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Nevus sebaceus in neonate
P807
P809
Nevus sebaceus in neonate Loan Towersey, MD, PhD, Instituto de Dermatologia Prof. Rubem David Azulay, Rio de Janeiro, RJ, Brazil; Mayra Carrijo Rochael, MD, PhD, Universidade Federal Fluminense, Niteroi, RJ, Brazil; Mariana Sasse, MD, Instituto de Dermatologia Prof. Rubem David Azulay, Rio de Janeiro, RJ, Brazil; Timothy George Berger, MD, University of California, San Francisco, CA, United States
Interferon beta-1a-associated neutrophilic panniculitis mimicking cellulitis Megan Weber, MD, Mayo Clinic, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States; Karen Warschaw, MD, Mayo Clinic, Scottsdale, AZ, United States; Susan Laman, MD, Mayo Clinic, Scottsdale, AZ, United States
Introduction: Adnexal tumors form a group of neoplasms with a predominant cell lineage of adnexal structure morphologic differentiation: pilosebaceous units and eccrine or apocrine glands. Compound nevus have more than one cell lineage and when all cell lineages are present, it is called organoid nevus. Nevus sebaceus was described by Jadassohn in 1895. This organoid nevus is a complex congenital hamartoma usually located on the scalp and face and presents different chronologic stages (prepubertal, pubertal, and postpubertal). Atypical aspects of nevus sebaceus in a newborn are highlighted. Case report: A 3-day-old boy, born and living in S~ ao Gonc¸alo, RJ, Brazil, was brought for consultation presenting a congenital tumor on the scalp. On the vertex, there was raised plaque formed by the gathering of papillated yellowish millimetric lesions. It measured 3 cm in diameter. A papillation was removed and sent for exam when the boy was 10 days old. Diagnostic hypothesis included nevus sebaceus, cylindroma, and syringocystoadenoma papilliferum. Histopathologic exam disclosed a follicle-sebaceous hamartoma. The tumor was completely removed when the boy was 4 months old and the hypothesis of nevus sebaceus was confirmed on exam. The first biopsy review detected sebaceous cells in a rudimentary infundibulum, a very lesion of nevus sebaceous. Discussion: Most adnexal tumors are benign neoplasms. Associated multiple internal abnormalities include internal neoplasms as seen in MuireTorre syndrome (sebaceous tumor), and Cowden’s disease (trichilemmoma). Adnexal tumors have a complex histogenesis. Nevus sebaceous may develop carcinomas in the late stages and these often show adnexal differentiation. Adnexal neoplasms, benign or malignant, may develop in 20% to 40% of these lesions. Early surgical removal is the therapy of choice. The different stages of nevus sebaceous observed in histopathology correspond to changes observed clinically. In early nevus sebaceous, the flat yellowish plaque shows epidermal hyperplasia, infundibular sebaceous cell lobules, rudimentary hair follicles, the presence of muscles of hair erection, and the absence of appocrine glands in the deep dermis. In neonates, because of the influence of maternal hormones, there may be mature sebaceous glands. Epithelial and connective tissue elements in the organoid nevus are also influenced. This may explain the atypical exophytic papillated tumor seen in this report.
A 58-year-old male with rheumatoid arthritis and multiple sclerosis presented with a 3-day history of progressive redness, swelling, and pain of the right thigh. He was admitted to the hospital for presumed cellulitis. The patient was taking multiple immunomodulating medications, including methotrexate, rituximab, and interferon (IFN) beta-1a. Computed tomography revealed subcutaneous thickening and edema of the thigh consistent with cellulitis. There was no evidence of abscess. The patient was started on broad spectrum intravenous antibacterial and antifungal therapy and his immunomodulating medications were held. After nearly 2 weeks, there was no improvement, and dermatology was consulted. Further history revealed the patient had been on IFN beta-1a for 4 years. He preferentially used his right thigh for injections of IFN, as he had previously required debridement and skin grafting to the left thigh because of a bacterial infection at that site. Physical exam on day 12 of hospitalization revealed a 12-cm erythematous, woody, indurated plaque with no overlying epidermal change. The induration extended beyond the erythema, both medially and laterally. A diagnosis of injection site reaction was favored. Punch biopsies were performed for microscopy and culture. Pathology revealed focal neutrophilic inflammation involving the eccrine glands. Methenamine silver and acid-fast stains were negative. The patient was sent home 2 days later and followed up in dermatology clinic. The physical exam 2 weeks after hospital discharge was essentially unchanged. Repeat punch biopsy revealed a neutrophilic panniculitis with widespread foci of fat necrosis and lipomembranous changes. Cultures for bacteria, fungi, and atypical mycobacteria were negative. This patient’s presentation was most consistent with a neutrophilic panniculitis and necrosis secondary to repeated local injections to the right thigh of subcutaneous IFN beta-1a for multiple sclerosis. Panniculitis and necrosis is a rarely reported side effect with interferons, and correlates with dose of IFN. Complications from local injections are predominantly seen in the first month of INF treatment. Prompt involvement of dermatology in a case such as the one presented here is important in order to spare patients weeks of costly hospitalizations and the extended use of intravenous antimicrobial therapy. Commercial support: None identified.
Commercial support: None identified.
P810 P808 Bartholin’s abscess arising in hidradenoma papilliferum: A case report Wonwoo Shon, New York College of Osteopathic Medicine, Old Westbury, NY, United States; David Elkowitz, DO, New York College of Osteopathic Medicine, Old Westbury, NY, United States Hidradenoma papilliferum is a rare, benign, cystic, and papillary tumor. It occurs almost exclusively in the female anogenital areas and is diagnostically challenging to differentiate from other cystic lesions. Bartholin’s abscess is also an anogenital cystic lesion which is often caused by partial or complete ductal obstruction and overlying infection. We report what is to our best knowledge the first case report of Bartholin’s abscess arising in hidradenoma papilliferum. A 43-year-old female presented with painful cystic mass on the left labia majora. Preoperatively, the lesion was diagnosed as Bartholin’s abscess. During the excision and drainage, an additional 2.0 cm 3 0.8 cm 3 0.8 cm tan-brown dermal nodule was identified and submitted for further histologic analysis. Microscopic examination revealed multiple pieces of fibronecrotic tissues associated with a well-circumscribed papillary neoplasm with cystic dilation. The papillary projections and cystic areas were lined by basophilic cuboidal to columnar cells with outer compressed myoepithelial cells. There were foci of active decapitation secretion and apocrine differentiation. The diagnosis of Bartholin’s abscess arising in hidradenoma papilliferum was made. This is the first reported case of Bartholin’s abscess arising in hidradenoma pailliferum. Although significant clinical correlations between these two lesions are not well understood at this point, the intimate association of hidradenoma papilliferum with Bartholin’s abscess suggests partial or complete obstruction of ductal drainage by the tumor mass. We conclude that clinical aspects of hidradenoma papilliferum are confusing especially simultaneous occurrence with other cystic lesions in the vulvar. It is important to keep this tumor in mind as the differential diagnosis and histologic analysis is recommended. Commercial support: None identified.
AB60
J AM ACAD DERMATOL
Cutaneous syringolymphoid hyperplasia in a renal transplant patient Leyre Aguado, MD, Department of Dermatology, Clinica Universitaria de Navarra, Pamplona, Spain; Miren Marquina, MD, Department of Dermatology, Clinica Universitaria de Navarra, Pamplona, Spain; Miguel Angel Idoate, MD, PhD, Department of Pathological Anatomy, Clinica Universitaria de Navarra, Pamplona, Spain; Agustin Espan˜a, MD, PhD, Department of Dermatology, Clinica Universitaria de Navarra, Pamplona, United States Introduction: The eccrine syringolymphoid hyperplasia with alopecia has been described as an atypical form of cutaneous lymphoma of T cells. Some cases have been reported as associated with Sjo ¨ gren syndrome and epidermoid carcinomas. Herein, we report a new case associated with cyclosporine administration in a renal transplant patient. Case report: We present the case of a 49-year-old male who received a renal transplant of a cadaver donor in August 1991 because of a chronic terminal renal insufficiency secondary to chronic pyelonephritis. In the periodic review for cutaneous tumor screening, we observed an alopecic plaque 4 cm in diameter, with erythematous papules centred with corneal plugs, arranged uniformly, on the right knee. The plaque was completely asymptomatic. The biopsy was informed as cutaneous syringolymphoid hyperplasia, with polyclonal, T-cell predominant infiltrate and negative in situ hybridization for EpsteineBarr virus. We substituted cyclosporine (anticalcineurin) with everolimus (m-TOR inhibitor), and the plaque resolved almost spontaneously in 2 months. Discussion: The disappearance of the lesions reaffirms the theory which defends that eccrine syringolymphoid hyperplasia with alopecia would be a reactive inflammatory process other than a real type of cutaneous lymphoma. The polyclonality of the process and the disappearance of the histopathologic lesions after removing the cyclosporine (which is known to induce lymphoproliferative processes) support this theory. Commercial support: None identified.
FEBRUARY 2008
P809
Nevus sebaceus in neonate Loan Towersey, MD, PhD, Instituto de Dermatologia Prof. Rubem David Azulay, Rio de Janeiro, RJ, Brazil; Mayra Carrijo Rochael, MD, PhD, Universidade Federal Fluminense, Niteroi, RJ, Brazil; Mariana Sasse, MD, Instituto de Dermatologia Prof. Rubem David Azulay, Rio de Janeiro, RJ, Brazil; Timothy George Berger, MD, University of California, San Francisco, CA, United States
Interferon beta-1a-associated neutrophilic panniculitis mimicking cellulitis Megan Weber, MD, Mayo Clinic, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States; Karen Warschaw, MD, Mayo Clinic, Scottsdale, AZ, United States; Susan Laman, MD, Mayo Clinic, Scottsdale, AZ, United States
Introduction: Adnexal tumors form a group of neoplasms with a predominant cell lineage of adnexal structure morphologic differentiation: pilosebaceous units and eccrine or apocrine glands. Compound nevus have more than one cell lineage and when all cell lineages are present, it is called organoid nevus. Nevus sebaceus was described by Jadassohn in 1895. This organoid nevus is a complex congenital hamartoma usually located on the scalp and face and presents different chronologic stages (prepubertal, pubertal, and postpubertal). Atypical aspects of nevus sebaceus in a newborn are highlighted. Case report: A 3-day-old boy, born and living in S~ ao Gonc¸alo, RJ, Brazil, was brought for consultation presenting a congenital tumor on the scalp. On the vertex, there was raised plaque formed by the gathering of papillated yellowish millimetric lesions. It measured 3 cm in diameter. A papillation was removed and sent for exam when the boy was 10 days old. Diagnostic hypothesis included nevus sebaceus, cylindroma, and syringocystoadenoma papilliferum. Histopathologic exam disclosed a follicle-sebaceous hamartoma. The tumor was completely removed when the boy was 4 months old and the hypothesis of nevus sebaceus was confirmed on exam. The first biopsy review detected sebaceous cells in a rudimentary infundibulum, a very lesion of nevus sebaceous. Discussion: Most adnexal tumors are benign neoplasms. Associated multiple internal abnormalities include internal neoplasms as seen in MuireTorre syndrome (sebaceous tumor), and Cowden’s disease (trichilemmoma). Adnexal tumors have a complex histogenesis. Nevus sebaceous may develop carcinomas in the late stages and these often show adnexal differentiation. Adnexal neoplasms, benign or malignant, may develop in 20% to 40% of these lesions. Early surgical removal is the therapy of choice. The different stages of nevus sebaceous observed in histopathology correspond to changes observed clinically. In early nevus sebaceous, the flat yellowish plaque shows epidermal hyperplasia, infundibular sebaceous cell lobules, rudimentary hair follicles, the presence of muscles of hair erection, and the absence of appocrine glands in the deep dermis. In neonates, because of the influence of maternal hormones, there may be mature sebaceous glands. Epithelial and connective tissue elements in the organoid nevus are also influenced. This may explain the atypical exophytic papillated tumor seen in this report.
A 58-year-old male with rheumatoid arthritis and multiple sclerosis presented with a 3-day history of progressive redness, swelling, and pain of the right thigh. He was admitted to the hospital for presumed cellulitis. The patient was taking multiple immunomodulating medications, including methotrexate, rituximab, and interferon (IFN) beta-1a. Computed tomography revealed subcutaneous thickening and edema of the thigh consistent with cellulitis. There was no evidence of abscess. The patient was started on broad spectrum intravenous antibacterial and antifungal therapy and his immunomodulating medications were held. After nearly 2 weeks, there was no improvement, and dermatology was consulted. Further history revealed the patient had been on IFN beta-1a for 4 years. He preferentially used his right thigh for injections of IFN, as he had previously required debridement and skin grafting to the left thigh because of a bacterial infection at that site. Physical exam on day 12 of hospitalization revealed a 12-cm erythematous, woody, indurated plaque with no overlying epidermal change. The induration extended beyond the erythema, both medially and laterally. A diagnosis of injection site reaction was favored. Punch biopsies were performed for microscopy and culture. Pathology revealed focal neutrophilic inflammation involving the eccrine glands. Methenamine silver and acid-fast stains were negative. The patient was sent home 2 days later and followed up in dermatology clinic. The physical exam 2 weeks after hospital discharge was essentially unchanged. Repeat punch biopsy revealed a neutrophilic panniculitis with widespread foci of fat necrosis and lipomembranous changes. Cultures for bacteria, fungi, and atypical mycobacteria were negative. This patient’s presentation was most consistent with a neutrophilic panniculitis and necrosis secondary to repeated local injections to the right thigh of subcutaneous IFN beta-1a for multiple sclerosis. Panniculitis and necrosis is a rarely reported side effect with interferons, and correlates with dose of IFN. Complications from local injections are predominantly seen in the first month of INF treatment. Prompt involvement of dermatology in a case such as the one presented here is important in order to spare patients weeks of costly hospitalizations and the extended use of intravenous antimicrobial therapy. Commercial support: None identified.
Commercial support: None identified.
P810 P808 Bartholin’s abscess arising in hidradenoma papilliferum: A case report Wonwoo Shon, New York College of Osteopathic Medicine, Old Westbury, NY, United States; David Elkowitz, DO, New York College of Osteopathic Medicine, Old Westbury, NY, United States Hidradenoma papilliferum is a rare, benign, cystic, and papillary tumor. It occurs almost exclusively in the female anogenital areas and is diagnostically challenging to differentiate from other cystic lesions. Bartholin’s abscess is also an anogenital cystic lesion which is often caused by partial or complete ductal obstruction and overlying infection. We report what is to our best knowledge the first case report of Bartholin’s abscess arising in hidradenoma papilliferum. A 43-year-old female presented with painful cystic mass on the left labia majora. Preoperatively, the lesion was diagnosed as Bartholin’s abscess. During the excision and drainage, an additional 2.0 cm 3 0.8 cm 3 0.8 cm tan-brown dermal nodule was identified and submitted for further histologic analysis. Microscopic examination revealed multiple pieces of fibronecrotic tissues associated with a well-circumscribed papillary neoplasm with cystic dilation. The papillary projections and cystic areas were lined by basophilic cuboidal to columnar cells with outer compressed myoepithelial cells. There were foci of active decapitation secretion and apocrine differentiation. The diagnosis of Bartholin’s abscess arising in hidradenoma papilliferum was made. This is the first reported case of Bartholin’s abscess arising in hidradenoma pailliferum. Although significant clinical correlations between these two lesions are not well understood at this point, the intimate association of hidradenoma papilliferum with Bartholin’s abscess suggests partial or complete obstruction of ductal drainage by the tumor mass. We conclude that clinical aspects of hidradenoma papilliferum are confusing especially simultaneous occurrence with other cystic lesions in the vulvar. It is important to keep this tumor in mind as the differential diagnosis and histologic analysis is recommended. Commercial support: None identified.
AB60
J AM ACAD DERMATOL
Cutaneous syringolymphoid hyperplasia in a renal transplant patient Leyre Aguado, MD, Department of Dermatology, Clinica Universitaria de Navarra, Pamplona, Spain; Miren Marquina, MD, Department of Dermatology, Clinica Universitaria de Navarra, Pamplona, Spain; Miguel Angel Idoate, MD, PhD, Department of Pathological Anatomy, Clinica Universitaria de Navarra, Pamplona, Spain; Agustin Espan˜a, MD, PhD, Department of Dermatology, Clinica Universitaria de Navarra, Pamplona, United States Introduction: The eccrine syringolymphoid hyperplasia with alopecia has been described as an atypical form of cutaneous lymphoma of T cells. Some cases have been reported as associated with Sjo ¨ gren syndrome and epidermoid carcinomas. Herein, we report a new case associated with cyclosporine administration in a renal transplant patient. Case report: We present the case of a 49-year-old male who received a renal transplant of a cadaver donor in August 1991 because of a chronic terminal renal insufficiency secondary to chronic pyelonephritis. In the periodic review for cutaneous tumor screening, we observed an alopecic plaque 4 cm in diameter, with erythematous papules centred with corneal plugs, arranged uniformly, on the right knee. The plaque was completely asymptomatic. The biopsy was informed as cutaneous syringolymphoid hyperplasia, with polyclonal, T-cell predominant infiltrate and negative in situ hybridization for EpsteineBarr virus. We substituted cyclosporine (anticalcineurin) with everolimus (m-TOR inhibitor), and the plaque resolved almost spontaneously in 2 months. Discussion: The disappearance of the lesions reaffirms the theory which defends that eccrine syringolymphoid hyperplasia with alopecia would be a reactive inflammatory process other than a real type of cutaneous lymphoma. The polyclonality of the process and the disappearance of the histopathologic lesions after removing the cyclosporine (which is known to induce lymphoproliferative processes) support this theory. Commercial support: None identified.
FEBRUARY 2008