Patient Presentation
A 60-year-old man presented with a 2-year history of extensive periungual erythema and scaling of the right index finger, for which he previously received treatment with liquid nitrogen three times but had no significant improvement. He denied trauma, chronic nail infection, arsenic exposure or radiation to this particular finger. The patient was immunocompetent. Physical examination revealed a well-demarcated erythematous patch with scaling involving the medial, lateral and proximal periungual areas of the finger (see photo above). There were also a few distinct warty papules at the level of the mid-nail plate. Attempt at mechanical scraping of these papules failed and elicited a painful sensation for the patient. In addition, there was a distal onychodystrophy of the fingernail with accumulation of keratotic debris in the hyponychium. Biopsy results are pending.
What’s Your Diagnosis?
Diagnosis: Bowen’s Disease (BD) of the Nail
Bowen’s disease of the nail is a rare cutaneous disorder that presents as periungual or subungual verrucous plaques, erosions and ulcerations, with nail discoloration, dystrophy or onycholysis. This disease of the nail is a form of intraepidermal (in situ) squamous cell carcinoma (SCC) of the nail apparatus. The term, epidermoid carcinoma of the nail, which encompasses description of both, BD and frank SCC of the nail, is also encountered in the literature.1
Velpeau first reported a case of epidermoid carcinoma of the nail bed of the index finger in 1850.2 In 1912, Bowen described the special form of SCC in situ of the skin and mucous membrane, which currently bears his name (Bowen’s disease).3 Finally, in 1972 Coskey et al described four cases of BD of the nail as verrucous growths, fissures and ulcerations involving the nail bed with destruction of the associated nail plate.4
The Nail Unit
It is crucial to comprehend the anatomy of, and terminology used to describe the nail unit. The nail apparatus consists of five main structures: the nail plate, the proximal nail fold, the nail matrix, the nail bed, and the hyponychium.5 BD of the nail indicates in situ SCC that may entail any component of the nail apparatus. The periungual and subungual lesions are regional terms used to describe disease around and under the nail, respectively. Initial BD of the nail may spread to periungual or subungual areas or vice versa. In our patient, BD lesions involved the nail and periungual regions.
Clinicophathologic Characteristics
The exact incidence of BD of the nail is unknown. However, epidermoid carcinoma is considered the most common type of malignancy of the nail unit. BD of the nail usually manifests in elderly males (around the fifth decade) and commonly involves the fingers.
BD of the nail presents with verrucous, scaly, crusting, erythematous or fissuring lesions that may involve any portion of the nail apparatus with associated onycholysis, nail dystrophy, longitudinal erythronychia or melanonychia (red or black longitudinal nail streak).6,7 Findings of bleeding, ulceration, or a nodule are more suggestive of invasive SCC than BD. Bowen’s disease of the nail may involve more than one digit on multiple extremities simultaneously or sequentially.8
The differential diagnosis of BD of the nail includes onychomycosis, paronychia, verruca vulgaris, eczema, pyogenic granuloma, glomus tumor, verrucous tuberculosis, subungual exostosis, onychomatricoma, dermatitis vegetans, amelanotic malignant melanoma, keratoacanthoma, fibrokeratoma, gouty tophus and SCC.7,9-11 Due to its similarity to these disorders, diagnosis of BD of the nail is difficult and is often delayed (1 to 15 years).12 Our patient had lesions for 2 years. It is critical to biopsy all chronic and recalcitrant lesions of the nail apparatus to rule out BD.
Diagnosis of BD of the nail is via clinical findings and biopsy confirmation. For optimal biopsy results, knowledge of nail anatomy and physiology, as well as good patient selection, preparation and proper technique are necessary.13 The matrix is a germinating portion of the nail that requires special care, because its damage may permanently effect nail formation and function. The nail apparatus is very vascular and well-innervated, hence, the need for appropriate anesthesia and hemostasis.
The histopathology of BD lesions is characterized by hyperkeratosis, parakeratosis, loss of orderly maturation, polarity and granular layer, and keratinocytic atypia involving the entire acanthotic epithelial layer.14 The atypia and dyskeratosis are confined to the epidermis.15 However, some microscopic specimens of BD may simultaneously demonstrate features of invasive SCC in other areas of the lesion.9 Microinvasion is common in long-term BD with reports of invasive carcinoma in approximately 15% of BD cases.16
BD of the nail is considered a persistent and localized lesion with small potential for invasion and development into SCC and very low probability for metastasis. However, BD of the nail is held to be more aggressive than its skin equivalent with reports of few fatal metastases.12,17 Recurrent epidermoid carcinoma of the nail has greater risk of metastasis.18 A common complication of BD of the nail is secondary infection.
An Overview of Etiology
The cause of BD of the nail is uncertain. Trauma, chronic paronychia, ionizing radiation, infectious agents, ultraviolet exposure, arsenic or pesticide exposure, and immunosuppression are a few proposed etiologic factors.8,19,20
Human papilloma virus (HPV) DNA has been identified in BD and SCC lesions. The theory is that HPV has an oncogenic potential and may play a significant role in the development of BD and invasive SCC of the nail and other cutaneous regions. The proposed pathogenesis describes the integration of HPV DNA within the host chromosomes that may cause transformation of these cells into malignant state.21 The specific HPV types that were found within epidermoid carcinoma of the nail include 16, 34 and 35.
Several reports have connected epidermoid carcinoma of the nail and BD of the anogenital area though HPV-16 infection in both regions. The postulated transmission is autoinoculation from anogenital area to the digit or vice versa.12,20 Due to a well-known close association between HPV-16 and cervical neoplasia, recently noted HPV-16 in epidermoid carcinoma lesions of the nail, and reported cases of BD of the nail in association with uterine-cervical carcinoma, it has been suggested to perform genital examination when managing patients with epidermoid carcinoma of the nail.17
Also, Tosti et al noted HPV-associated epidermoid carcinoma of the nail in HIV patients and stipulated that these patients’ decreased cell-mediated immune state may predispose them to developing these lesions.20 Since SCC may arise from BD of the nail, it is important to consider HIV or another immunocompromised condition in young adults with these lesions.
Sau et al have proposed the pathogenesis behind polydactylous BD of the nail as an autochthonous mechanism related to factors such as trauma and radiation.9
Several earlier investigative reports projected a relationship between BD and internal malignancy.22 However, larger studies did not support the idea that BD is a skin marker for internal malignancy and therefore, discouraged routine investigation for such malignancy in patients with BD.23,24
Managing This Condition
Various therapeutic modalities have been employed for BD of the nail: electrodessication and curettage, 5% fluorouracil ointment (Efudex), cryosurgery, and radiotherapy.17 Most of these treatments have not been successful, with high recurrence rates.Simple excision of nail bed and matrix is successful in small and localized BD lesions.1 The CO2 laser therapy for periungual BD has a demonstrated 80% cure rate, with less scarring and contractures when compared with surgical excision.25 Recently, imiquimod (Aldara 5% cream) has been used as a viable option for BD of the nail, especially recurrent disease.26
Mohs surgery is regarded as the optimum method of therapy with cure rate up to 96%.27 This success is largely due to adequate depth of tumor resection, great preservation of normal digit function, and excellent cosmetic results with healing by secondary intention.27 Although the 5-year recurrence rate after Mohs surgery is small (about 3%), it is still judicious to follow the patient closely to assess for potential relapse.18
If there is any suspicion for invasive SCC, evaluate the patient’s regional lymph nodes for possible metastasis and obtain radiologic study of the digit(s) to assess for bone invasion.28 Amputation of the affected digit is considered a drastic measure with significant effects on hand function. However, if there is evidence of bone involvement, amputation is still judged a preferred management.28
Do you have a case you’d like to see published in this column? If so, please send a write-up (about 600 to 800 words) and an image of the patient’s condition. You may also include a follow-up image of the patient to accompany the discussion portion. Please send materials to: Dr. Amor Khachemoune, Georgetown University Medical Center, Division of Dermatology, 3800 Reservoir Road, NW 5PHC, Washington, DC 20007. Or, e-mail them to amorkh@pol.net.
Patient Presentation
A 60-year-old man presented with a 2-year history of extensive periungual erythema and scaling of the right index finger, for which he previously received treatment with liquid nitrogen three times but had no significant improvement. He denied trauma, chronic nail infection, arsenic exposure or radiation to this particular finger. The patient was immunocompetent. Physical examination revealed a well-demarcated erythematous patch with scaling involving the medial, lateral and proximal periungual areas of the finger (see photo above). There were also a few distinct warty papules at the level of the mid-nail plate. Attempt at mechanical scraping of these papules failed and elicited a painful sensation for the patient. In addition, there was a distal onychodystrophy of the fingernail with accumulation of keratotic debris in the hyponychium. Biopsy results are pending.
What’s Your Diagnosis?
Diagnosis: Bowen’s Disease (BD) of the Nail
Bowen’s disease of the nail is a rare cutaneous disorder that presents as periungual or subungual verrucous plaques, erosions and ulcerations, with nail discoloration, dystrophy or onycholysis. This disease of the nail is a form of intraepidermal (in situ) squamous cell carcinoma (SCC) of the nail apparatus. The term, epidermoid carcinoma of the nail, which encompasses description of both, BD and frank SCC of the nail, is also encountered in the literature.1
Velpeau first reported a case of epidermoid carcinoma of the nail bed of the index finger in 1850.2 In 1912, Bowen described the special form of SCC in situ of the skin and mucous membrane, which currently bears his name (Bowen’s disease).3 Finally, in 1972 Coskey et al described four cases of BD of the nail as verrucous growths, fissures and ulcerations involving the nail bed with destruction of the associated nail plate.4
The Nail Unit
It is crucial to comprehend the anatomy of, and terminology used to describe the nail unit. The nail apparatus consists of five main structures: the nail plate, the proximal nail fold, the nail matrix, the nail bed, and the hyponychium.5 BD of the nail indicates in situ SCC that may entail any component of the nail apparatus. The periungual and subungual lesions are regional terms used to describe disease around and under the nail, respectively. Initial BD of the nail may spread to periungual or subungual areas or vice versa. In our patient, BD lesions involved the nail and periungual regions.
Clinicophathologic Characteristics
The exact incidence of BD of the nail is unknown. However, epidermoid carcinoma is considered the most common type of malignancy of the nail unit. BD of the nail usually manifests in elderly males (around the fifth decade) and commonly involves the fingers.
BD of the nail presents with verrucous, scaly, crusting, erythematous or fissuring lesions that may involve any portion of the nail apparatus with associated onycholysis, nail dystrophy, longitudinal erythronychia or melanonychia (red or black longitudinal nail streak).6,7 Findings of bleeding, ulceration, or a nodule are more suggestive of invasive SCC than BD. Bowen’s disease of the nail may involve more than one digit on multiple extremities simultaneously or sequentially.8
The differential diagnosis of BD of the nail includes onychomycosis, paronychia, verruca vulgaris, eczema, pyogenic granuloma, glomus tumor, verrucous tuberculosis, subungual exostosis, onychomatricoma, dermatitis vegetans, amelanotic malignant melanoma, keratoacanthoma, fibrokeratoma, gouty tophus and SCC.7,9-11 Due to its similarity to these disorders, diagnosis of BD of the nail is difficult and is often delayed (1 to 15 years).12 Our patient had lesions for 2 years. It is critical to biopsy all chronic and recalcitrant lesions of the nail apparatus to rule out BD.
Diagnosis of BD of the nail is via clinical findings and biopsy confirmation. For optimal biopsy results, knowledge of nail anatomy and physiology, as well as good patient selection, preparation and proper technique are necessary.13 The matrix is a germinating portion of the nail that requires special care, because its damage may permanently effect nail formation and function. The nail apparatus is very vascular and well-innervated, hence, the need for appropriate anesthesia and hemostasis.
The histopathology of BD lesions is characterized by hyperkeratosis, parakeratosis, loss of orderly maturation, polarity and granular layer, and keratinocytic atypia involving the entire acanthotic epithelial layer.14 The atypia and dyskeratosis are confined to the epidermis.15 However, some microscopic specimens of BD may simultaneously demonstrate features of invasive SCC in other areas of the lesion.9 Microinvasion is common in long-term BD with reports of invasive carcinoma in approximately 15% of BD cases.16
BD of the nail is considered a persistent and localized lesion with small potential for invasion and development into SCC and very low probability for metastasis. However, BD of the nail is held to be more aggressive than its skin equivalent with reports of few fatal metastases.12,17 Recurrent epidermoid carcinoma of the nail has greater risk of metastasis.18 A common complication of BD of the nail is secondary infection.
An Overview of Etiology
The cause of BD of the nail is uncertain. Trauma, chronic paronychia, ionizing radiation, infectious agents, ultraviolet exposure, arsenic or pesticide exposure, and immunosuppression are a few proposed etiologic factors.8,19,20
Human papilloma virus (HPV) DNA has been identified in BD and SCC lesions. The theory is that HPV has an oncogenic potential and may play a significant role in the development of BD and invasive SCC of the nail and other cutaneous regions. The proposed pathogenesis describes the integration of HPV DNA within the host chromosomes that may cause transformation of these cells into malignant state.21 The specific HPV types that were found within epidermoid carcinoma of the nail include 16, 34 and 35.
Several reports have connected epidermoid carcinoma of the nail and BD of the anogenital area though HPV-16 infection in both regions. The postulated transmission is autoinoculation from anogenital area to the digit or vice versa.12,20 Due to a well-known close association between HPV-16 and cervical neoplasia, recently noted HPV-16 in epidermoid carcinoma lesions of the nail, and reported cases of BD of the nail in association with uterine-cervical carcinoma, it has been suggested to perform genital examination when managing patients with epidermoid carcinoma of the nail.17
Also, Tosti et al noted HPV-associated epidermoid carcinoma of the nail in HIV patients and stipulated that these patients’ decreased cell-mediated immune state may predispose them to developing these lesions.20 Since SCC may arise from BD of the nail, it is important to consider HIV or another immunocompromised condition in young adults with these lesions.
Sau et al have proposed the pathogenesis behind polydactylous BD of the nail as an autochthonous mechanism related to factors such as trauma and radiation.9
Several earlier investigative reports projected a relationship between BD and internal malignancy.22 However, larger studies did not support the idea that BD is a skin marker for internal malignancy and therefore, discouraged routine investigation for such malignancy in patients with BD.23,24
Managing This Condition
Various therapeutic modalities have been employed for BD of the nail: electrodessication and curettage, 5% fluorouracil ointment (Efudex), cryosurgery, and radiotherapy.17 Most of these treatments have not been successful, with high recurrence rates.Simple excision of nail bed and matrix is successful in small and localized BD lesions.1 The CO2 laser therapy for periungual BD has a demonstrated 80% cure rate, with less scarring and contractures when compared with surgical excision.25 Recently, imiquimod (Aldara 5% cream) has been used as a viable option for BD of the nail, especially recurrent disease.26
Mohs surgery is regarded as the optimum method of therapy with cure rate up to 96%.27 This success is largely due to adequate depth of tumor resection, great preservation of normal digit function, and excellent cosmetic results with healing by secondary intention.27 Although the 5-year recurrence rate after Mohs surgery is small (about 3%), it is still judicious to follow the patient closely to assess for potential relapse.18
If there is any suspicion for invasive SCC, evaluate the patient’s regional lymph nodes for possible metastasis and obtain radiologic study of the digit(s) to assess for bone invasion.28 Amputation of the affected digit is considered a drastic measure with significant effects on hand function. However, if there is evidence of bone involvement, amputation is still judged a preferred management.28
Do you have a case you’d like to see published in this column? If so, please send a write-up (about 600 to 800 words) and an image of the patient’s condition. You may also include a follow-up image of the patient to accompany the discussion portion. Please send materials to: Dr. Amor Khachemoune, Georgetown University Medical Center, Division of Dermatology, 3800 Reservoir Road, NW 5PHC, Washington, DC 20007. Or, e-mail them to amorkh@pol.net.
Patient Presentation
A 60-year-old man presented with a 2-year history of extensive periungual erythema and scaling of the right index finger, for which he previously received treatment with liquid nitrogen three times but had no significant improvement. He denied trauma, chronic nail infection, arsenic exposure or radiation to this particular finger. The patient was immunocompetent. Physical examination revealed a well-demarcated erythematous patch with scaling involving the medial, lateral and proximal periungual areas of the finger (see photo above). There were also a few distinct warty papules at the level of the mid-nail plate. Attempt at mechanical scraping of these papules failed and elicited a painful sensation for the patient. In addition, there was a distal onychodystrophy of the fingernail with accumulation of keratotic debris in the hyponychium. Biopsy results are pending.
What’s Your Diagnosis?
Diagnosis: Bowen’s Disease (BD) of the Nail
Bowen’s disease of the nail is a rare cutaneous disorder that presents as periungual or subungual verrucous plaques, erosions and ulcerations, with nail discoloration, dystrophy or onycholysis. This disease of the nail is a form of intraepidermal (in situ) squamous cell carcinoma (SCC) of the nail apparatus. The term, epidermoid carcinoma of the nail, which encompasses description of both, BD and frank SCC of the nail, is also encountered in the literature.1
Velpeau first reported a case of epidermoid carcinoma of the nail bed of the index finger in 1850.2 In 1912, Bowen described the special form of SCC in situ of the skin and mucous membrane, which currently bears his name (Bowen’s disease).3 Finally, in 1972 Coskey et al described four cases of BD of the nail as verrucous growths, fissures and ulcerations involving the nail bed with destruction of the associated nail plate.4
The Nail Unit
It is crucial to comprehend the anatomy of, and terminology used to describe the nail unit. The nail apparatus consists of five main structures: the nail plate, the proximal nail fold, the nail matrix, the nail bed, and the hyponychium.5 BD of the nail indicates in situ SCC that may entail any component of the nail apparatus. The periungual and subungual lesions are regional terms used to describe disease around and under the nail, respectively. Initial BD of the nail may spread to periungual or subungual areas or vice versa. In our patient, BD lesions involved the nail and periungual regions.
Clinicophathologic Characteristics
The exact incidence of BD of the nail is unknown. However, epidermoid carcinoma is considered the most common type of malignancy of the nail unit. BD of the nail usually manifests in elderly males (around the fifth decade) and commonly involves the fingers.
BD of the nail presents with verrucous, scaly, crusting, erythematous or fissuring lesions that may involve any portion of the nail apparatus with associated onycholysis, nail dystrophy, longitudinal erythronychia or melanonychia (red or black longitudinal nail streak).6,7 Findings of bleeding, ulceration, or a nodule are more suggestive of invasive SCC than BD. Bowen’s disease of the nail may involve more than one digit on multiple extremities simultaneously or sequentially.8
The differential diagnosis of BD of the nail includes onychomycosis, paronychia, verruca vulgaris, eczema, pyogenic granuloma, glomus tumor, verrucous tuberculosis, subungual exostosis, onychomatricoma, dermatitis vegetans, amelanotic malignant melanoma, keratoacanthoma, fibrokeratoma, gouty tophus and SCC.7,9-11 Due to its similarity to these disorders, diagnosis of BD of the nail is difficult and is often delayed (1 to 15 years).12 Our patient had lesions for 2 years. It is critical to biopsy all chronic and recalcitrant lesions of the nail apparatus to rule out BD.
Diagnosis of BD of the nail is via clinical findings and biopsy confirmation. For optimal biopsy results, knowledge of nail anatomy and physiology, as well as good patient selection, preparation and proper technique are necessary.13 The matrix is a germinating portion of the nail that requires special care, because its damage may permanently effect nail formation and function. The nail apparatus is very vascular and well-innervated, hence, the need for appropriate anesthesia and hemostasis.
The histopathology of BD lesions is characterized by hyperkeratosis, parakeratosis, loss of orderly maturation, polarity and granular layer, and keratinocytic atypia involving the entire acanthotic epithelial layer.14 The atypia and dyskeratosis are confined to the epidermis.15 However, some microscopic specimens of BD may simultaneously demonstrate features of invasive SCC in other areas of the lesion.9 Microinvasion is common in long-term BD with reports of invasive carcinoma in approximately 15% of BD cases.16
BD of the nail is considered a persistent and localized lesion with small potential for invasion and development into SCC and very low probability for metastasis. However, BD of the nail is held to be more aggressive than its skin equivalent with reports of few fatal metastases.12,17 Recurrent epidermoid carcinoma of the nail has greater risk of metastasis.18 A common complication of BD of the nail is secondary infection.
An Overview of Etiology
The cause of BD of the nail is uncertain. Trauma, chronic paronychia, ionizing radiation, infectious agents, ultraviolet exposure, arsenic or pesticide exposure, and immunosuppression are a few proposed etiologic factors.8,19,20
Human papilloma virus (HPV) DNA has been identified in BD and SCC lesions. The theory is that HPV has an oncogenic potential and may play a significant role in the development of BD and invasive SCC of the nail and other cutaneous regions. The proposed pathogenesis describes the integration of HPV DNA within the host chromosomes that may cause transformation of these cells into malignant state.21 The specific HPV types that were found within epidermoid carcinoma of the nail include 16, 34 and 35.
Several reports have connected epidermoid carcinoma of the nail and BD of the anogenital area though HPV-16 infection in both regions. The postulated transmission is autoinoculation from anogenital area to the digit or vice versa.12,20 Due to a well-known close association between HPV-16 and cervical neoplasia, recently noted HPV-16 in epidermoid carcinoma lesions of the nail, and reported cases of BD of the nail in association with uterine-cervical carcinoma, it has been suggested to perform genital examination when managing patients with epidermoid carcinoma of the nail.17
Also, Tosti et al noted HPV-associated epidermoid carcinoma of the nail in HIV patients and stipulated that these patients’ decreased cell-mediated immune state may predispose them to developing these lesions.20 Since SCC may arise from BD of the nail, it is important to consider HIV or another immunocompromised condition in young adults with these lesions.
Sau et al have proposed the pathogenesis behind polydactylous BD of the nail as an autochthonous mechanism related to factors such as trauma and radiation.9
Several earlier investigative reports projected a relationship between BD and internal malignancy.22 However, larger studies did not support the idea that BD is a skin marker for internal malignancy and therefore, discouraged routine investigation for such malignancy in patients with BD.23,24
Managing This Condition
Various therapeutic modalities have been employed for BD of the nail: electrodessication and curettage, 5% fluorouracil ointment (Efudex), cryosurgery, and radiotherapy.17 Most of these treatments have not been successful, with high recurrence rates.Simple excision of nail bed and matrix is successful in small and localized BD lesions.1 The CO2 laser therapy for periungual BD has a demonstrated 80% cure rate, with less scarring and contractures when compared with surgical excision.25 Recently, imiquimod (Aldara 5% cream) has been used as a viable option for BD of the nail, especially recurrent disease.26
Mohs surgery is regarded as the optimum method of therapy with cure rate up to 96%.27 This success is largely due to adequate depth of tumor resection, great preservation of normal digit function, and excellent cosmetic results with healing by secondary intention.27 Although the 5-year recurrence rate after Mohs surgery is small (about 3%), it is still judicious to follow the patient closely to assess for potential relapse.18
If there is any suspicion for invasive SCC, evaluate the patient’s regional lymph nodes for possible metastasis and obtain radiologic study of the digit(s) to assess for bone invasion.28 Amputation of the affected digit is considered a drastic measure with significant effects on hand function. However, if there is evidence of bone involvement, amputation is still judged a preferred management.28
Do you have a case you’d like to see published in this column? If so, please send a write-up (about 600 to 800 words) and an image of the patient’s condition. You may also include a follow-up image of the patient to accompany the discussion portion. Please send materials to: Dr. Amor Khachemoune, Georgetown University Medical Center, Division of Dermatology, 3800 Reservoir Road, NW 5PHC, Washington, DC 20007. Or, e-mail them to amorkh@pol.net.
