The Diagnosis: PELVIS Syndrome

Infantile hemangiomas (IHs) are present in up to 10% of infants by 1 year of age and are most commonly located on the face and upper extremities. Less than 10% of IHs develop in the perineum.¹ Perineal IHs are benign tumors of the vascular endothelium that present as plaques and commonly are accompanied by painful ulcerations. Ulceration is more common in the diaper area secondary to irritation from urine, stool, and friction.² Although most IHs are benign isolated findings, facial IHs have been associated with several syndromes including Sturge-Weber and PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, cardiac anomalies and coarctation of the aorta, and eye and endocrine abnormalities) syndromes.³ Researchers also have identified an association between lumbosacral IHs and spinal dysraphism (tethered spinal cord).⁴

A smaller number of studies have investigated congenital anomalies related to perineal IH,^1,5 specifically PELVIS syndrome. The acronym PELVIS has been used to describe a syndrome of congenital malformations including perineal hemangioma, external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag.¹ An alternative description of similar findings is LUMBAR (lower body hemangioma and other cutaneous defects; urogenital anomalies, ulceration; myelopathy; bony deformities; anorectal malformations, arterial anomalies; and renal anomalies).⁵ Researchers have suggested that both of these acronyms describe the same syndrome, and it is common for the syndrome to be incomplete.⁶ One study (N=11) found that perineal hemangiomas are most commonly associated with anal malformations (8 patients), followed by urinary tract abnormalities (7 patients) and malformation of the external genitalia (7 patients). A skin tag was present in 5 patients.¹ The pathogenesis of PELVIS syndrome is unknown.

When an infant presents with a perineal hemangioma and physical examination suggests PELVIS syndrome, imaging should be performed to evaluate for other anomalies. Before 4 months of age, ultrasound should be utilized to investigate the presence of reno-genitourinary or spinal malformations. Magnetic resonance imaging is the preferred imaging modality in children older than 4 months.⁷ Management of PELVIS syndrome requires a multidisciplinary approach and early recognition of the full extent of congenital malformations. Pediatric dermatologists, urologists, endocrinologists, and neonatologists have a role in its diagnosis and treatment.

The Diagnosis: PELVIS Syndrome

Infantile hemangiomas (IHs) are present in up to 10% of infants by 1 year of age and are most commonly located on the face and upper extremities. Less than 10% of IHs develop in the perineum.¹ Perineal IHs are benign tumors of the vascular endothelium that present as plaques and commonly are accompanied by painful ulcerations. Ulceration is more common in the diaper area secondary to irritation from urine, stool, and friction.² Although most IHs are benign isolated findings, facial IHs have been associated with several syndromes including Sturge-Weber and PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, cardiac anomalies and coarctation of the aorta, and eye and endocrine abnormalities) syndromes.³ Researchers also have identified an association between lumbosacral IHs and spinal dysraphism (tethered spinal cord).⁴

A smaller number of studies have investigated congenital anomalies related to perineal IH,^1,5 specifically PELVIS syndrome. The acronym PELVIS has been used to describe a syndrome of congenital malformations including perineal hemangioma, external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag.¹ An alternative description of similar findings is LUMBAR (lower body hemangioma and other cutaneous defects; urogenital anomalies, ulceration; myelopathy; bony deformities; anorectal malformations, arterial anomalies; and renal anomalies).⁵ Researchers have suggested that both of these acronyms describe the same syndrome, and it is common for the syndrome to be incomplete.⁶ One study (N=11) found that perineal hemangiomas are most commonly associated with anal malformations (8 patients), followed by urinary tract abnormalities (7 patients) and malformation of the external genitalia (7 patients). A skin tag was present in 5 patients.¹ The pathogenesis of PELVIS syndrome is unknown.

When an infant presents with a perineal hemangioma and physical examination suggests PELVIS syndrome, imaging should be performed to evaluate for other anomalies. Before 4 months of age, ultrasound should be utilized to investigate the presence of reno-genitourinary or spinal malformations. Magnetic resonance imaging is the preferred imaging modality in children older than 4 months.⁷ Management of PELVIS syndrome requires a multidisciplinary approach and early recognition of the full extent of congenital malformations. Pediatric dermatologists, urologists, endocrinologists, and neonatologists have a role in its diagnosis and treatment.

The Diagnosis: PELVIS Syndrome

Infantile hemangiomas (IHs) are present in up to 10% of infants by 1 year of age and are most commonly located on the face and upper extremities. Less than 10% of IHs develop in the perineum.¹ Perineal IHs are benign tumors of the vascular endothelium that present as plaques and commonly are accompanied by painful ulcerations. Ulceration is more common in the diaper area secondary to irritation from urine, stool, and friction.² Although most IHs are benign isolated findings, facial IHs have been associated with several syndromes including Sturge-Weber and PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, cardiac anomalies and coarctation of the aorta, and eye and endocrine abnormalities) syndromes.³ Researchers also have identified an association between lumbosacral IHs and spinal dysraphism (tethered spinal cord).⁴

A smaller number of studies have investigated congenital anomalies related to perineal IH,^1,5 specifically PELVIS syndrome. The acronym PELVIS has been used to describe a syndrome of congenital malformations including perineal hemangioma, external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag.¹ An alternative description of similar findings is LUMBAR (lower body hemangioma and other cutaneous defects; urogenital anomalies, ulceration; myelopathy; bony deformities; anorectal malformations, arterial anomalies; and renal anomalies).⁵ Researchers have suggested that both of these acronyms describe the same syndrome, and it is common for the syndrome to be incomplete.⁶ One study (N=11) found that perineal hemangiomas are most commonly associated with anal malformations (8 patients), followed by urinary tract abnormalities (7 patients) and malformation of the external genitalia (7 patients). A skin tag was present in 5 patients.¹ The pathogenesis of PELVIS syndrome is unknown.

When an infant presents with a perineal hemangioma and physical examination suggests PELVIS syndrome, imaging should be performed to evaluate for other anomalies. Before 4 months of age, ultrasound should be utilized to investigate the presence of reno-genitourinary or spinal malformations. Magnetic resonance imaging is the preferred imaging modality in children older than 4 months.⁷ Management of PELVIS syndrome requires a multidisciplinary approach and early recognition of the full extent of congenital malformations. Pediatric dermatologists, urologists, endocrinologists, and neonatologists have a role in its diagnosis and treatment.

Disseminated superficial actinic porokeratosis (DSAP) is a chronic condition characterized by numerous atrophic papules and patches with a distinctive peripheral keratotic ridge, typically found on sun-exposed areas.^1,2 Treatment of DSAP is warranted not only for cosmetic and symptomatic benefits but also to prevent malignant transformation.^3,4 Successful treatment of DSAP often is difficult and frequently requires the use of multiple modalities. Ingenol mebutate gel 0.05% is a topical medication primarily used for the treatment of actinic keratosis (AK) by inducing cell death.⁵ We report a case of DSAP treated effectively with ingenol mebutate gel 0.05%.

Case Report

A 37-year-old woman was referred to the dermatology department for counseling for pseudoxanthoma elasticum (PXE), which had been proven on biopsy by an outside dermatologist 2 years prior. Physical examination revealed yellow papules on the neck that were characteristic of PXE, but no lesions were noted on the arms or legs. The only other cutaneous finding was a soft nodule on the right hip consistent with a lipoma. The patient returned to our institution 6 years later with lesions on both lower legs. She reported that these lesions had been present for 3 years and were exacerbated by sun exposure. On physical examination, multiple scattered, erythematous, annular, scaling papules and plaques were noted on the bilateral legs. A biopsy showed the histopathologic findings of DSAP (Figure 1). The patient had no family history of DSAP or PXE.

To determine the best treatment modality, we treated 4 test areas on both upper and lower legs: one with trichloroacetic acid (TCA), one with cryotherapy, one with imiquimod cream 5%, and one with tretinoin cream 0.1%. The patient returned 4 weeks later and showed modest response to TCA, cryotherapy, and tretinoin cream. Because cryotherapy was determined to be most effective, 20 more lesions were frozen at that visit. Over the next 2 years, the patient was treated with TCA, imiquimod cream 5%, and tretinoin cream 0.1%, but all ultimately proved ineffective for DSAP.

The patient returned 2 years after treatment failure (age 47 years) and was prescribed ingenol mebutate gel 0.05% for 2 days over an area of 25 cm² on the right lower leg (Figure 2A). She returned for follow-up at days 3, 15, 30, and 60. At day 3, the patient developed an inflammatory response to the medication with moderate erythema and scaling of individual lesions. No vesiculation, pustulation, edema, or ulceration was exhibited (Figure 2B). At day 30, there was a marked reduction in scaling with some postinflammatory erythema (Figure 2C). At day 60, much of the erythema had faded and the scale remained notably reduced (Figure 2D).

Comment

Disseminated superficial actinic porokeratosis is the most common subtype of porokeratosis, a keratinization disorder. There are 6 subtypes of porokeratosis identified in the literature: DSAP, disseminated superficial porokeratosis, classic porokeratosis of Mibelli, porokeratosis plantaris palmaris et disseminata, linear porokeratosis, and punctate porokeratosis.⁶ Disseminated superficial actinic porokeratosis has a female predominance (1.8:1 ratio)⁷ and generally appears in the third or fourth decades of life. Clonal proliferations of atypical keratinocytes have been implicated in the etiology of DSAP; however, the exact pathogenesis is unclear. Risk factors for DSAP include genetic susceptibility (eg, autosomal-dominant inheritance pattern), exposure to UV radiation, and drug-related immunosuppression or immunodeficiency.⁷ Other proposed etiologic risk factors include trauma and infection.⁸ Clinical diagnosis of DSAP is confirmed by the histological presence of a cornoid lamella (a thin column ofparakeratotic cells), a thinning epidermis, an absent or thinned granular cell layer, and a prominent dermal lymphocytic infiltrate.^9,10

Disseminated superficial actinic porokeratosis clinically presents as small atrophic scaly papules and/or patches with raised peripheral ridges symmetrically dispersed on sun-exposed areas of the arms, legs, back, and shoulders. Although these lesions are extensive, they typically spare the mucous membranes, palms, and soles¹¹; only a small percentage of cases report facial lesions,¹² which often are asymptomatic but cosmetically bothersome. Additionally, approximately half of patients report symptoms of pruritus and/or stinging,¹³ thus treatment of DSAP is mainly indicated for symptomatic relief and cosmetic purposes. Malignant degeneration^14,15 occurs in approximately 7.5% to 11% of porokeratosis cases,^10,16 warranting treatment for preventative measures.

Management of DSAP is dependent on the extent of the disease and the level of concern for malignant transformation. Localized disease can be treated with cryotherapy, CO₂ laser, and/or ablative techniques (eg, excision, curettage, dermabrasion) with variable degrees of success but high risk for scarring.¹ More extensive disease requires treatment with topical retinoids, topical 5-fluorouracil, imiquimod cream 5%, diclofenac gel 3%, topical vitamin D₃ analogues, and photodynamic therapy.¹ Several other therapies have been reported in the literature with partial and/or complete success, including systemic retinoids (eg, acitretin), Q-switched ruby laser, Nd:YAG laser, fractional photothermolysis, Grenz rays, pulsed dye laser, fractional photothermolysis, topical corticosteroids, and fluor-hydroxy pulse peel.⁶ Although there is an extensive array of therapies for DSAP, treatment results are variable with mostly limited success. Successful treatment of DSAP is difficult and often requires the use of multiple modalities.

Ingenol mebutate is the active compound found in the sap of Euphorbia peplus used for the topical treatment of various skin conditions, including AKs.¹⁷ Ingenol mebutate gel 0.05% once daily for 2 days has been approved by the US Food and Drug Administration for the topical treatment of AKs. The mechanism of action of ingenol mebutate in AK therapy is not yet fully understood. In vivo and in vitro models have demonstrated both an induction of local lesion cell death and promotion of lesion-specific inflammatory response.¹⁸ When used in the treatment of AKs, ingenol mebutate gel 0.05% may cause a mild to moderate localized inflammatory response (eg, erythema, flaking/scaling, crusting, vesiculation/pustulation, erosion/ulceration, edema).

Our case is a rare report of successful treatment of DSAP with ingenol mebutate gel 0.05%. We found that treatment with ingenol mebutate gel 0.05% resulted in clinical improvement of DSAP lesions with minimal discomfort and good cosmetic response. This 2-day regimen is easy to use and patient friendly, improving medication compliance in such a cumbersome disease. We hope this case suggests that ingenol mebutate gel 0.05% could be a useful treatment alternative for DSAP, but future clinical studies should be conducted.

Disseminated superficial actinic porokeratosis (DSAP) is a chronic condition characterized by numerous atrophic papules and patches with a distinctive peripheral keratotic ridge, typically found on sun-exposed areas.^1,2 Treatment of DSAP is warranted not only for cosmetic and symptomatic benefits but also to prevent malignant transformation.^3,4 Successful treatment of DSAP often is difficult and frequently requires the use of multiple modalities. Ingenol mebutate gel 0.05% is a topical medication primarily used for the treatment of actinic keratosis (AK) by inducing cell death.⁵ We report a case of DSAP treated effectively with ingenol mebutate gel 0.05%.

Case Report

A 37-year-old woman was referred to the dermatology department for counseling for pseudoxanthoma elasticum (PXE), which had been proven on biopsy by an outside dermatologist 2 years prior. Physical examination revealed yellow papules on the neck that were characteristic of PXE, but no lesions were noted on the arms or legs. The only other cutaneous finding was a soft nodule on the right hip consistent with a lipoma. The patient returned to our institution 6 years later with lesions on both lower legs. She reported that these lesions had been present for 3 years and were exacerbated by sun exposure. On physical examination, multiple scattered, erythematous, annular, scaling papules and plaques were noted on the bilateral legs. A biopsy showed the histopathologic findings of DSAP (Figure 1). The patient had no family history of DSAP or PXE.

To determine the best treatment modality, we treated 4 test areas on both upper and lower legs: one with trichloroacetic acid (TCA), one with cryotherapy, one with imiquimod cream 5%, and one with tretinoin cream 0.1%. The patient returned 4 weeks later and showed modest response to TCA, cryotherapy, and tretinoin cream. Because cryotherapy was determined to be most effective, 20 more lesions were frozen at that visit. Over the next 2 years, the patient was treated with TCA, imiquimod cream 5%, and tretinoin cream 0.1%, but all ultimately proved ineffective for DSAP.

The patient returned 2 years after treatment failure (age 47 years) and was prescribed ingenol mebutate gel 0.05% for 2 days over an area of 25 cm² on the right lower leg (Figure 2A). She returned for follow-up at days 3, 15, 30, and 60. At day 3, the patient developed an inflammatory response to the medication with moderate erythema and scaling of individual lesions. No vesiculation, pustulation, edema, or ulceration was exhibited (Figure 2B). At day 30, there was a marked reduction in scaling with some postinflammatory erythema (Figure 2C). At day 60, much of the erythema had faded and the scale remained notably reduced (Figure 2D).

Comment

Disseminated superficial actinic porokeratosis is the most common subtype of porokeratosis, a keratinization disorder. There are 6 subtypes of porokeratosis identified in the literature: DSAP, disseminated superficial porokeratosis, classic porokeratosis of Mibelli, porokeratosis plantaris palmaris et disseminata, linear porokeratosis, and punctate porokeratosis.⁶ Disseminated superficial actinic porokeratosis has a female predominance (1.8:1 ratio)⁷ and generally appears in the third or fourth decades of life. Clonal proliferations of atypical keratinocytes have been implicated in the etiology of DSAP; however, the exact pathogenesis is unclear. Risk factors for DSAP include genetic susceptibility (eg, autosomal-dominant inheritance pattern), exposure to UV radiation, and drug-related immunosuppression or immunodeficiency.⁷ Other proposed etiologic risk factors include trauma and infection.⁸ Clinical diagnosis of DSAP is confirmed by the histological presence of a cornoid lamella (a thin column ofparakeratotic cells), a thinning epidermis, an absent or thinned granular cell layer, and a prominent dermal lymphocytic infiltrate.^9,10

Disseminated superficial actinic porokeratosis clinically presents as small atrophic scaly papules and/or patches with raised peripheral ridges symmetrically dispersed on sun-exposed areas of the arms, legs, back, and shoulders. Although these lesions are extensive, they typically spare the mucous membranes, palms, and soles¹¹; only a small percentage of cases report facial lesions,¹² which often are asymptomatic but cosmetically bothersome. Additionally, approximately half of patients report symptoms of pruritus and/or stinging,¹³ thus treatment of DSAP is mainly indicated for symptomatic relief and cosmetic purposes. Malignant degeneration^14,15 occurs in approximately 7.5% to 11% of porokeratosis cases,^10,16 warranting treatment for preventative measures.

Management of DSAP is dependent on the extent of the disease and the level of concern for malignant transformation. Localized disease can be treated with cryotherapy, CO₂ laser, and/or ablative techniques (eg, excision, curettage, dermabrasion) with variable degrees of success but high risk for scarring.¹ More extensive disease requires treatment with topical retinoids, topical 5-fluorouracil, imiquimod cream 5%, diclofenac gel 3%, topical vitamin D₃ analogues, and photodynamic therapy.¹ Several other therapies have been reported in the literature with partial and/or complete success, including systemic retinoids (eg, acitretin), Q-switched ruby laser, Nd:YAG laser, fractional photothermolysis, Grenz rays, pulsed dye laser, fractional photothermolysis, topical corticosteroids, and fluor-hydroxy pulse peel.⁶ Although there is an extensive array of therapies for DSAP, treatment results are variable with mostly limited success. Successful treatment of DSAP is difficult and often requires the use of multiple modalities.

Ingenol mebutate is the active compound found in the sap of Euphorbia peplus used for the topical treatment of various skin conditions, including AKs.¹⁷ Ingenol mebutate gel 0.05% once daily for 2 days has been approved by the US Food and Drug Administration for the topical treatment of AKs. The mechanism of action of ingenol mebutate in AK therapy is not yet fully understood. In vivo and in vitro models have demonstrated both an induction of local lesion cell death and promotion of lesion-specific inflammatory response.¹⁸ When used in the treatment of AKs, ingenol mebutate gel 0.05% may cause a mild to moderate localized inflammatory response (eg, erythema, flaking/scaling, crusting, vesiculation/pustulation, erosion/ulceration, edema).

Our case is a rare report of successful treatment of DSAP with ingenol mebutate gel 0.05%. We found that treatment with ingenol mebutate gel 0.05% resulted in clinical improvement of DSAP lesions with minimal discomfort and good cosmetic response. This 2-day regimen is easy to use and patient friendly, improving medication compliance in such a cumbersome disease. We hope this case suggests that ingenol mebutate gel 0.05% could be a useful treatment alternative for DSAP, but future clinical studies should be conducted.

Disseminated superficial actinic porokeratosis (DSAP) is a chronic condition characterized by numerous atrophic papules and patches with a distinctive peripheral keratotic ridge, typically found on sun-exposed areas.^1,2 Treatment of DSAP is warranted not only for cosmetic and symptomatic benefits but also to prevent malignant transformation.^3,4 Successful treatment of DSAP often is difficult and frequently requires the use of multiple modalities. Ingenol mebutate gel 0.05% is a topical medication primarily used for the treatment of actinic keratosis (AK) by inducing cell death.⁵ We report a case of DSAP treated effectively with ingenol mebutate gel 0.05%.

Case Report

A 37-year-old woman was referred to the dermatology department for counseling for pseudoxanthoma elasticum (PXE), which had been proven on biopsy by an outside dermatologist 2 years prior. Physical examination revealed yellow papules on the neck that were characteristic of PXE, but no lesions were noted on the arms or legs. The only other cutaneous finding was a soft nodule on the right hip consistent with a lipoma. The patient returned to our institution 6 years later with lesions on both lower legs. She reported that these lesions had been present for 3 years and were exacerbated by sun exposure. On physical examination, multiple scattered, erythematous, annular, scaling papules and plaques were noted on the bilateral legs. A biopsy showed the histopathologic findings of DSAP (Figure 1). The patient had no family history of DSAP or PXE.

To determine the best treatment modality, we treated 4 test areas on both upper and lower legs: one with trichloroacetic acid (TCA), one with cryotherapy, one with imiquimod cream 5%, and one with tretinoin cream 0.1%. The patient returned 4 weeks later and showed modest response to TCA, cryotherapy, and tretinoin cream. Because cryotherapy was determined to be most effective, 20 more lesions were frozen at that visit. Over the next 2 years, the patient was treated with TCA, imiquimod cream 5%, and tretinoin cream 0.1%, but all ultimately proved ineffective for DSAP.

The patient returned 2 years after treatment failure (age 47 years) and was prescribed ingenol mebutate gel 0.05% for 2 days over an area of 25 cm² on the right lower leg (Figure 2A). She returned for follow-up at days 3, 15, 30, and 60. At day 3, the patient developed an inflammatory response to the medication with moderate erythema and scaling of individual lesions. No vesiculation, pustulation, edema, or ulceration was exhibited (Figure 2B). At day 30, there was a marked reduction in scaling with some postinflammatory erythema (Figure 2C). At day 60, much of the erythema had faded and the scale remained notably reduced (Figure 2D).

Comment

Disseminated superficial actinic porokeratosis is the most common subtype of porokeratosis, a keratinization disorder. There are 6 subtypes of porokeratosis identified in the literature: DSAP, disseminated superficial porokeratosis, classic porokeratosis of Mibelli, porokeratosis plantaris palmaris et disseminata, linear porokeratosis, and punctate porokeratosis.⁶ Disseminated superficial actinic porokeratosis has a female predominance (1.8:1 ratio)⁷ and generally appears in the third or fourth decades of life. Clonal proliferations of atypical keratinocytes have been implicated in the etiology of DSAP; however, the exact pathogenesis is unclear. Risk factors for DSAP include genetic susceptibility (eg, autosomal-dominant inheritance pattern), exposure to UV radiation, and drug-related immunosuppression or immunodeficiency.⁷ Other proposed etiologic risk factors include trauma and infection.⁸ Clinical diagnosis of DSAP is confirmed by the histological presence of a cornoid lamella (a thin column ofparakeratotic cells), a thinning epidermis, an absent or thinned granular cell layer, and a prominent dermal lymphocytic infiltrate.^9,10

Disseminated superficial actinic porokeratosis clinically presents as small atrophic scaly papules and/or patches with raised peripheral ridges symmetrically dispersed on sun-exposed areas of the arms, legs, back, and shoulders. Although these lesions are extensive, they typically spare the mucous membranes, palms, and soles¹¹; only a small percentage of cases report facial lesions,¹² which often are asymptomatic but cosmetically bothersome. Additionally, approximately half of patients report symptoms of pruritus and/or stinging,¹³ thus treatment of DSAP is mainly indicated for symptomatic relief and cosmetic purposes. Malignant degeneration^14,15 occurs in approximately 7.5% to 11% of porokeratosis cases,^10,16 warranting treatment for preventative measures.

Management of DSAP is dependent on the extent of the disease and the level of concern for malignant transformation. Localized disease can be treated with cryotherapy, CO₂ laser, and/or ablative techniques (eg, excision, curettage, dermabrasion) with variable degrees of success but high risk for scarring.¹ More extensive disease requires treatment with topical retinoids, topical 5-fluorouracil, imiquimod cream 5%, diclofenac gel 3%, topical vitamin D₃ analogues, and photodynamic therapy.¹ Several other therapies have been reported in the literature with partial and/or complete success, including systemic retinoids (eg, acitretin), Q-switched ruby laser, Nd:YAG laser, fractional photothermolysis, Grenz rays, pulsed dye laser, fractional photothermolysis, topical corticosteroids, and fluor-hydroxy pulse peel.⁶ Although there is an extensive array of therapies for DSAP, treatment results are variable with mostly limited success. Successful treatment of DSAP is difficult and often requires the use of multiple modalities.

Ingenol mebutate is the active compound found in the sap of Euphorbia peplus used for the topical treatment of various skin conditions, including AKs.¹⁷ Ingenol mebutate gel 0.05% once daily for 2 days has been approved by the US Food and Drug Administration for the topical treatment of AKs. The mechanism of action of ingenol mebutate in AK therapy is not yet fully understood. In vivo and in vitro models have demonstrated both an induction of local lesion cell death and promotion of lesion-specific inflammatory response.¹⁸ When used in the treatment of AKs, ingenol mebutate gel 0.05% may cause a mild to moderate localized inflammatory response (eg, erythema, flaking/scaling, crusting, vesiculation/pustulation, erosion/ulceration, edema).

Our case is a rare report of successful treatment of DSAP with ingenol mebutate gel 0.05%. We found that treatment with ingenol mebutate gel 0.05% resulted in clinical improvement of DSAP lesions with minimal discomfort and good cosmetic response. This 2-day regimen is easy to use and patient friendly, improving medication compliance in such a cumbersome disease. We hope this case suggests that ingenol mebutate gel 0.05% could be a useful treatment alternative for DSAP, but future clinical studies should be conducted.

MAGNIFY is a phase IIIB randomized trial actively recruiting patients with relapsed/refractory mantle cell lymphoma, based on studies posted at ClinicalTrials.gov.

MAGNIFY (NCT01996865) is a study of lenalidomide (CC-5013) plus rituximab maintenance therapy, followed by lenalidomide single-agent maintenance therapy, versus rituximab. Sponsored by Celgene, the maker of lenalidomide (Revlimid), the trial’s primary outcome measure is progression-free survival at up to 8 years.

The MAGNIFY trial includes patients with grades 1-3b or transformed follicular lymphoma, marginal zone lymphoma, or mantle cell lymphoma who had received at least one prior therapy and had stage I-IV measurable disease. About 500 patients are planned to enroll in 12 cycles of R2 induction, and slightly more than 300 patients are projected to be randomized after induction to the two maintenance arms. Induction includes oral lenalidomide 20 mg/day, days 1-21 per 28-day cycle; plus intravenous rituximab 375 mg/m² on days 1, 8, 15, and 22 of cycle 1 and day 1 of cycles 3, 5, 7, 9, and 11 (28-day cycles).

Patients will then be randomized to maintenance lenalidomide 10 mg/day, given on days 1-21 per 28-day cycle for cycles 13-30; plus rituximab 375 mg/m², given on day 1 of cycles 13, 15, 17, 19, 21, 23, 25, 27, and 29 (R2, Arm A), or rituximab alone (same schedule, Arm B). Patients receiving R2 maintenance after 18 cycles may continue maintenance lenalidomide monotherapy at 10 mg/day, days 1-21 per 28-day cycle, at the discretion of the patient and/or investigator, until disease progression as tolerated.

The primary endpoint is progression-free survival. Secondary endpoints include safety, overall survival, response rates, duration of response, and quality of life. Patients will be followed for at least 5 years after the last patient-initiated induction therapy. Enrollment in MAGNIFY began in March 2014; as of January 2016, 133 patients are enrolled, according to the study page at ClinicalTrials.gov.
 

MAGNIFY is a phase IIIB randomized trial actively recruiting patients with relapsed/refractory mantle cell lymphoma, based on studies posted at ClinicalTrials.gov.

MAGNIFY (NCT01996865) is a study of lenalidomide (CC-5013) plus rituximab maintenance therapy, followed by lenalidomide single-agent maintenance therapy, versus rituximab. Sponsored by Celgene, the maker of lenalidomide (Revlimid), the trial’s primary outcome measure is progression-free survival at up to 8 years.

The MAGNIFY trial includes patients with grades 1-3b or transformed follicular lymphoma, marginal zone lymphoma, or mantle cell lymphoma who had received at least one prior therapy and had stage I-IV measurable disease. About 500 patients are planned to enroll in 12 cycles of R2 induction, and slightly more than 300 patients are projected to be randomized after induction to the two maintenance arms. Induction includes oral lenalidomide 20 mg/day, days 1-21 per 28-day cycle; plus intravenous rituximab 375 mg/m² on days 1, 8, 15, and 22 of cycle 1 and day 1 of cycles 3, 5, 7, 9, and 11 (28-day cycles).

Patients will then be randomized to maintenance lenalidomide 10 mg/day, given on days 1-21 per 28-day cycle for cycles 13-30; plus rituximab 375 mg/m², given on day 1 of cycles 13, 15, 17, 19, 21, 23, 25, 27, and 29 (R2, Arm A), or rituximab alone (same schedule, Arm B). Patients receiving R2 maintenance after 18 cycles may continue maintenance lenalidomide monotherapy at 10 mg/day, days 1-21 per 28-day cycle, at the discretion of the patient and/or investigator, until disease progression as tolerated.

The primary endpoint is progression-free survival. Secondary endpoints include safety, overall survival, response rates, duration of response, and quality of life. Patients will be followed for at least 5 years after the last patient-initiated induction therapy. Enrollment in MAGNIFY began in March 2014; as of January 2016, 133 patients are enrolled, according to the study page at ClinicalTrials.gov.
 

MAGNIFY is a phase IIIB randomized trial actively recruiting patients with relapsed/refractory mantle cell lymphoma, based on studies posted at ClinicalTrials.gov.

MAGNIFY (NCT01996865) is a study of lenalidomide (CC-5013) plus rituximab maintenance therapy, followed by lenalidomide single-agent maintenance therapy, versus rituximab. Sponsored by Celgene, the maker of lenalidomide (Revlimid), the trial’s primary outcome measure is progression-free survival at up to 8 years.

The MAGNIFY trial includes patients with grades 1-3b or transformed follicular lymphoma, marginal zone lymphoma, or mantle cell lymphoma who had received at least one prior therapy and had stage I-IV measurable disease. About 500 patients are planned to enroll in 12 cycles of R2 induction, and slightly more than 300 patients are projected to be randomized after induction to the two maintenance arms. Induction includes oral lenalidomide 20 mg/day, days 1-21 per 28-day cycle; plus intravenous rituximab 375 mg/m² on days 1, 8, 15, and 22 of cycle 1 and day 1 of cycles 3, 5, 7, 9, and 11 (28-day cycles).

Patients will then be randomized to maintenance lenalidomide 10 mg/day, given on days 1-21 per 28-day cycle for cycles 13-30; plus rituximab 375 mg/m², given on day 1 of cycles 13, 15, 17, 19, 21, 23, 25, 27, and 29 (R2, Arm A), or rituximab alone (same schedule, Arm B). Patients receiving R2 maintenance after 18 cycles may continue maintenance lenalidomide monotherapy at 10 mg/day, days 1-21 per 28-day cycle, at the discretion of the patient and/or investigator, until disease progression as tolerated.

The primary endpoint is progression-free survival. Secondary endpoints include safety, overall survival, response rates, duration of response, and quality of life. Patients will be followed for at least 5 years after the last patient-initiated induction therapy. Enrollment in MAGNIFY began in March 2014; as of January 2016, 133 patients are enrolled, according to the study page at ClinicalTrials.gov.
 

MIAMI BEACH – Survival for patients with hilar cholangiocarcinoma was similar between those who underwent transplantation and those who underwent resection, but neoadjuvant therapy may give transplant strategy the edge, findings of a study and meta-analysis suggest.

“Neoadjuvant chemoradiation therapy is clearly a factor that affects patient survival, and may be the only reason the patients who received transplantation had better overall survival than [did those who had] resection,” Michele Gage, MD, a general surgeon at Johns Hopkins Medicine in Baltimore, said at the annual meeting of the Americas Hepato-Pancreato-Biliary Association.

Comstock/Thinkstock

MIAMI BEACH – Survival for patients with hilar cholangiocarcinoma was similar between those who underwent transplantation and those who underwent resection, but neoadjuvant therapy may give transplant strategy the edge, findings of a study and meta-analysis suggest.

“Neoadjuvant chemoradiation therapy is clearly a factor that affects patient survival, and may be the only reason the patients who received transplantation had better overall survival than [did those who had] resection,” Michele Gage, MD, a general surgeon at Johns Hopkins Medicine in Baltimore, said at the annual meeting of the Americas Hepato-Pancreato-Biliary Association.

Comstock/Thinkstock

MIAMI BEACH – Survival for patients with hilar cholangiocarcinoma was similar between those who underwent transplantation and those who underwent resection, but neoadjuvant therapy may give transplant strategy the edge, findings of a study and meta-analysis suggest.

“Neoadjuvant chemoradiation therapy is clearly a factor that affects patient survival, and may be the only reason the patients who received transplantation had better overall survival than [did those who had] resection,” Michele Gage, MD, a general surgeon at Johns Hopkins Medicine in Baltimore, said at the annual meeting of the Americas Hepato-Pancreato-Biliary Association.

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SAN ANTONIO – Investigators at the University of Tennessee, Chattanooga, have come up with a simple scoring system to predict if an intact uterus can be delivered vaginally during laparoscopic hysterectomy.

Age greater than 50 years counts as 1 point and uterine length greater than 11 cm, height greater than 8 cm, and width greater than 6.9 cm each count for 3 points. A score of 4 or higher suggests the need for an alternative to vaginal extraction, they reported at the annual scientific meeting of the Society of Gynecologic Surgeons.

* The meeting sponsor information was updated 6/9/2017.

[email protected]

SAN ANTONIO – Investigators at the University of Tennessee, Chattanooga, have come up with a simple scoring system to predict if an intact uterus can be delivered vaginally during laparoscopic hysterectomy.

Age greater than 50 years counts as 1 point and uterine length greater than 11 cm, height greater than 8 cm, and width greater than 6.9 cm each count for 3 points. A score of 4 or higher suggests the need for an alternative to vaginal extraction, they reported at the annual scientific meeting of the Society of Gynecologic Surgeons.

* The meeting sponsor information was updated 6/9/2017.

[email protected]

SAN ANTONIO – Investigators at the University of Tennessee, Chattanooga, have come up with a simple scoring system to predict if an intact uterus can be delivered vaginally during laparoscopic hysterectomy.

Age greater than 50 years counts as 1 point and uterine length greater than 11 cm, height greater than 8 cm, and width greater than 6.9 cm each count for 3 points. A score of 4 or higher suggests the need for an alternative to vaginal extraction, they reported at the annual scientific meeting of the Society of Gynecologic Surgeons.

* The meeting sponsor information was updated 6/9/2017.

[email protected]

You walk into an examining room and discover a 3-year-old in his underpants wearing a fireman’s hat and what must be his older sister’s rubber boots. You have to ask: “Are you going to be a firefighter when you grow up?” If he is a sensitive kid he will resist answering, “What do you think, Dr. Obvious?” and instead he politely replies, “Yes, and an EMT [emergency medical technician] too.”

Adults, even ones who have devoted their professional lives to the care of children, can’t seem to stifle the urge ask every young person they meet about his or her career plans. It is a strange sort of obsession, and may simply reflect the fact that most adults are at a loss for conversation starters when it comes to talking with young people. Children don’t seem to have much concern about the weather. And most of them don’t have opinions about the current political situation. They don’t have stories about their grandchildren they would love to bore you with. You could ask if the child has a pet, but that may be picking the scab of an unresolved family issue.

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected].

You walk into an examining room and discover a 3-year-old in his underpants wearing a fireman’s hat and what must be his older sister’s rubber boots. You have to ask: “Are you going to be a firefighter when you grow up?” If he is a sensitive kid he will resist answering, “What do you think, Dr. Obvious?” and instead he politely replies, “Yes, and an EMT [emergency medical technician] too.”

Adults, even ones who have devoted their professional lives to the care of children, can’t seem to stifle the urge ask every young person they meet about his or her career plans. It is a strange sort of obsession, and may simply reflect the fact that most adults are at a loss for conversation starters when it comes to talking with young people. Children don’t seem to have much concern about the weather. And most of them don’t have opinions about the current political situation. They don’t have stories about their grandchildren they would love to bore you with. You could ask if the child has a pet, but that may be picking the scab of an unresolved family issue.

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected].

You walk into an examining room and discover a 3-year-old in his underpants wearing a fireman’s hat and what must be his older sister’s rubber boots. You have to ask: “Are you going to be a firefighter when you grow up?” If he is a sensitive kid he will resist answering, “What do you think, Dr. Obvious?” and instead he politely replies, “Yes, and an EMT [emergency medical technician] too.”

Adults, even ones who have devoted their professional lives to the care of children, can’t seem to stifle the urge ask every young person they meet about his or her career plans. It is a strange sort of obsession, and may simply reflect the fact that most adults are at a loss for conversation starters when it comes to talking with young people. Children don’t seem to have much concern about the weather. And most of them don’t have opinions about the current political situation. They don’t have stories about their grandchildren they would love to bore you with. You could ask if the child has a pet, but that may be picking the scab of an unresolved family issue.

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected].

Almost 200 cases of pregnant women with Zika virus infection were reported in the United States during the 2 weeks ending March 28, with the number split evenly between the territories and the 50 states and Washington, D.C., according to the Centers for Disease Control and Prevention.

These latest 197 cases – 98 in the territories and 99 in the states/D.C. – bring the U.S. total since the beginning of 2016 to 5,177 pregnant women with laboratory evidence of Zika virus infection: 3,461 in the U.S. territories and 1,716 in the states/D.C., the CDC reported on April 6.

[email protected]

Almost 200 cases of pregnant women with Zika virus infection were reported in the United States during the 2 weeks ending March 28, with the number split evenly between the territories and the 50 states and Washington, D.C., according to the Centers for Disease Control and Prevention.

These latest 197 cases – 98 in the territories and 99 in the states/D.C. – bring the U.S. total since the beginning of 2016 to 5,177 pregnant women with laboratory evidence of Zika virus infection: 3,461 in the U.S. territories and 1,716 in the states/D.C., the CDC reported on April 6.

[email protected]

Almost 200 cases of pregnant women with Zika virus infection were reported in the United States during the 2 weeks ending March 28, with the number split evenly between the territories and the 50 states and Washington, D.C., according to the Centers for Disease Control and Prevention.

These latest 197 cases – 98 in the territories and 99 in the states/D.C. – bring the U.S. total since the beginning of 2016 to 5,177 pregnant women with laboratory evidence of Zika virus infection: 3,461 in the U.S. territories and 1,716 in the states/D.C., the CDC reported on April 6.

[email protected]

SAN DIEGO – Despite the popularity of medical marijuana, robust evidence for its use is limited or nonexistent for most medical conditions.

“This is a tough subject to study,” Ellie Grossman, MD, said at the annual meeting of the American College of Physicians. “There is federal money that can only be used in very limited ways to study it. Our science is way behind the times in terms of what our patients are doing and using.”

Typical limitations of marijuana studies include self-report of quantity/duration used and the fact that biochemical/quantifiable measures are lacking. “For inhaled marijuana, there is variability in how much is inhaled and how deeply it’s being inhaled,” said Dr. Grossman, an internist who practices in Somerville, Mass.

[email protected]

SAN DIEGO – Despite the popularity of medical marijuana, robust evidence for its use is limited or nonexistent for most medical conditions.

“This is a tough subject to study,” Ellie Grossman, MD, said at the annual meeting of the American College of Physicians. “There is federal money that can only be used in very limited ways to study it. Our science is way behind the times in terms of what our patients are doing and using.”

Typical limitations of marijuana studies include self-report of quantity/duration used and the fact that biochemical/quantifiable measures are lacking. “For inhaled marijuana, there is variability in how much is inhaled and how deeply it’s being inhaled,” said Dr. Grossman, an internist who practices in Somerville, Mass.

[email protected]

SAN DIEGO – Despite the popularity of medical marijuana, robust evidence for its use is limited or nonexistent for most medical conditions.

“This is a tough subject to study,” Ellie Grossman, MD, said at the annual meeting of the American College of Physicians. “There is federal money that can only be used in very limited ways to study it. Our science is way behind the times in terms of what our patients are doing and using.”

Typical limitations of marijuana studies include self-report of quantity/duration used and the fact that biochemical/quantifiable measures are lacking. “For inhaled marijuana, there is variability in how much is inhaled and how deeply it’s being inhaled,” said Dr. Grossman, an internist who practices in Somerville, Mass.

[email protected]

Where do we go from here?

More than 20 years after IV tissue plasminogen activator was officially approved for use in acute ischemic stroke, now data suggest mechanical thrombectomy is superior, regardless of whether tPA is given.

Granted, these are preliminary trials, and a lot more research needs to be done: randomized studies, determinations of which patients are the best candidates, which devices are most useful, etc.

Dr. Block has a solo neurology practice in Scottsdale, Ariz.

Where do we go from here?

More than 20 years after IV tissue plasminogen activator was officially approved for use in acute ischemic stroke, now data suggest mechanical thrombectomy is superior, regardless of whether tPA is given.

Granted, these are preliminary trials, and a lot more research needs to be done: randomized studies, determinations of which patients are the best candidates, which devices are most useful, etc.

Dr. Block has a solo neurology practice in Scottsdale, Ariz.

Where do we go from here?

More than 20 years after IV tissue plasminogen activator was officially approved for use in acute ischemic stroke, now data suggest mechanical thrombectomy is superior, regardless of whether tPA is given.

Granted, these are preliminary trials, and a lot more research needs to be done: randomized studies, determinations of which patients are the best candidates, which devices are most useful, etc.

Dr. Block has a solo neurology practice in Scottsdale, Ariz.

Your first patient of the afternoon is a 9-year-old boy who moved to town several months ago. Mercifully, the second patient of the afternoon has canceled, giving you a few more minutes to get acquainted with this young man whose chief complaint is listed as “behavior problem.” You learn quickly that this family has relocated from a town just 20 miles away because they are seeking a school that is a “better fit” for your new patient.

Due to some miscommunications, the child’s old records have not arrived at your office. The mother says that her son is not taking any medication, and she isn’t sure if he has ever been given a diagnosis. You learn that he likes to argue and is prone to violent temper tantrums. Your initial brief exam does not suggest any cognitive deficits, but he exudes an aura of anger and discontent. You tell his mother that you will be glad to try to help, but you will need his old records and another longer visit before you can make any recommendations.

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected]

Your first patient of the afternoon is a 9-year-old boy who moved to town several months ago. Mercifully, the second patient of the afternoon has canceled, giving you a few more minutes to get acquainted with this young man whose chief complaint is listed as “behavior problem.” You learn quickly that this family has relocated from a town just 20 miles away because they are seeking a school that is a “better fit” for your new patient.

Due to some miscommunications, the child’s old records have not arrived at your office. The mother says that her son is not taking any medication, and she isn’t sure if he has ever been given a diagnosis. You learn that he likes to argue and is prone to violent temper tantrums. Your initial brief exam does not suggest any cognitive deficits, but he exudes an aura of anger and discontent. You tell his mother that you will be glad to try to help, but you will need his old records and another longer visit before you can make any recommendations.

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected]

Your first patient of the afternoon is a 9-year-old boy who moved to town several months ago. Mercifully, the second patient of the afternoon has canceled, giving you a few more minutes to get acquainted with this young man whose chief complaint is listed as “behavior problem.” You learn quickly that this family has relocated from a town just 20 miles away because they are seeking a school that is a “better fit” for your new patient.

Due to some miscommunications, the child’s old records have not arrived at your office. The mother says that her son is not taking any medication, and she isn’t sure if he has ever been given a diagnosis. You learn that he likes to argue and is prone to violent temper tantrums. Your initial brief exam does not suggest any cognitive deficits, but he exudes an aura of anger and discontent. You tell his mother that you will be glad to try to help, but you will need his old records and another longer visit before you can make any recommendations.

Dr. Wilkoff practiced primary care pediatrics in Brunswick, Maine for nearly 40 years. He has authored several books on behavioral pediatrics, including “How to Say No to Your Toddler.” Email him at [email protected]