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Aleukemic leukemia cutis preceding monocytic leukemia: A rare case diagnosed by dermatology
P7546
P8142
A case of pravastatin-induced DRESS syndrome Spencer Bezalel, University of South Florida Morsani College of Medicine, Tampa, FL, United States; Brooke Baldwin, MD, James A. Haley Veteran’s Hospital, Tampa, FL, United States; Drazen Jukic, MD, James A. Haley Veteran’s Hospital, Tampa, FL, United States; Nishit Patel, MD, University of South Florida Morsani College of Medicine, Tampa, FL, United States
Acral green pigmentation in cholestatic jaundice patient: A case report Daranporn Triwongwaranat, MD, Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Pranee Kasemsarn, MD, Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Waranya Boonchai, MD, Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand Cutaneous manifestations of hepatic diseases are various including jaundice, pruritus, palmar erythema, spider telangiectasia, Terry nails, Muehrcke nails, diffuse hyperpigmentation, and gynecomastia. Acral green pigmentation is a rare cutaneous manifestation found only in patients with hyperbilirubinemia. We report a 56-yearold man with intrahepatic cholangiocarcinoma presented with abrupt onset green macules and patches on both hyperkeratotic palms and soles. Dermatoscopic examination revealed linear distributions of green pigments predominate on dermatoglyphic ridges with furrow sparing. Histopathologic examination from left palm stained with hematoxylineeosin showed hyperkeratosis and pink amorphous material within stratum corneum. Special staining for bilirubin named Fouchet stain or Hall stain was negative. Other causes of green-pigmented lesions including pseudomonas infection and exogenous staining were excluded. Green pigmentation on palms and soles in patients with hyperbilirubinemia is a rare condition, with only 5 reported cases on literatures. Previous articles reported that the green pigmentations are mainly along dermatoglyphic ridges where the eccrines sweat pores open. In addition, all of reported patients have cutaneous distribution only on palms and soles; the most abundant areas of sweat glands. The evidences from clinical findings and dermatoscopic examination support that this condition is the variant of eccrine chromhidrosis. Although special staining for billirubin result was negative, not all previously reported cases showed positive for bile stain. There are many patients with hyperbilirubinemia from various causes, but acral green pigmentation is still rarely noticed. Some hidden factors may exist which will require examination more affected patients to elucidate. We report the first case of acral green pigmentation in cholangiocarcinoma patient.
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a drug-induced hypersensitivity reaction characterized by cutaneous eruption, eosinophilia, and involvement of other organ systems. It is differentiated from other drug reactions by its late onset (3 weeks to 3 months), the involvement of other organs, and the persistence of the reaction despite withdrawal of the inciting medication. The underlying mechanism remains to be elucidated, but abnormal epoxide hydroxylase, reactivation of latent viruses, and genetic predisposition involving specific allelic variants of human leukocyte antigen (HLA) are suggested to be involved. The majority of cases are related to aromatic anticonvulsants. Here we present a case of pravastatin-induced DRESS syndrome. Currently there is only 1 other published case involving a statin (atorvastatin). Keeping in mind a mortality rate between 10% and 20%, identification of all potential offending drugs is of utmost importance. With statins being one of the most highly prescribed medications worldwide, health care providers should be aware of this potentially lethal adverse reaction in a subset of the population. Commercial support: None identified.
Commercial support: None identified.
P7599 A woman with unusual nodules on her trunk and extremities Theresa Zaleski, DO, St. Joseph Mercy Health System, Ypsilanti, MI, United States; Ann LaFond, MD, St. Joseph Mercy Health System, Ypsilanti, MI, United States; David Blum, MD, St. Joseph Mercy Health System, Ypsilanti, MI, United States; James Ramirez, MD, St. Joseph Mercy Health System, Ypsilanti, MI, United States History and course: A 58-year-old white female presented with a 20-year history of slightly tender nodules on her trunk and bilateral upper and lower extremities. The patient’s medical history was significant for leiomyomata of the uterus, resulting in a hysterectomy in the early 1990’s. Family history was positive for a renal mass of unknown type in the patient’s sister. Laboratory: DNA extract, HLRCC full screen: positive for a heterozygous deletion of a single base at nucleotide 1293 of the fumarate hydratase gene (c. 1293delA). Histopathology: Excisional biopsies taken from the patient’s proximal forearm showed nonencapsulated, circumscribed dermal nodules composed of interlacing fascicles of mature smooth muscle cells. Diagnosis: Reed syndrome.
P8322 Aleukemic leukemia cutis preceding monocytic leukemia: A rare case diagnosed by dermatology Tracey Brown-Maher, MD, Memorial University of Newfoundland, St. John’s, Canada; Roxanna Astani, MD, Memorial University of Newfoundland, St. John’s, Canada
Discussion: Reed syndrome (multiple cutaneous and uterine leiomyomatosis, MCUL) is an autosomal dominant condition characterized by the development of leiomyomata of the uterus in affected women, in addition to leiomyomata of the skin in both sexes. A subset of patients with Reed syndrome are at risk for renal cancer, in particular renal papillary type II cancer and renal collecting duct cancer. This disease variant is known as hereditary leiomyomatosis and renal cell cancer (HLRCC). Clinically, cutaneous leiomyomatosis presents as painful nodules on the trunk and extremities during the second to fourth decade of life. The presence of these lesions is considered the most sensitive and specific clinical marker of HLRCC. The risk of developing renal cancer in HLRCC is relatively low, estimated to be about 2% to 6%. However, it must be noted that when patients do develop renal carcinoma, it is usually metastatic by the time of diagnosis. To diagnose this condition, genetic testing for mutations of the fumarate hydratase (FH) gene must be performed. This gene is found on chromosome 1q 42-43 and encodes a Krebs cycle enzyme, which catalyzes the conversion of fumarate to malate. Management of this condition involves symptomatic treatment of cutaneous lesions and close surveillance and monitoring for the development of renal tumors. Because of the aggressive nature of the renal cancer associated with this disease, initial screening with computed tomography or magnetic resonance imaging, in addition to periodic imaging, should be performed in patients who have tested positive for a FH mutation.
Leukemia cutis is the presence of neoplastic leukocytes/precursors in skin, which may present as macules, papules, plaques, nodules, purpura, or ulcers. Aleukemic leukemia cutis (ALC), the presence of blasts in the skin before the appearance in peripheral blood or bone marrow is extremely rare (7% of leukemia cutis cases). It is associated with poor outcome, with mean survival of 3 to 30 months after cells infiltrate peripheral blood/bone marrow. A 55-year-old male presented with a 2month history of asymptomatic nodules on the trunk. He had symptoms of fever, chills, and night sweats in the 2 weeks prior. On examination, there were 0.5 to 2.0 cm firm, rubbery blue nodules on the back, abdomen, and chest. Leukemia/lymphoma was highly suspected. A punch biopsy showed atypical monocytic infiltrate in keeping with monocytic leukemia cutis, CD4+, CD43+. Laboratory work-up was negative. The patient was referred to hematology for bone marrow biopsy and aspiration and another skin biopsy was performed for flow cytometry. Initial bone marrow was negative. The skin biopsy sent for flow cytometry suggested a leukemic infiltrate of monocytic lineage. Dermatology strongly recommended a repeat bone marrow biopsy (1 week after initial one), although hematology was not convinced of the diagnosis. Repeat biopsy showed a large infiltrate of abnormal cells, marking as blasts. Hematology was now convinced the patient had an acute monocytic leukemia, a subtype of AML, and the patient was admitted for induction and consolidation chemotherapy. He is currently awaiting stem cell transplant from a matched sibling. ALC occurs at least 1 month before the appearance in peripheral blood/bone marrow, and is commonly misdiagnosed. Early treatment is essential for improving morbidity and mortality. Our patient was diagnosed within 1 week and started on treatment within 1 month of presentation to dermatology. This case illustrates the importance of high clinical suspicion for leukemia cutis.
Commercial support: None identified.
Commercial support: None identified.
AB114
J AM ACAD DERMATOL
MAY 2014
P8142
A case of pravastatin-induced DRESS syndrome Spencer Bezalel, University of South Florida Morsani College of Medicine, Tampa, FL, United States; Brooke Baldwin, MD, James A. Haley Veteran’s Hospital, Tampa, FL, United States; Drazen Jukic, MD, James A. Haley Veteran’s Hospital, Tampa, FL, United States; Nishit Patel, MD, University of South Florida Morsani College of Medicine, Tampa, FL, United States
Acral green pigmentation in cholestatic jaundice patient: A case report Daranporn Triwongwaranat, MD, Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Pranee Kasemsarn, MD, Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Waranya Boonchai, MD, Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand Cutaneous manifestations of hepatic diseases are various including jaundice, pruritus, palmar erythema, spider telangiectasia, Terry nails, Muehrcke nails, diffuse hyperpigmentation, and gynecomastia. Acral green pigmentation is a rare cutaneous manifestation found only in patients with hyperbilirubinemia. We report a 56-yearold man with intrahepatic cholangiocarcinoma presented with abrupt onset green macules and patches on both hyperkeratotic palms and soles. Dermatoscopic examination revealed linear distributions of green pigments predominate on dermatoglyphic ridges with furrow sparing. Histopathologic examination from left palm stained with hematoxylineeosin showed hyperkeratosis and pink amorphous material within stratum corneum. Special staining for bilirubin named Fouchet stain or Hall stain was negative. Other causes of green-pigmented lesions including pseudomonas infection and exogenous staining were excluded. Green pigmentation on palms and soles in patients with hyperbilirubinemia is a rare condition, with only 5 reported cases on literatures. Previous articles reported that the green pigmentations are mainly along dermatoglyphic ridges where the eccrines sweat pores open. In addition, all of reported patients have cutaneous distribution only on palms and soles; the most abundant areas of sweat glands. The evidences from clinical findings and dermatoscopic examination support that this condition is the variant of eccrine chromhidrosis. Although special staining for billirubin result was negative, not all previously reported cases showed positive for bile stain. There are many patients with hyperbilirubinemia from various causes, but acral green pigmentation is still rarely noticed. Some hidden factors may exist which will require examination more affected patients to elucidate. We report the first case of acral green pigmentation in cholangiocarcinoma patient.
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a drug-induced hypersensitivity reaction characterized by cutaneous eruption, eosinophilia, and involvement of other organ systems. It is differentiated from other drug reactions by its late onset (3 weeks to 3 months), the involvement of other organs, and the persistence of the reaction despite withdrawal of the inciting medication. The underlying mechanism remains to be elucidated, but abnormal epoxide hydroxylase, reactivation of latent viruses, and genetic predisposition involving specific allelic variants of human leukocyte antigen (HLA) are suggested to be involved. The majority of cases are related to aromatic anticonvulsants. Here we present a case of pravastatin-induced DRESS syndrome. Currently there is only 1 other published case involving a statin (atorvastatin). Keeping in mind a mortality rate between 10% and 20%, identification of all potential offending drugs is of utmost importance. With statins being one of the most highly prescribed medications worldwide, health care providers should be aware of this potentially lethal adverse reaction in a subset of the population. Commercial support: None identified.
Commercial support: None identified.
P7599 A woman with unusual nodules on her trunk and extremities Theresa Zaleski, DO, St. Joseph Mercy Health System, Ypsilanti, MI, United States; Ann LaFond, MD, St. Joseph Mercy Health System, Ypsilanti, MI, United States; David Blum, MD, St. Joseph Mercy Health System, Ypsilanti, MI, United States; James Ramirez, MD, St. Joseph Mercy Health System, Ypsilanti, MI, United States History and course: A 58-year-old white female presented with a 20-year history of slightly tender nodules on her trunk and bilateral upper and lower extremities. The patient’s medical history was significant for leiomyomata of the uterus, resulting in a hysterectomy in the early 1990’s. Family history was positive for a renal mass of unknown type in the patient’s sister. Laboratory: DNA extract, HLRCC full screen: positive for a heterozygous deletion of a single base at nucleotide 1293 of the fumarate hydratase gene (c. 1293delA). Histopathology: Excisional biopsies taken from the patient’s proximal forearm showed nonencapsulated, circumscribed dermal nodules composed of interlacing fascicles of mature smooth muscle cells. Diagnosis: Reed syndrome.
P8322 Aleukemic leukemia cutis preceding monocytic leukemia: A rare case diagnosed by dermatology Tracey Brown-Maher, MD, Memorial University of Newfoundland, St. John’s, Canada; Roxanna Astani, MD, Memorial University of Newfoundland, St. John’s, Canada
Discussion: Reed syndrome (multiple cutaneous and uterine leiomyomatosis, MCUL) is an autosomal dominant condition characterized by the development of leiomyomata of the uterus in affected women, in addition to leiomyomata of the skin in both sexes. A subset of patients with Reed syndrome are at risk for renal cancer, in particular renal papillary type II cancer and renal collecting duct cancer. This disease variant is known as hereditary leiomyomatosis and renal cell cancer (HLRCC). Clinically, cutaneous leiomyomatosis presents as painful nodules on the trunk and extremities during the second to fourth decade of life. The presence of these lesions is considered the most sensitive and specific clinical marker of HLRCC. The risk of developing renal cancer in HLRCC is relatively low, estimated to be about 2% to 6%. However, it must be noted that when patients do develop renal carcinoma, it is usually metastatic by the time of diagnosis. To diagnose this condition, genetic testing for mutations of the fumarate hydratase (FH) gene must be performed. This gene is found on chromosome 1q 42-43 and encodes a Krebs cycle enzyme, which catalyzes the conversion of fumarate to malate. Management of this condition involves symptomatic treatment of cutaneous lesions and close surveillance and monitoring for the development of renal tumors. Because of the aggressive nature of the renal cancer associated with this disease, initial screening with computed tomography or magnetic resonance imaging, in addition to periodic imaging, should be performed in patients who have tested positive for a FH mutation.
Leukemia cutis is the presence of neoplastic leukocytes/precursors in skin, which may present as macules, papules, plaques, nodules, purpura, or ulcers. Aleukemic leukemia cutis (ALC), the presence of blasts in the skin before the appearance in peripheral blood or bone marrow is extremely rare (7% of leukemia cutis cases). It is associated with poor outcome, with mean survival of 3 to 30 months after cells infiltrate peripheral blood/bone marrow. A 55-year-old male presented with a 2month history of asymptomatic nodules on the trunk. He had symptoms of fever, chills, and night sweats in the 2 weeks prior. On examination, there were 0.5 to 2.0 cm firm, rubbery blue nodules on the back, abdomen, and chest. Leukemia/lymphoma was highly suspected. A punch biopsy showed atypical monocytic infiltrate in keeping with monocytic leukemia cutis, CD4+, CD43+. Laboratory work-up was negative. The patient was referred to hematology for bone marrow biopsy and aspiration and another skin biopsy was performed for flow cytometry. Initial bone marrow was negative. The skin biopsy sent for flow cytometry suggested a leukemic infiltrate of monocytic lineage. Dermatology strongly recommended a repeat bone marrow biopsy (1 week after initial one), although hematology was not convinced of the diagnosis. Repeat biopsy showed a large infiltrate of abnormal cells, marking as blasts. Hematology was now convinced the patient had an acute monocytic leukemia, a subtype of AML, and the patient was admitted for induction and consolidation chemotherapy. He is currently awaiting stem cell transplant from a matched sibling. ALC occurs at least 1 month before the appearance in peripheral blood/bone marrow, and is commonly misdiagnosed. Early treatment is essential for improving morbidity and mortality. Our patient was diagnosed within 1 week and started on treatment within 1 month of presentation to dermatology. This case illustrates the importance of high clinical suspicion for leukemia cutis.
Commercial support: None identified.
Commercial support: None identified.
AB114
J AM ACAD DERMATOL
MAY 2014