Basaloid Follicular Hamartoma

Updated: July 25, 2024

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Background

Basaloid follicular hamartoma (BFH) is a rare benign adnexal neoplasm that is characteristically presented by small markedly asymptomatic papules or nodules on the skin. At first glance, it appears like “hamartoma” which is a harmless tissue growth, not an actual tumor, containing some disorganized but mature and native to the site tissues where this growth occurs. BFHs are the most ubiquitous areas affected by these linings between follicular cells. The facial areas such as the face, neck or scalp are most susceptible to fibrous deposits’ formation. They can also develop in other parts of the human body.

The lesions are histologically marked by basaloid cells that are formed in a nodular or serpiginous pattern, surrounded by stroma resembling the hair follicle and its outermost root appendage. Immunohistochemical staining may represent the expression of markers such as cytokeratins, carcinoembryonic antigen (CEA), and epithelial membrane antigen (EMA), the latter of which favors the follicular origin.

Epidemiology

It is extremely uncommon to have both hereditary and nonhereditary variants of linear unilaterally basaloid follicular hamartoma (LUBFH) and numerous basaloid follicular hamartoma (BFH). There are no records indicating the yearly occurrence or frequency of basaloid follicular hamartomas. Although sporadic occurrences are noted, they need to be distinguished from basal cell carcinoma.

Anatomy

Pathophysiology

Genetically, it is not so accurate to specify the exact gene, which causes the hamartoma, but it is known that its pathogenesis is associated with a violation of SHH signaling system, which is critical for basal cell carcinoma and stimulates increased Gli-1 transcription. The research results of genetics point to the fact that there is a q22.3 to q31 gene that contains a patched mutation. The presence of the genetic polymorphisms in the BHF clearly caused low expression of the PTCH1 gene message whereas the PCC group displayed higher level of the PTCH1 message suggesting that the abundance of SHH signaling decides the tumor phenotype considerably. It has been suggested that inflammation and follicular mucinosis are the etiology of BHF.

Etiology

Linear unilateral basaloid follicular hamartoma (LUBFH) is thought to result from a postzygotic, somatic mutation occurring during embryogenesis in a gene that has yet to be identified. This genetic anomaly would consequently be present solely in cells originating from the precursor cell that underwent the mutation.

Genetics

Prognostic Factors

The prognosis for SFH is usually excellent unless the patient has some related systemic disorders developed. Most basaloid follicular hamartomas are benign, superficial, and stable growths. In rare cases, the appearance of basal cell carcinoma within basaloid follicular hamartoma lesions has been experimentally brought to notice.

Clinical History

Age Group: The BFH can come at any age these days but is typically seen in the adults the most. In addition, a few cases of this strain in children are also being reported.

Physical Examination

Location: BFH lesions are mostly seen on the face and neck of a person, but their location can also be observed on other body parts like the scalp. The quantity and placement of the lesions is also observed.

Appearance: The BFH lesions characteristically present as red, tiny, hard nodules or papules on the skin. Different people may have a skin that is flesh-coloured, pink, or tinted slightly.

Symmetry: BFH spots either occur with similar patterns on both sides or in asymmetric distribution between side. Symmetrical lesions are usually has been found more in the benign growths, on the other hand, the asymmetrical pattern is often notifying the chances of more evaluation.

Age group

Associated comorbidity

The pathognomonic lesions of bacterial folliculitis in the form of asymptomatic papules or nodules manifest primarily on the face, neck, and scalp. As with any kind of glomerulopathy, no association of comorbidity comes with BFH, and it may, on occasion, coexist with other conditions, like basal cell carcinoma, trichoepithelioma, or genetic syndromes. 

Associated activity

The pathognomonic lesions of bacterial folliculitis in the form of asymptomatic papules or nodules manifest primarily on the face, neck, and scalp. The glomerulopathy has no association with the comorbidities that comes with BFH and it may coexist with other conditions like basal cell carcinoma, trichoepithelioma, or genetic syndromes.

Acuity of presentation

BFH’s symptoms are often barely noticeable or progress too slowly until the disease affects the vital organs.  The lesions may develop gradually and by just chance can be discovered during the general examination of the skin. Occasionally, BFH can also be sensation-provoking or uncomfortable because it can cause itching, pain, and sometimes even a touchy feeling.

Differential Diagnoses

  • Fibrofolliculoma
  • Tumor of the follicular infundibulum
  • Seborrheic keratosis
  • Poroma
  • Trichofolliculoma
  • Trichoblastic fibroma
  • Melanocytic nevus
  • Sebaceous hyperplasia
  • Merkel cell carcinoma

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Observation: Tissue pulsation within BFH complication is considered benign in a greater number of cases which do not need intrusive curing except periodical diagnostic evaluation. For patients, dermatologists will recommend periodic skin checkup to detect any development of lesion resembling the existing one or the same symptoms.

Surgical Excision: The surgical excision is the main remedial treatment for the appearance acceptable or symptomatic BFH lesions. This is the surgical operation where all the tumor and the surround healthy tissue is taken off as this is an assurance of no remaining tumour.

Laser Therapy: Different laser-centered techniques may appear, like carbon dioxide (CO2) laser or erbium: YAG laser, and their main purpose would be to remove superficial BFH lesions. Laser therapy is a commonly used treatment for lesions per vinculum, or in cosmetically sensitive areas, and for individuals who want to avoid invasive treatments.

Topical Treatments: Topical regimens consisting of corticosteroids or retinoids could be advised to deal BFH lesions-related symptoms, for instance itchiness or inflammation.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-a-non-pharmacological-approach-for-treating-basaloid-follicular-hamartoma

  • Surgical Excision: The principal cure for BFH is doing surgical removal of the tumor, especially if it is symptomatic, or cosmetically insulting, or demonstrates the features of the disease. Excision requires the entire lesion plus the margin of healthy tissue surrounding it to be removed for a successful targeted outcome.
  • Laser Therapy: Encapsulated by carbon dioxide (CO2) laser or erbium: YAG laser, laser treatment is appropriate in dealing with the superficial lesions of linea border cutis filiformis hirsutialis. By this way, it is possible to deal with those lesions which show in the cosmetically exposed area or those patients who wish the less invasive treatments.
  • Cryotherapy: This method is frequently applied for easily detectable and smaller lesions which may also be safe and recommended for patients who are not the candidates of surgical excision.
  • Electrosurgery: Electrosurgery commonly implies electrocautery or electrodessication, in that it creates electrical current of high frequency that allows destruction of BFH lesions. This model is applicable for the marks which are limited in size, but it is still not easy to remove them through this surgery.
  • Dermabrasion: It uses mechanical peeling to scrape the top layers of skin. These steps could improve BFH lesions through resizing them and yields less accurate results.

Role of topical steroids in the treatment of basaloid follicular hamartoma

Topical steroids may be used for basaloid follicular hamartoma (BFH) when they are only limited in the treatment, particularly when one wants to control the itching or inflammation associated with it. On the other hand, it should be highlighted that topical steroids, although a wonderful and highly effective treatment for seborrheic dermatitis are designed to suppress the disease, and not the entity of the lesion itself, BFH being a benign tumor-like growth of the hair follicles.

The therapeutic function of the best topical steroids during BFH treatment is evident owing to the provision of temporary relief from the symptoms. Topical corticosteroids work by attenuating and sacrificing some immune function in the skin. The different strengths and formulations of oral steroids allow for the treatment of various symptoms along with the selection of the steroid and strength being based on the severity of symptoms and the lesion location.

use-of-intervention-with-a-procedure-in-treating-basaloid-follicular-hamartoma

  • Surgical Excision: The pillar of surgical removal is the treatment of the BFH tumor type. The BFH lesion is removed along with part of the surrounding healthy tissue to carefully margin complete removal of the residual tumor during this procedure. Surgical excision is mainly preferred for symptoms which appear abruptly in an aggressive phase, for the ones which are in cosmetically affected regions.
  • Laser Therapy: The application of laser treatment may be performed by carbon dioxide (CO2) laser or erbium: YAG laser, the latter of which is mainly intended to destroy and remove multiple BFH lesions. One more powerful advantage of laser therapy is the ability to target lesions located in critical areas such as the face or in the patients who prefer minimally invasive treatments. It could also be used as a treatment in addition to excision surgery being done to improve cosmetic results after the procedure.
  • Cryotherapy: Since the artificial cryo-therapy uses liquid nitrogen to burn the BFH lesion, it leaves a blister which eventually falls off. Such a procedure is often applied for the removal of small surface lesions which will damage the treated areas. It is commonly used with such patients who do not have contraindications to surgical excision. Cryoablation, apart from being used as a primary treatment method, also can be applied as an adjunctive to surgically resected residual or recurrent TMN.
  • Electrosurgery: Electrocautery or electrodessication are other forms of electrosurgery that involve the use of high frequency electric currents at the beginning stages for destruction of BFH lesions. With this method, the doctor may have to limit to small lesions or lesions in areas which are difficult to dissect them. Electrosurgical device can be deployed to interfere with the remaining areas or recurring lesions by means of surgery.

use-of-phases-in-managing-basaloid-follicular-hamartoma

  • Assessment Phase: The first part is assessment of shaving or skin care lesions through clinical evaluation or employing dermoscopy if need be. Step by step some features like location, colour and texture are judged.
  • Diagnosis Phase: The diagnostic phase is the next step after that, where a definitive diagnosis can be achieved by histopathology examination, commonly using a biopsy. As a result, it will assure the character of the adhering and prevail in the possibility of the malignancy or other skin disorders.
  • Treatment Planning Phase: According to the diagnostic approach and the assessment, a treatment plan comes up (which gets acted upon). Such as the dimensions of the BFH lesion and if it is in an area that is bothersome to patient. Also, the presence of symptoms and the patient’s preferences and general health status.
  • Intervention Phase: A phase in which the formulated plan gets its real-life implementation with the selected intervention. Among those procedures can be surgical excision, as well as laser therapy, freeze-off by means of cryotherapy, and other methods that are intended to either remove or manage BFH lesions.
  • Monitoring Phase: After the intervention, the patient is monitored to know whether he/she is healing, recurring or is vulnerable to any other side effects. The frequency patients need to be seen for follow-up appointments depends on how the therapy is advancing.5.

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Basaloid Follicular Hamartoma

Updated : July 25, 2024

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Basaloid follicular hamartoma (BFH) is a rare benign adnexal neoplasm that is characteristically presented by small markedly asymptomatic papules or nodules on the skin. At first glance, it appears like “hamartoma” which is a harmless tissue growth, not an actual tumor, containing some disorganized but mature and native to the site tissues where this growth occurs. BFHs are the most ubiquitous areas affected by these linings between follicular cells. The facial areas such as the face, neck or scalp are most susceptible to fibrous deposits’ formation. They can also develop in other parts of the human body.

The lesions are histologically marked by basaloid cells that are formed in a nodular or serpiginous pattern, surrounded by stroma resembling the hair follicle and its outermost root appendage. Immunohistochemical staining may represent the expression of markers such as cytokeratins, carcinoembryonic antigen (CEA), and epithelial membrane antigen (EMA), the latter of which favors the follicular origin.

It is extremely uncommon to have both hereditary and nonhereditary variants of linear unilaterally basaloid follicular hamartoma (LUBFH) and numerous basaloid follicular hamartoma (BFH). There are no records indicating the yearly occurrence or frequency of basaloid follicular hamartomas. Although sporadic occurrences are noted, they need to be distinguished from basal cell carcinoma.

Genetically, it is not so accurate to specify the exact gene, which causes the hamartoma, but it is known that its pathogenesis is associated with a violation of SHH signaling system, which is critical for basal cell carcinoma and stimulates increased Gli-1 transcription. The research results of genetics point to the fact that there is a q22.3 to q31 gene that contains a patched mutation. The presence of the genetic polymorphisms in the BHF clearly caused low expression of the PTCH1 gene message whereas the PCC group displayed higher level of the PTCH1 message suggesting that the abundance of SHH signaling decides the tumor phenotype considerably. It has been suggested that inflammation and follicular mucinosis are the etiology of BHF.

Linear unilateral basaloid follicular hamartoma (LUBFH) is thought to result from a postzygotic, somatic mutation occurring during embryogenesis in a gene that has yet to be identified. This genetic anomaly would consequently be present solely in cells originating from the precursor cell that underwent the mutation.

The prognosis for SFH is usually excellent unless the patient has some related systemic disorders developed. Most basaloid follicular hamartomas are benign, superficial, and stable growths. In rare cases, the appearance of basal cell carcinoma within basaloid follicular hamartoma lesions has been experimentally brought to notice.

Age Group: The BFH can come at any age these days but is typically seen in the adults the most. In addition, a few cases of this strain in children are also being reported.

Location: BFH lesions are mostly seen on the face and neck of a person, but their location can also be observed on other body parts like the scalp. The quantity and placement of the lesions is also observed.

Appearance: The BFH lesions characteristically present as red, tiny, hard nodules or papules on the skin. Different people may have a skin that is flesh-coloured, pink, or tinted slightly.

Symmetry: BFH spots either occur with similar patterns on both sides or in asymmetric distribution between side. Symmetrical lesions are usually has been found more in the benign growths, on the other hand, the asymmetrical pattern is often notifying the chances of more evaluation.

The pathognomonic lesions of bacterial folliculitis in the form of asymptomatic papules or nodules manifest primarily on the face, neck, and scalp. As with any kind of glomerulopathy, no association of comorbidity comes with BFH, and it may, on occasion, coexist with other conditions, like basal cell carcinoma, trichoepithelioma, or genetic syndromes. 

BFH’s symptoms are often barely noticeable or progress too slowly until the disease affects the vital organs.  The lesions may develop gradually and by just chance can be discovered during the general examination of the skin. Occasionally, BFH can also be sensation-provoking or uncomfortable because it can cause itching, pain, and sometimes even a touchy feeling.

The pathognomonic lesions of bacterial folliculitis in the form of asymptomatic papules or nodules manifest primarily on the face, neck, and scalp. The glomerulopathy has no association with the comorbidities that comes with BFH and it may coexist with other conditions like basal cell carcinoma, trichoepithelioma, or genetic syndromes.

  • Fibrofolliculoma
  • Tumor of the follicular infundibulum
  • Seborrheic keratosis
  • Poroma
  • Trichofolliculoma
  • Trichoblastic fibroma
  • Melanocytic nevus
  • Sebaceous hyperplasia
  • Merkel cell carcinoma

Observation: Tissue pulsation within BFH complication is considered benign in a greater number of cases which do not need intrusive curing except periodical diagnostic evaluation. For patients, dermatologists will recommend periodic skin checkup to detect any development of lesion resembling the existing one or the same symptoms.

Surgical Excision: The surgical excision is the main remedial treatment for the appearance acceptable or symptomatic BFH lesions. This is the surgical operation where all the tumor and the surround healthy tissue is taken off as this is an assurance of no remaining tumour.

Laser Therapy: Different laser-centered techniques may appear, like carbon dioxide (CO2) laser or erbium: YAG laser, and their main purpose would be to remove superficial BFH lesions. Laser therapy is a commonly used treatment for lesions per vinculum, or in cosmetically sensitive areas, and for individuals who want to avoid invasive treatments.

Topical Treatments: Topical regimens consisting of corticosteroids or retinoids could be advised to deal BFH lesions-related symptoms, for instance itchiness or inflammation.

Dermatology, General

Surgery, General

  • Surgical Excision: The principal cure for BFH is doing surgical removal of the tumor, especially if it is symptomatic, or cosmetically insulting, or demonstrates the features of the disease. Excision requires the entire lesion plus the margin of healthy tissue surrounding it to be removed for a successful targeted outcome.
  • Laser Therapy: Encapsulated by carbon dioxide (CO2) laser or erbium: YAG laser, laser treatment is appropriate in dealing with the superficial lesions of linea border cutis filiformis hirsutialis. By this way, it is possible to deal with those lesions which show in the cosmetically exposed area or those patients who wish the less invasive treatments.
  • Cryotherapy: This method is frequently applied for easily detectable and smaller lesions which may also be safe and recommended for patients who are not the candidates of surgical excision.
  • Electrosurgery: Electrosurgery commonly implies electrocautery or electrodessication, in that it creates electrical current of high frequency that allows destruction of BFH lesions. This model is applicable for the marks which are limited in size, but it is still not easy to remove them through this surgery.
  • Dermabrasion: It uses mechanical peeling to scrape the top layers of skin. These steps could improve BFH lesions through resizing them and yields less accurate results.

Dermatology, General

Surgery, General

Topical steroids may be used for basaloid follicular hamartoma (BFH) when they are only limited in the treatment, particularly when one wants to control the itching or inflammation associated with it. On the other hand, it should be highlighted that topical steroids, although a wonderful and highly effective treatment for seborrheic dermatitis are designed to suppress the disease, and not the entity of the lesion itself, BFH being a benign tumor-like growth of the hair follicles.

The therapeutic function of the best topical steroids during BFH treatment is evident owing to the provision of temporary relief from the symptoms. Topical corticosteroids work by attenuating and sacrificing some immune function in the skin. The different strengths and formulations of oral steroids allow for the treatment of various symptoms along with the selection of the steroid and strength being based on the severity of symptoms and the lesion location.

Dermatology, General

Surgery, General

  • Surgical Excision: The pillar of surgical removal is the treatment of the BFH tumor type. The BFH lesion is removed along with part of the surrounding healthy tissue to carefully margin complete removal of the residual tumor during this procedure. Surgical excision is mainly preferred for symptoms which appear abruptly in an aggressive phase, for the ones which are in cosmetically affected regions.
  • Laser Therapy: The application of laser treatment may be performed by carbon dioxide (CO2) laser or erbium: YAG laser, the latter of which is mainly intended to destroy and remove multiple BFH lesions. One more powerful advantage of laser therapy is the ability to target lesions located in critical areas such as the face or in the patients who prefer minimally invasive treatments. It could also be used as a treatment in addition to excision surgery being done to improve cosmetic results after the procedure.
  • Cryotherapy: Since the artificial cryo-therapy uses liquid nitrogen to burn the BFH lesion, it leaves a blister which eventually falls off. Such a procedure is often applied for the removal of small surface lesions which will damage the treated areas. It is commonly used with such patients who do not have contraindications to surgical excision. Cryoablation, apart from being used as a primary treatment method, also can be applied as an adjunctive to surgically resected residual or recurrent TMN.
  • Electrosurgery: Electrocautery or electrodessication are other forms of electrosurgery that involve the use of high frequency electric currents at the beginning stages for destruction of BFH lesions. With this method, the doctor may have to limit to small lesions or lesions in areas which are difficult to dissect them. Electrosurgical device can be deployed to interfere with the remaining areas or recurring lesions by means of surgery.

Dermatology, General

Physical Medicine and Rehabilitation

  • Assessment Phase: The first part is assessment of shaving or skin care lesions through clinical evaluation or employing dermoscopy if need be. Step by step some features like location, colour and texture are judged.
  • Diagnosis Phase: The diagnostic phase is the next step after that, where a definitive diagnosis can be achieved by histopathology examination, commonly using a biopsy. As a result, it will assure the character of the adhering and prevail in the possibility of the malignancy or other skin disorders.
  • Treatment Planning Phase: According to the diagnostic approach and the assessment, a treatment plan comes up (which gets acted upon). Such as the dimensions of the BFH lesion and if it is in an area that is bothersome to patient. Also, the presence of symptoms and the patient’s preferences and general health status.
  • Intervention Phase: A phase in which the formulated plan gets its real-life implementation with the selected intervention. Among those procedures can be surgical excision, as well as laser therapy, freeze-off by means of cryotherapy, and other methods that are intended to either remove or manage BFH lesions.
  • Monitoring Phase: After the intervention, the patient is monitored to know whether he/she is healing, recurring or is vulnerable to any other side effects. The frequency patients need to be seen for follow-up appointments depends on how the therapy is advancing.5.

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