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Methotrexate treatment for primary cutaneous CD30+ lymphoproliferative disorders: A long-term follow-up
5822
5156
Metabolic syndrome, cardiovascular risk and carotid atherosclerosis in children and adolescents with psoriasis Andrea Tovar Garza, Instituto Dermatol ogico de Jalisco ‘‘Dr Jose Barba Rubio’’ Psoriasis is an inflammatory skin condition that affects 1-3% of the world’s population. The association between psoriasis and cardiovascular risk in children and adolescents is still unclear. A case-control study was conducted with children and adolescents, 2-18 years old, with mild to moderate-severe psoriasis with clinical and biopsy proven diagnosis, and a healthy control group comprising children, matched according to age, gender and body mass index (BMI). Metabolic syndrome (MS) was defined by ATPIII modified criteria, cardiovascular risk was measured by high-sensitivity C reactive protein (hs-CRP) and atherosclerosis was determined by carotid intima-media thickness (IMT). Twenty-six patients were included (77% mild psoriasis). Nine (35%) with psoriasis had MS vs 8 (31%) of the control group (P ¼.75, x 2 test). There was no significant difference in HOMA-IR (P ¼.23, x2 test). Moderate cardiovascular risk (hs-CRP 1-3mg/L) was present in 12 (46%), however, no statistical significant difference was observed (P ¼ .16, x2 test). Moderate-high cardiovascular risk was present in 6 (100%) of moderate-severe psoriasis and 13 (65%) with mild psoriasis (P ¼ .04, x 2 test). Twenty-five (95%) with psoriasis had carotid atherosclerosis. Bilateral carotid atherosclerosis was present in 6 (100%) of moderate-severe psoriasis and 19 (95%) with mild psoriasis (P ¼ .0003, x2 test). There was no difference between groups with regard to MS prevalence. However, 95% of psoriasis patients had carotid atherosclerosis and 100% had cardiovascular risk, directly related to the severity of the psoriasis. Our lack of statistical significance difference might be due to the fact that the subjects were matched by BMI and most patients had mild psoriasis.
Metastatic pleomorphic dermal sarcoma: Case report of an uncommon skin cancer Georgios Kravvas, University College London Hospitals; David Veitch, University College London Hospitals; Conal Perrett, University College London Hospitals Case history: A 69-year-old, white man was reviewed with a 3-month history of an enlarging, asymptomatic forehead nodule. Examination revealed a 15-mm, shiny, erythematous nodule with no associated lymphadenopathy. Further examination was unremarkable. Excision biopsy with 3mm clinical margins revealed a CD10positive, dermal-based tumor extending from the dermoepidermal junction to the subcutaneous tissue with no lymphovascular invasion. Histologic margins were clear by 2 mm peripherally and 1.2 mm deep. Findings were consistent with pleomorphic dermal sarcoma. On his 6-month follow-up a new 5-mm papule was noted on the surgical scar. Further excision revealed recurrent pleomorphic dermal sarcoma with clear histologic margins, which was treated further by local adjuvant radiotherapy. 1-month later, surveillance CT chest noted an isolated left lower-lobe nodule, the biopsy of which confirmed metastatic pleomorphic dermal sarcoma. Shortly afterwards the patient developed pneumonia and despite treatment deteriorated rapidly and passed away a few days later.
Commercial support: None identified.
Discussion: Pleomorphic dermal sarcoma (PDS) is an aggressive variant of atypical fibroxanthoma (AFX) that classically arises in sun-damaged areas of skin in the elderly. It is a dermal-based tumor that behaves mainly in a benign fashion but may also lead to local recurrences and distant metastases. PDS has similar histopathological features to AFX supporting the theory that they are part of the same disease spectrum. Some authors however support the view that the two are synonymous. A retrospective study of 18 patients with PDS reported tumor predilection on the scalp and face in patients with a median age of 81 years. Consistent histopathological features included spindle cells in a fascicular pattern, epithelioid and multinucleate giant cells with pleomorphic vesicular nuclei, and tumor invasion to the subcutis. 20% of patients developed local recurrences, and 20% distant metastases to the skin, lymph nodes and/or lungs. Another study reported tumor necrosis in 53%, lymphovascular invasion in 26%, and perineural infiltration in 29%. In certain cases, where adequate histologic margin clearance is unachievable or in the event of recurrence, treatment with adjuvant radiotherapy may also be considered. In large defects, the initiation of adjuvant radiotherapy tends to be delayed whilst waiting for wound healing. M€ uller et al reported successful graft healing without complications by treating a patient with adjuvant radiotherapy immediately following excision, performing reconstruction at a later stage. In conclusion, we report a rare tumor with local recurrence and metastases despite repeat excisions and adjuvant radiotherapy. Commercial support: None identified.
4344 Metastatic Croh ns disease: Is it easy to do an appropriate therapy approach? Ricardo Ruiz-Villaverde, PhD, Dermatolgy, Complejo Hospitalario de Granada; Daniel Sanchez-Cano, PhD, Internal Medicine, Complejo Hospitalario de Granada; Israel Perez-Lopez, PhD, Dermatology, Complejo Hospitalario de Granada; Antonio Lopez-Martinez, PhD, Dermatology, Complejo Hospitalario de Granada; Jose Aneiros-Fernandez, PhD, Pathology, Complejo Hospitalario de Granada Introduction: Skin involvement in Crohn’s disease (CD) occurs according to the series published in the literature up to 44% of patients with this entity. From the clinical point of view mucocutaneous lesions in CD may be categorized as: a) CDspecific lesions (involving the skin by the same mechanism occurring in the GI tract), reactive (with a different mechanism from the GI tract) or associated (with an unknown or different well-defined mechanism). Metastatic EC is included in the first group. It was first described in 1965 been the less frequent specific CD cutaneous lesion. Case presentation: A 35-year-old male with personal history of Crohn’s disease (CD) for 9 years, controlled with infliximab 5 mg/kg every 8 weeks, was admitted to out unit complaining eczemas on the front surface of both thighs, refractory to topical treatment with clobetasol cream for 5 weeks. Histologic examination revealed the presence of chronic granulomatous inflammatory infiltrate in the dermis with intense epithelial hyperplasia with mild local ulceration. At higher magnification non-caseating granulomas with multinucleated giant cells with perivascular distribution could be identified. Complementary tests showed results within normal levels (blood count, general biochemistry, erythrocyte sedimentation rate (ERS), Creactive protein-CRP-, autoantibodies, circulating immune complexes, angiotensinconverting enzyme and calcium in urine analysis. Chest x-ray performed showed no abnormalities. Levels of infliximab were normal with high levels of anti-drug antibodies (ADA). Prednisone at a dose of 0.5 mg/kg/ day was initiated with moderate control, so it was suggested to change the treatment of the underlying pathology to therapeutic doses of adalimumab in EC, which allowed definitively resolve the metastatic Crohn’s disease. Discussion: There are no well-established guidelines regarding metastatic Crohn’s disease, so we should prescribe our treatment according to patient preferences, adverse effects or the needing to be monitored. First-line use to be topical therapy (corticosteroids or calcineurin inhibitors). If there is no response, systemic corticosteroids (prednisone 0.5 mg / kg) may be an interesting option. Oral metronidazole (800-1500 mg / day), conventional immunosuppressants (methotrexate, azathioprine, cyclosporine) or biological therapy (infliximab, adalimumab or certolizumab) have been successfully used in individual cases and case series. Commercial support: None identified.
JUNE 2017
4660 Methotrexate treatment for primary cutaneous CD30+ lymphoproliferative disorders: A long-term follow-up Min Soo Jang, MD, Department of Dermatology, Kosin University College of Medicine; Ji Yun Jang, MD, Department of Dermatology, Kosin University College of Medicine; Myeong Hyeon Yang, MD, Department of Dermatology, Kosin University College of Medicine; Joon Hee Kim, MD, Department of Dermatology, Kosin University College of Medicine; Kang Hoon Lee, MD, Department of Dermatology, Kosin University College of Medicine; Hyun HwangBo, MD, Department of Dermatology, Kosin University College of Medicine Background: Primary cutaneous CD30+ lymphoproliferative disorders (CD30+ LPDs) include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (PCALCL). A broad variety of therapeutic strategies for these diseases have been reported and range from simple observation to aggressive systemic chemotherapy. Objective: To investigate the effectiveness and outcome of methotrexate (MTX) treatment in primary cutaneous CD30+ LPDs. Methods: This retrospective study reviewed the response to MTX and long-term follow-up data in 25 patients of LyP and 6 patients of PCALCL, who were histologically diagnosed, at one institution. Results: The patients’ ages ranged from 5 to 65 years. Mean maximum dose of MTX was 15.2 mg/week. Mean duration of MTX therapy per each cycle was 8.3 weeks in LyP patients and 9.8 weeks in PCALCL patients. Among 25 patients of LyP, 19 (76%) showed complete remission (CR) and 6 (24%) showed partial remission (PR). Among 6 patients of PCALCL, 5 (83.3%) showed CR and 1 (16.7%) showed PR. During mean follow-up of 83 months, 8 patients (32%) of LyP and 5 patients (83.3%) of PCALCL showed relapse. PCALCL developed in 1 patient of LyP. Mycosis fungoides developed in 2 patients of PCALCL and LyP developed in 3 patients of PCALCL. At the final follow up, 2 patients of PCALCL survived more than 10 years and none of PCALCL patients died. Conclusion: Low-dose MTX is an effective and safe treatment for primary cutaneous CD30+ LPDs. Commercial support: None identified.
J AM ACAD DERMATOL
AB169
5156
Metabolic syndrome, cardiovascular risk and carotid atherosclerosis in children and adolescents with psoriasis Andrea Tovar Garza, Instituto Dermatol ogico de Jalisco ‘‘Dr Jose Barba Rubio’’ Psoriasis is an inflammatory skin condition that affects 1-3% of the world’s population. The association between psoriasis and cardiovascular risk in children and adolescents is still unclear. A case-control study was conducted with children and adolescents, 2-18 years old, with mild to moderate-severe psoriasis with clinical and biopsy proven diagnosis, and a healthy control group comprising children, matched according to age, gender and body mass index (BMI). Metabolic syndrome (MS) was defined by ATPIII modified criteria, cardiovascular risk was measured by high-sensitivity C reactive protein (hs-CRP) and atherosclerosis was determined by carotid intima-media thickness (IMT). Twenty-six patients were included (77% mild psoriasis). Nine (35%) with psoriasis had MS vs 8 (31%) of the control group (P ¼.75, x 2 test). There was no significant difference in HOMA-IR (P ¼.23, x2 test). Moderate cardiovascular risk (hs-CRP 1-3mg/L) was present in 12 (46%), however, no statistical significant difference was observed (P ¼ .16, x2 test). Moderate-high cardiovascular risk was present in 6 (100%) of moderate-severe psoriasis and 13 (65%) with mild psoriasis (P ¼ .04, x 2 test). Twenty-five (95%) with psoriasis had carotid atherosclerosis. Bilateral carotid atherosclerosis was present in 6 (100%) of moderate-severe psoriasis and 19 (95%) with mild psoriasis (P ¼ .0003, x2 test). There was no difference between groups with regard to MS prevalence. However, 95% of psoriasis patients had carotid atherosclerosis and 100% had cardiovascular risk, directly related to the severity of the psoriasis. Our lack of statistical significance difference might be due to the fact that the subjects were matched by BMI and most patients had mild psoriasis.
Metastatic pleomorphic dermal sarcoma: Case report of an uncommon skin cancer Georgios Kravvas, University College London Hospitals; David Veitch, University College London Hospitals; Conal Perrett, University College London Hospitals Case history: A 69-year-old, white man was reviewed with a 3-month history of an enlarging, asymptomatic forehead nodule. Examination revealed a 15-mm, shiny, erythematous nodule with no associated lymphadenopathy. Further examination was unremarkable. Excision biopsy with 3mm clinical margins revealed a CD10positive, dermal-based tumor extending from the dermoepidermal junction to the subcutaneous tissue with no lymphovascular invasion. Histologic margins were clear by 2 mm peripherally and 1.2 mm deep. Findings were consistent with pleomorphic dermal sarcoma. On his 6-month follow-up a new 5-mm papule was noted on the surgical scar. Further excision revealed recurrent pleomorphic dermal sarcoma with clear histologic margins, which was treated further by local adjuvant radiotherapy. 1-month later, surveillance CT chest noted an isolated left lower-lobe nodule, the biopsy of which confirmed metastatic pleomorphic dermal sarcoma. Shortly afterwards the patient developed pneumonia and despite treatment deteriorated rapidly and passed away a few days later.
Commercial support: None identified.
Discussion: Pleomorphic dermal sarcoma (PDS) is an aggressive variant of atypical fibroxanthoma (AFX) that classically arises in sun-damaged areas of skin in the elderly. It is a dermal-based tumor that behaves mainly in a benign fashion but may also lead to local recurrences and distant metastases. PDS has similar histopathological features to AFX supporting the theory that they are part of the same disease spectrum. Some authors however support the view that the two are synonymous. A retrospective study of 18 patients with PDS reported tumor predilection on the scalp and face in patients with a median age of 81 years. Consistent histopathological features included spindle cells in a fascicular pattern, epithelioid and multinucleate giant cells with pleomorphic vesicular nuclei, and tumor invasion to the subcutis. 20% of patients developed local recurrences, and 20% distant metastases to the skin, lymph nodes and/or lungs. Another study reported tumor necrosis in 53%, lymphovascular invasion in 26%, and perineural infiltration in 29%. In certain cases, where adequate histologic margin clearance is unachievable or in the event of recurrence, treatment with adjuvant radiotherapy may also be considered. In large defects, the initiation of adjuvant radiotherapy tends to be delayed whilst waiting for wound healing. M€ uller et al reported successful graft healing without complications by treating a patient with adjuvant radiotherapy immediately following excision, performing reconstruction at a later stage. In conclusion, we report a rare tumor with local recurrence and metastases despite repeat excisions and adjuvant radiotherapy. Commercial support: None identified.
4344 Metastatic Croh ns disease: Is it easy to do an appropriate therapy approach? Ricardo Ruiz-Villaverde, PhD, Dermatolgy, Complejo Hospitalario de Granada; Daniel Sanchez-Cano, PhD, Internal Medicine, Complejo Hospitalario de Granada; Israel Perez-Lopez, PhD, Dermatology, Complejo Hospitalario de Granada; Antonio Lopez-Martinez, PhD, Dermatology, Complejo Hospitalario de Granada; Jose Aneiros-Fernandez, PhD, Pathology, Complejo Hospitalario de Granada Introduction: Skin involvement in Crohn’s disease (CD) occurs according to the series published in the literature up to 44% of patients with this entity. From the clinical point of view mucocutaneous lesions in CD may be categorized as: a) CDspecific lesions (involving the skin by the same mechanism occurring in the GI tract), reactive (with a different mechanism from the GI tract) or associated (with an unknown or different well-defined mechanism). Metastatic EC is included in the first group. It was first described in 1965 been the less frequent specific CD cutaneous lesion. Case presentation: A 35-year-old male with personal history of Crohn’s disease (CD) for 9 years, controlled with infliximab 5 mg/kg every 8 weeks, was admitted to out unit complaining eczemas on the front surface of both thighs, refractory to topical treatment with clobetasol cream for 5 weeks. Histologic examination revealed the presence of chronic granulomatous inflammatory infiltrate in the dermis with intense epithelial hyperplasia with mild local ulceration. At higher magnification non-caseating granulomas with multinucleated giant cells with perivascular distribution could be identified. Complementary tests showed results within normal levels (blood count, general biochemistry, erythrocyte sedimentation rate (ERS), Creactive protein-CRP-, autoantibodies, circulating immune complexes, angiotensinconverting enzyme and calcium in urine analysis. Chest x-ray performed showed no abnormalities. Levels of infliximab were normal with high levels of anti-drug antibodies (ADA). Prednisone at a dose of 0.5 mg/kg/ day was initiated with moderate control, so it was suggested to change the treatment of the underlying pathology to therapeutic doses of adalimumab in EC, which allowed definitively resolve the metastatic Crohn’s disease. Discussion: There are no well-established guidelines regarding metastatic Crohn’s disease, so we should prescribe our treatment according to patient preferences, adverse effects or the needing to be monitored. First-line use to be topical therapy (corticosteroids or calcineurin inhibitors). If there is no response, systemic corticosteroids (prednisone 0.5 mg / kg) may be an interesting option. Oral metronidazole (800-1500 mg / day), conventional immunosuppressants (methotrexate, azathioprine, cyclosporine) or biological therapy (infliximab, adalimumab or certolizumab) have been successfully used in individual cases and case series. Commercial support: None identified.
JUNE 2017
4660 Methotrexate treatment for primary cutaneous CD30+ lymphoproliferative disorders: A long-term follow-up Min Soo Jang, MD, Department of Dermatology, Kosin University College of Medicine; Ji Yun Jang, MD, Department of Dermatology, Kosin University College of Medicine; Myeong Hyeon Yang, MD, Department of Dermatology, Kosin University College of Medicine; Joon Hee Kim, MD, Department of Dermatology, Kosin University College of Medicine; Kang Hoon Lee, MD, Department of Dermatology, Kosin University College of Medicine; Hyun HwangBo, MD, Department of Dermatology, Kosin University College of Medicine Background: Primary cutaneous CD30+ lymphoproliferative disorders (CD30+ LPDs) include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (PCALCL). A broad variety of therapeutic strategies for these diseases have been reported and range from simple observation to aggressive systemic chemotherapy. Objective: To investigate the effectiveness and outcome of methotrexate (MTX) treatment in primary cutaneous CD30+ LPDs. Methods: This retrospective study reviewed the response to MTX and long-term follow-up data in 25 patients of LyP and 6 patients of PCALCL, who were histologically diagnosed, at one institution. Results: The patients’ ages ranged from 5 to 65 years. Mean maximum dose of MTX was 15.2 mg/week. Mean duration of MTX therapy per each cycle was 8.3 weeks in LyP patients and 9.8 weeks in PCALCL patients. Among 25 patients of LyP, 19 (76%) showed complete remission (CR) and 6 (24%) showed partial remission (PR). Among 6 patients of PCALCL, 5 (83.3%) showed CR and 1 (16.7%) showed PR. During mean follow-up of 83 months, 8 patients (32%) of LyP and 5 patients (83.3%) of PCALCL showed relapse. PCALCL developed in 1 patient of LyP. Mycosis fungoides developed in 2 patients of PCALCL and LyP developed in 3 patients of PCALCL. At the final follow up, 2 patients of PCALCL survived more than 10 years and none of PCALCL patients died. Conclusion: Low-dose MTX is an effective and safe treatment for primary cutaneous CD30+ LPDs. Commercial support: None identified.
J AM ACAD DERMATOL
AB169