Epidermal nevus syndrome with bilateral renal artery stenosis and midaortic syndrome
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P5961 Epidermal nevus syndrome with bilateral renal artery stenosis and midaortic syndrome Erin Ducharme, MD, Scott and White Memorial Hospital, Texas A&M University Health Science Center, Temple, TX, United States; Jennifer Pike, Scott and White Memorial Hospital, Texas A&M University Health Science Center, Temple, TX, United States A 7-year-old black girl with hypertension secondary to bilateral renal artery stenosis (RAS) was referred to our clinic for evaluation of hyperpigmented lesions noted since birth. Imaging also revealed a small caliber aorta consistent with midaortic syndrome (MAS). Previous medical services diagnosed the lesions as ‘‘atypical’’ caf e- au-lait macules with a presumptive diagnosis of neurofibromatosis type 1 (NF), the most common underlying syndrome in children with bilateral RAS as well as MAS. When genetic testing failed to confirm NF she presented with the request for lesional biopsy with chromosomal analysis. Clinical and histopathologic examina- tion were consistent with epidermal nevi involving the back, chest and right arm and she was diagnosed with epidermal nevus syndrome (ENS). A review of the literature revealed only two previous reports of ENS with renal artery stenosis; in both of these cases other vascular anomalies were also present. We suggest that the link between ENS and BAS may be underreported or underrecognized and recommend a blood pressure measurement in any child with epidermal nevus syndrome as an inexpensive screening tool. Commercial support: None identified. P6359 Histopathology and clinical features can predict the risk of renal and other systemic involvement in adult HenocheSch€ onlein purpura Timothy Poterucha, Mayo Clinic College of Medicine, Rochester, MN, United States; Christine Lohse, MS, Mayo Clinic Division of Biomedical Statistics and Informatics, Rochester, MN, United States; David Wetter, MD, Mayo Clinic Department of Dermatology, Rochester, MN, United States; Lawrence Gibson, MD, Mayo Clinic Department of Dermatology, Rochester, MN, United States; Michael Camilleri, MD, Mayo Clinic Department of Dermatology, Rochester, MN, United States Background: The histopathology of HenocheSch€ onlein purpura (HSP) is well defined, but specific markers have not been correlated with systemic involvement. Objective: To evaluate whether histopathologic markers were associated with renal or other systemic involvement in adult HSP. Methods: We retrospectively reviewed clinical information and pathology slides of 68 adult patients with HSP seen at Mayo Clinic between 1992 and 2011. Results: Of the 68 patients, mean age was 45.8 years and 41 (60%) of the patients were male. Renal involvement was observed in 30 patients (44%), gastrointestinal (GI) tract in 27 (40%), joint in 32 (47%), and any systemic signs in 52 (76%). Patients who were older than 40 years and had leukocytoclastic vasculitis (LCV) and an absence of eosinophils on skin biopsy had higher rates of renal involvement than those who did not have both of these features (75% vs 27%; P \.001). Patients with skin biopsies showing LCV and an absence of histiocytes had higher rates of GI tract involvement (P ¼ .03). Age of 40 years or less was associated with increased risk for GI tract involvement and a nonsignificant trend for joint involvement (P ¼.004 and P ¼.06, respectively). Limitations: This study is retrospective, and the etiologic factors of HSP were unable to be determined in many patients. Conclusion: Patients older than 40 years of age with HSP who had an absence of eosinophils on skin biopsy had a nearly 3-times increased risk of renal involvement compared with patients who did not have both features. Commercial support: None identified. P6407 Intravenous immunoglobulin as a steroid-sparing agent in recalcitrant drug reaction with eosinophilia and systemic symptoms syndrome Elisha Singer, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States; Karolyn Wanat, MD, Department of Dermatology, University of Pennsylvania, Philadelphia, PA, United States; Misha Rosenbach, MD, Department of Dermatology, University of Pennsylvania, Philadelphia, PA, United States DRESS (drug reaction with eosinophilia and systemic symptoms) is a life-threatening syndrome characterized by rash, fever, lymphadenopathy, hematologic abnormal- ities and internal organ involvement. Potential long-term sequelae include type 1 diabetes, hypothyroidism, hepatic failure, renal failure, and myocarditis. We report a case of a 21-year-old female with bipolar disorder who was started on lamotrigine and two weeks later presented to an outside hospital with signs and symptoms consistent with DRESS syndrome. She was successfully treated with methylpred- nisolone and discharged on a prednisone taper but had recrudescence of systemic symptoms during the course of her prednisone taper. She was readmitted and treated with corticosteroids but again became symptomatic while tapering from prednisone, prompting referral to our institution for further management. She was subsequently treated with prednisone and then started her on intravenous immu- noglobulin as a steroid-sparing agent. After 4 months of IVIG treatment, she was successfully tapered entirely off steroids without symptom recurrence. Though IVIG has been used as an adjunctive treatment in cases unresponsive to corticoste- roids alone, we present a case of IVIG used specifically as a steroid-sparing agent in DRESS syndrome, highlighting its potential role in this clinical setting. IVIG has been used as a steroid-tapering agent in multiple diseases, and its anti-inflammatory and immune regulatory effects are effective in treating other severe adverse drug reactions, particularly toxic epidermal necrolysis. We suggest considering IVIG use in severe, steroid-dependent DRESS cases. Commercial support: None identified. P6977 Lichen planus pigmentosus in patients with endocrinopathies and hepa- titis C Jiram Torres, MD, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico; Adriana Guadalupe Pe~ na Romero, MD, Instituto Nacional De Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico; Edgardo Reyes, MD, Instituto Nacional De Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico; Linda Garcia Hidalgo, MD, Instituto Nacional De Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico Background: Lichen planus pigmentosus (LPP) is a chronic inflammatory disease characterized by hyperpigmented, dark-brown macules in sun exposed areas and flexural folds. This disease tends to occur in patients with darker-pigmented skin. Although its pathophysiology is not well understood, some studies confirm that immunological mechanisms are involved in its development. It is related to many diseases, such as infection by hepatitis C virus (HCV), autoimmune diseases, and recently with endocrinopathies, such as dyslipidemia, thyroid disease, and a defective enzyme expression in keratinocytes. The last one is related with an impaired carbohydrate metabolism in these patients. This relationship with multiple endocrine abnormalities is poorly understood but has been explained by a chronic inflammatory state with increased activity of cytotoxic T lymphocytes, interleukin 6 and interferon alfa, the latter causes a dephosphorylation of the insulin receptor causing insulin resistance. However there is no studies on Latin American population. Methods: We performed a data collection of patients with clinical and/or biopsy diagnosis of LPP in a Mexican hospital during a period of 24 years. Demographic and clinical data were collected. Results: We found a total of 18 patients with this diagnosis, 14 (77.7%) were female and 4 (22.2%) males, 7 (38.8%) patients had a diagnosis of diabetes mellitus (DM), 9 (50%) dyslipidemia, 4 (22.2%) autoimmune thyroid disorders, 2 (11.1%) hypogo- nadotropic hypogonadism. Only one of the patients had HCV. Conclusion: In our study the presence of LPP could be related to the phototype IV and V of our population and this entity could be also related to endocrinopathies mainly dyslipidemia and DM, this could be explained by the proinflammatory state that characterizes the LPP. In addition LPP might have a less tight relationship with HCV infection compared with other subtypes of lichen planus. Therefore, it may be prudent to follow-up patients with LP for the development of cardiovascular risk factors to permit an early detection and initiation of appropriate treatment. Commercial support: None identified. APRIL 2013 JAM ACAD DERMATOL AB139
Transcript of Epidermal nevus syndrome with bilateral renal artery stenosis and midaortic syndrome
P5961Epidermal nevus syndrome with bilateral renal artery stenosis andmidaortic syndrome
Erin Ducharme, MD, Scott and White Memorial Hospital, Texas A&M UniversityHealth Science Center, Temple, TX, United States; Jennifer Pike, Scott and WhiteMemorial Hospital, Texas A&M University Health Science Center, Temple, TX,United States
A 7-year-old black girl with hypertension secondary to bilateral renal artery stenosis(RAS) was referred to our clinic for evaluation of hyperpigmented lesions notedsince birth. Imaging also revealed a small caliber aorta consistent with midaorticsyndrome (MAS). Previous medical services diagnosed the lesions as ‘‘atypical’’ caf�e-au-lait macules with a presumptive diagnosis of neurofibromatosis type 1 (NF), themost common underlying syndrome in children with bilateral RAS as well as MAS.When genetic testing failed to confirm NF she presented with the request forlesional biopsy with chromosomal analysis. Clinical and histopathologic examina-tion were consistent with epidermal nevi involving the back, chest and right armand she was diagnosed with epidermal nevus syndrome (ENS). A review of theliterature revealed only two previous reports of ENS with renal artery stenosis; inboth of these cases other vascular anomalies were also present. We suggest that thelink between ENS and BAS may be underreported or underrecognized andrecommend a blood pressure measurement in any child with epidermal nevussyndrome as an inexpensive screening tool.
APRIL 20
cial support: None identified.
CommerP6359Histopathology and clinical features can predict the risk of renal andother systemic involvement in adult HenocheSch€onlein purpura
Timothy Poterucha, Mayo Clinic College of Medicine, Rochester, MN, UnitedStates; Christine Lohse, MS, Mayo Clinic Division of Biomedical Statistics andInformatics, Rochester, MN, United States; David Wetter, MD, Mayo ClinicDepartment of Dermatology, Rochester, MN, United States; Lawrence Gibson,MD, Mayo Clinic Department of Dermatology, Rochester, MN, United States;Michael Camilleri, MD, Mayo Clinic Department of Dermatology, Rochester, MN,United States
Background: The histopathology of HenocheSch€onlein purpura (HSP) is welldefined, but specific markers have not been correlated with systemic involvement.
Objective: To evaluate whether histopathologic markers were associated with renalor other systemic involvement in adult HSP.
Methods: We retrospectively reviewed clinical information and pathology slides of68 adult patients with HSP seen at Mayo Clinic between 1992 and 2011. Results: Ofthe 68 patients, mean age was 45.8 years and 41 (60%) of the patients were male.Renal involvement was observed in 30 patients (44%), gastrointestinal (GI) tract in27 (40%), joint in 32 (47%), and any systemic signs in 52 (76%). Patients who wereolder than 40 years and had leukocytoclastic vasculitis (LCV) and an absence ofeosinophils on skin biopsy had higher rates of renal involvement than those who didnot have both of these features (75% vs 27%; P\.001). Patients with skin biopsiesshowing LCV and an absence of histiocytes had higher rates of GI tract involvement(P ¼ .03). Age of 40 years or less was associated with increased risk for GI tractinvolvement and a nonsignificant trend for joint involvement (P ¼.004 and P ¼.06,respectively).
Limitations: This study is retrospective, and the etiologic factors of HSP were unableto be determined in many patients.
Conclusion: Patients older than 40 years of age with HSP who had an absence ofeosinophils on skin biopsy had a nearly 3-times increased risk of renal involvementcompared with patients who did not have both features.
cial support: None identified.
Commer13
P6407Intravenous immunoglobulin as a steroid-sparing agent in recalcitrantdrug reaction with eosinophilia and systemic symptoms syndrome
Elisha Singer, Perelman School of Medicine at the University of Pennsylvania,Philadelphia, PA, United States; Karolyn Wanat, MD, Department of Dermatology,University of Pennsylvania, Philadelphia, PA, United States; Misha Rosenbach,MD, Department of Dermatology, University of Pennsylvania, Philadelphia, PA,United States
DRESS (drug reaction with eosinophilia and systemic symptoms) is a life-threateningsyndrome characterized by rash, fever, lymphadenopathy, hematologic abnormal-ities and internal organ involvement. Potential long-term sequelae include type1 diabetes, hypothyroidism, hepatic failure, renal failure, and myocarditis. We reporta case of a 21-year-old female with bipolar disorder who was started on lamotrigineand two weeks later presented to an outside hospital with signs and symptomsconsistent with DRESS syndrome. She was successfully treated with methylpred-nisolone and discharged on a prednisone taper but had recrudescence of systemicsymptoms during the course of her prednisone taper. She was readmitted andtreated with corticosteroids but again became symptomatic while tapering fromprednisone, prompting referral to our institution for further management. She wassubsequently treated with prednisone and then started her on intravenous immu-noglobulin as a steroid-sparing agent. After 4 months of IVIG treatment, she wassuccessfully tapered entirely off steroids without symptom recurrence. ThoughIVIG has been used as an adjunctive treatment in cases unresponsive to corticoste-roids alone, we present a case of IVIG used specifically as a steroid-sparing agent inDRESS syndrome, highlighting its potential role in this clinical setting. IVIG has beenused as a steroid-tapering agent in multiple diseases, and its anti-inflammatory andimmune regulatory effects are effective in treating other severe adverse drugreactions, particularly toxic epidermal necrolysis. We suggest considering IVIG usein severe, steroid-dependent DRESS cases.
cial support: None identified.
CommerP6977Lichen planus pigmentosus in patients with endocrinopathies and hepa-titis C
Jiram Torres, MD, Instituto Nacional de Ciencias Medicas y Nutricion SalvadorZubiran, Mexico City, Mexico; Adriana Guadalupe Pe~na Romero, MD, InstitutoNacional De Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City,Mexico; Edgardo Reyes, MD, Instituto Nacional De Ciencias Medicas yNutricion Salvador Zubiran, Mexico City, Mexico; Linda Garcia Hidalgo, MD,Instituto Nacional De Ciencias Medicas y Nutricion Salvador Zubiran, MexicoCity, Mexico
Background: Lichen planus pigmentosus (LPP) is a chronic inflammatory diseasecharacterized by hyperpigmented, dark-brown macules in sun exposed areas andflexural folds. This disease tends to occur in patients with darker-pigmented skin.Although its pathophysiology is not well understood, some studies confirm thatimmunological mechanisms are involved in its development. It is related to manydiseases, such as infection by hepatitis C virus (HCV), autoimmune diseases, andrecently with endocrinopathies, such as dyslipidemia, thyroid disease, and adefective enzyme expression in keratinocytes. The last one is related with animpaired carbohydrate metabolism in these patients. This relationship withmultipleendocrine abnormalities is poorly understood but has been explained by a chronicinflammatory state with increased activity of cytotoxic T lymphocytes, interleukin 6and interferon alfa, the latter causes a dephosphorylation of the insulin receptorcausing insulin resistance. However there is no studies on Latin Americanpopulation.
Methods: We performed a data collection of patients with clinical and/or biopsydiagnosis of LPP in a Mexican hospital during a period of 24 years. Demographic andclinical data were collected.
Results: We found a total of 18 patients with this diagnosis, 14 (77.7%) were femaleand 4 (22.2%) males, 7 (38.8%) patients had a diagnosis of diabetes mellitus (DM), 9(50%) dyslipidemia, 4 (22.2%) autoimmune thyroid disorders, 2 (11.1%) hypogo-nadotropic hypogonadism. Only one of the patients had HCV.
Conclusion: In our study the presence of LPP could be related to the phototype IVand V of our population and this entity could be also related to endocrinopathiesmainly dyslipidemia and DM, this could be explained by the proinflammatory statethat characterizes the LPP. In addition LPP might have a less tight relationship withHCV infection compared with other subtypes of lichen planus. Therefore, it may beprudent to follow-up patients with LP for the development of cardiovascular riskfactors to permit an early detection and initiation of appropriate treatment.
cial support: None identified.
CommerJ AM ACAD DERMATOL AB139
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