occurrence of multiple 'perifollicular fibromas' represented a distinct autosomal dominant trait heralding extracutaneous cancer proneness. By contrast, Arthur R. Birt, Georgina R. Hogg and W. James Dubé from Winnipeg, Manitoba, Canada, claimed in 1977 that they had discovered 'a previously unrecognized hereditary pilar hamartoma' for which they proposed the name 'fibrofolliculoma', to be distinguished from the perifollicular fibromas as reported by Hornstein and Knickenberg. Today, many authors believe that 'fibrofolliculoma' is identical with 'perifollicular fibroma', but for the purpose of the present article this question can be left open. More importantly, the Canadian authors did not mention any association with extracutaneous cancer proneness within the large family examined in Winnipeg, nor when discussing the report from Erlangen, which means that they have neither described nor redescribed the syndrome that presently bears their names. Hence, the autosomal dominant disorder of multiple perifollicular fibromas heralding proclivity to extracutaneous cancer should be called after the original authors, Hornstein-Knickenberg syndrome.

Birt-Hogg-Dubé syndrome

A 45-year-old woman with a history of renal carcinoma was observed for facial, cervical and truncal flesh-colored papules. Relatives had similar skin findings and a brother had repeated episodes of pneumothorax. The computerized tomography scan revealed multiple cysts on both lungs. A skin biopsy revealed a perifollicular fibroma. The clinical diagnosis of Birt-Hogg-Dubé syndrome (BHDS) was corroborated by identification of a novel frameshift c.573delGAinsT (p.G191fsX31) mutation in heterozygosity on exon 6 of the folliculin gene. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.

Perifollicular fibroma in Birt–Hogg–Dubé syndrome: an association revisited

Mutation in the folliculin gene in Birt-Hogg-Dubé (BHD) syndrome leads to a spectrum of benign tumors of the hair follicle, classically including both fibrofolliculoma and trichodiscoma. In addition, lesions clinically indistinguishable from fibrofolliculoma/ trichodiscoma may show histopathologic findings of perifollicular fibroma or angiofibroma. Although some consider perifollicular fibroma to be a variant of angiofibroma, the specific histopathologic findings of perifollicular fibroma are uncommon. This is a case series. In 4 patients with multiple facial lesions, recognizing perifollicular fibroma would have been helpful in leading to the diagnosis of BHD syndrome. Perifollicular fibroma is on a spectrum with fibrofolliculoma and trichodiscoma; in a patient with multiple lesions, the diagnosis of perifollicular fibroma is suggestive of BHD syndrome.

Hornstein–Birt–Hogg–Dubé syndrome: A renaming and reconsideration

The so-called Birt-Hogg-Dubé syndrome, an autosomal dominant trait characterized by multiple fibrofolliculomas and extracutaneous cancer proneness, was not first recognized by Birt, Hogg, and Dubé. Hence, the presently used eponymic designation reflects a historical error. In fact, the disorder was discovered in the following way. In 1975, Hornstein and Knickenberg described a "distinct nosological entity" in two sibs with multiple perifollicular fibromas, multiple skin tags, and polyps of the colon with a tendency to malignancy. The father had similar skin lesions and "bilateral kidney cysts" and unilateral lung cysts. In 1976, Hornstein et al. informed, in two additional articles, both geneticists and gastroenterologists about the new autosomal dominant trait. When Birt et al. presented their report in 1977, they knew of Hornstein's first publication but were convinced that they had discovered "a previously unrecognized hereditary pilar hamartoma." This was a misconception because what they called "fibrofolliculoma" has turned out to be identical with "perifollicular fibroma" as described by Hornstein et al. Moreover, Birt et al. failed to mention any associated extracutaneous cancer proneness, whereas Hornstein et al. had delineated the complete syndrome. For all of these reasons, the new term "Hornstein-Birt-Hogg-Dubé syndrome" appears to be appropriate.

A Case of Perifollicular Fibroma

Perifollicular fibroma (PFF) is a very rare proliferative lesion of the perifollicular sheath. A flesh-colored, dome-shaped papulonodule is usually located on the face or the neck. These papulonodules may be single or multiple. Histologically, the lesion consists of a concentric arrangement of cellular fibrous tissue around a normal hair follicle. We describe here an unusual case of PFF in a 41-year-old female who had an asymptomatic small skin colored papule on the scalp, and this is an uncommon location for PFF.

A Case of Perifollicular Fibroma Adjacent to Basal Cell Carcinoma

A Case of Perifollicular Fibroma Adjacent to Basal Cell Carcinoma

Perifollicular fibroma: A case successfully diagnosed with deeply cut sections

Perifollicular fibroma: A case successfully diagnosed with deeply cut sections

Perifollicular fibroma

Perifollicular fibroma(PFF) is a rare cutaneous hamartoma that shows differentiation in the connective tissue sheath of the hair follicle. It may be single (congenital or acquired) or multiple (late onset). We report a 14-year-old Korean boy with a congenital solitary PFF on the face, which supports the nevoid concept of origin rather than a reactive response to injury.

Multiple perifollicular fibromas

Perifollicular fibroma is a benign mesodermal tumour of the hair follicle. It can occur as a solitary papule or as multiple lesions that are clinically indistinguishable from other tumours of the pilar apparatus. Multiple perifollicular fibromas may be inherited although the pattern remains unclear. Adnexal tumours can be associated with internal malignancy and perifollicular fibromas have been linked with adenomatous colonic polyps. This report describes a patient with multiple perifollicular fibromas with no associated malignancy to date and a family pedigree suggestive of an autosomal dominant pattern of inheritance.

Multiple perifollicular fibromas

Perifollicular fibroma is a benign mesodermal tumour of the hair follicle. It can occur as a solitary papule or as multiple lesions that are clinically indistinguishable from other tumours of the pilar apparatus. Multiple perifollicular fibromas may be inherited although the pattern remains unclear. Adnexal tumours can be associated with internal malignancy and perifollicular fibromas have been linked with adenomatous colonic polyps. This report describes a patient with multiple perifollicular fibromas with no associated malignancy to date and a family pedigree suggestive of an autosomal dominant pattern of inheritance.

Multiple perifollicular fibromas: report of a case and analysis of the literature

Perifollicular fibroma is a cutaneous hamartomatous proliferation of the pilar connective tissue sheath. We describe a patient with multiple perifollicular fibromas and analyze the literature on this topic. Histologically, perifollicular fibroma is characterized by a concentric arrangement of collagen fibers surrounding a generally unaltered hair follicle. Clinically, it is usually multiple and occurs predominantly on the face and upper trunk. This clinical presentation is similar to that observed in patients with the Birt-Hogg-Dubé syndrome where, in addition to perifollicular fibromas, fibrofolliculomas, trichodiscomas, and acrochordons are found. Several reports of multiple perifollicular fibroma prior to the recognition of this syndrome may, in fact, represent cases of the Birt-Hogg-Dubé syndrome.

Multiple fibrofolliculomas with trichodiscomas and acrochordons

• The syndrome of multiple fibrofolliculomas with trichodiscomas and acrochordons clinically is characterized by asymptomatic dome-shaped papules primarily involving the head, neck, chest, back, and arms. These tumors represent benign proliferations of the mesodermal and ectodermal components of the pilar apparatus. Substantial overlap between each of these lesions exists. Individual patients also exhibit a disease spectrum in which various tumors preponderate. The stimulus for this proliferation may be genetically determined, but the cause remains unknown. Merkel's cells were not identified in an electron microscopic study of a single trichodiscoma, but banded structures similar to those described in the closely related lesions of perifollicular fibroma and fibrous papule were seen. (<i>Arch Dermatol</i>117:32-35, 1981)

Multiple Fibrofolliculomas With Trichodiscomas and Acrochordons

The syndrome of multiple fibrofolliculomas with trichodiscomas and acrochordons clinically is characterized by asymptomatic dome-shaped papules primarily involving the head, neck, chest, back, and arms. These tumors represent benign proliferations of the mesodermal and ectodermal components of the pilar apparatus. Substantial overlap between each of these lesions exists. Individual patients also exhibit a disease spectrum in which various tumors preponderate. The stimulus for this proliferation may be genetically determined, but the cause remains unknown. Merkel's cells were not identified in an electron microscopic study of a single trichodiscoma, but banded structures similar to those described in the closely related lesions of perifollicular fibroma and fibrous papule were seen.

A Guide to Dermatohistopathology

There are many excellent American textbooks on dermal pathology. Is there a justification for another? Pinkus, who has described at least five new dermopathologic entities (follicular mucinosis, exocrine poroma, fibroepithelial tumor of the skin, trichofolliculoma, and perifollicular fibroma), is more than adequately justified, and is highly qualified to write a dermal pathologic treatise. If further proof is needed, then it would be his approach to the subject. He has broken away from the traditional texts on dermal pathology which are usually systematic compilations of thoughts and ideas of many students, teachers, and researchers in the field. This book is all Pinkus. It is his personal experience based on many years of teaching and studying dermal pathology so that the reader knows his personal thoughts, experience, and thinking on the whole spectrum of dermal pathology. I may disagree with Pinkus's interpretation of Jessner's lymphocytic infiltration or his concept that mycosis fungoides