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Mucoceles that weren't—Two case reports
P1102
P1104
Cutaneous sinus histiocytosis (RosaieDorfman disease) presenting histologicly as subcutaneous panniculitis-like T-cell lymphoma versus lupus panniculitis (lupus erythematosus profundus) Regina Ramon, MD, PhD, Hospital General Alicante, Alicante, Spain; Irene Ballester, Hospital General Alicante, Alicante, Spain; Isabel Betlloch, Hospital General Alicante, Alicante, Spain; Jaime Guijarro, Hospital General Alicante, Alicante, Spain; Maria Perez, Hospital General Alicante, Alicante, Spain A 51-year-old female presented to our clinic with a 2-month history of a large, unique, asymptomatic nodule located on her right abdomen. She was otherwise healthy, with no history of fever, malaise, or weight loss. Biopsy specimens from the lesion were suggestive of subcutaneous panniculitis-like T-cell lymphoma versus lupus panniculitis (lupus erythematosus profundus), including lobular panniculitis with small fat lobules and thickened septa, with abundant mucin between collagen bundles of the reticular dermis. The dense infiltrate was mainly composed of small to medium atypical lymphocytes, sometimes showing an angiocentric distribution and forming rings around the adipose tissue lobules, plasma cells among necrotic adipocytes, and sclerotic collagen bundles in the thickened septa. No clonality was documented by T-cell receptor gene rearrangement studies. Routine blood investigations showed an elevated erythrocyte sedimentation rate and mild leukocytosis. Results of autoimmune screening for lupus erythematosus were negative. There was no lymphadenopathy or hepatosplenomegaly. With the histologic diagnostic doubt and because the nodule was quickly growing the lesion was fully excised. The histopathologic findings of the full lesion were strongly suggestive of cutaneous sinus histiocytosis (RosaieDorfman disease): pale-staining, large, polygonal histiocytes admixed with plasma cells, neutrophils, many lymphocytes, and occasional Touton giant cells. Emperipolesis of plasma cells with the typical halo were also seen.
Tuberculoid leprosy: An immune reconstitution inflammatory syndrome in an HIV-positive patient Carla Oliveira, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil; Bruno Aquino, MD, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil; Omar Lupi, PhD, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil; Ricardo Lima, MD, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil Background: The introduction of highly active antiretroviral therapy (HAART) has led to the emergence of a new clinical syndrome known as immune reconstitution inflammatory syndrome (IRIS). This syndrome has been described in association with many diseases; however, only recently has IRIS has been associated with leprosy. A paradox is that HIV coinfection does not affect the tuberculoidelepromatous clinical spectrum of leprosy. These patients might have more severe leprosy neuritis and an increased frequency of immune mediated reversal reaction (type I).
Commercial support: None identified.
Case report: A 31-year-old HIV-positive male from Nova Iguac¸u who has been on HAART for 18 months related a lesion on right forearm for about 8 months. Clinical examination revealed a single erythematous infiltrate plaque, measuring 2 cm, painless, and with a thick nerve emerging on it. This lesion had amendment of thermal sensitivity. A skin biopsy was performed and showed a granulomatosis inflammatory tuberculoid pattern, BAAR negative, confirming a tuberculoid leprosy with reversal reaction type1 diagnosis. The patient started prednisone 60 mg/day and a paucibacillary scheme therapy with rifampicin and dapsone. After 9 months of the beginning of HAART, the pacient developed several erythematous plaque with neuritis on the right forearm. Discussion: The temporal association between the development of these lesions and CD41 lymphocytopenia observed during HAART strongly suggests that this apresentation of leprosy was a result from immune reconstitution. Interations between HIV infection and leprosy in use of HAART may lead to increased cellmediated immunity to Mycobacterium leprae, triggering reversal reactions and making control of the disease a hard task to achieve. Although this case represents a pacient with a borderline tuberculoid leprosy, based on the Ridley and Jopling classification, a paucibacillary scheme was started according to the operational division of World Health Organization that classifies up to five lesions as a paucibacillary form. Systemic corticosteroids are indicated in severe leprosy reaction with neuritis and were prescribed in the present case. Commercial support: None identified.
P1103 Mucoceles that weren’t—Two case reports Kristyna Lee, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Daniel M. Siegel, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Laura Cepeda, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States
P1105
Discussion: Oral cysticercosis and salivary gland adenocarcinoma are rare diseases of the oral mucosa. It is important to consider these and other uncommon diagnoses when evaluating an oral nodule presumed to be a mucocele. We discuss the natural history and pathogenesis of mucoceles and these disease processes.
Purpura annularis telangiectodes of majocchi: Two case reports of this uncommon dermatoses Kristyna Lee, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Usha Alapati, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States At least five subtypes of pigmented purpuric dermatoses have been described. Purpura annularis telangiectodes of Majocchi is an uncommon pigmented purpuric eruption characterized by symmetrical, purpuric, telangiectatic, and atrophic patches with a predilection for the lower extremities and buttocks. We present two cases of this uncommon dermatoses and discuss the natural history and pathogenesis of this and other types of purpura. The first case is an 83-year-old male who presented complaining of a 1-week history of nonpruritic red spots on his lower extremities. The second case is a 79-year-old male who presented with a 2-month history of a pruritic patchy rash along his waistline. Both patients had no significant medical history. Both patients denied any history of trauma, preceding illness, prodromal symptoms, new medication use, or easy bruisability. Their reviews of symptoms were both noncontributory. In patient 1, there were multiple discrete annular and arcuate purpuric and erythematous patches extensively distributed along his bilateral proximal pretibial and posterior calves and ankles. In patient 2, there were multiple discrete annular purpuric and erythematous patches along the lower abdomen and posterior waistline. The patches in both patients were nonblanchable. Laboratory evaluation including antinuclear antibody titer, coagulation panel, and complete blood cell count with differential revealed no abnormalities in both patients. Given the lack of constitutional symptoms in either of these otherwise healthy patients with physical examinations unremarkable outside of classic annular purpura and unremarkable laboratory evaluations, the diagnoses of coagulation disorders, vasculitis, thrombocytopenic states, or purpuras secondary to acute infections were unlikely. Histopathologic examination for both patients revealed a superficial perivascular and interstitial infiltrate of lymphocytes admixed with red blood cells and mild edema in the papillary dermis. The diagnosis of purpura annularis telangiectodes of Majocchi was made for both patients. Supportive therapy was offered and all lesions in both patients gradually resolved with residual postinflammatory hyperpigmentation.
Commercial support: None identified.
Commercial support: None identified.
Background: A mucocele is one of the more commonly encountered disorders of the oral mucosa. It develops when a minor salivary duct is injured or blocked, resulting in the escape of mucus into the adjacent submucosal connective tissue. We present two cases that were initially diagnosed as mucoceles. Histologic examination, however, revealed dissimilar and medically significant pathologies. Case 1: A healthy 38-year-old male was referred for multiple intraoral nodules. They were firm, painless, and not related to any traumatic episode. They had been present for 1 year after traveling to Puerto Rico and 6 months after traveling to Costa Rica. A large solitary nodule arose first, followed by three smaller nodules 6 months later that developed around the primary lesion. The patient had no headaches, seizures, or any neurologic deficits. On examination, all nodules were smooth and well defined with intact overlying mucosa. An 8- 3 6-mm nodule and three surrounding 3- 3 3-mm nodules were seen on the lower labial mucosa. Clinically, the nodules resembled mucoceles. Histopathologic examination of the largest lesion revealed a cystic space containing a cysticercus larva. The diagnosis of oral cysticercosis was made. Magnetic resonance imaging scans showed no evidence of cysticerci in the brain or eye. An stool examination revealed no ova, cysts, or parasites. The lesions resolved after an oral course of albendazole 400 mg twice daily. Case 2: A 71-year-old female with a medical history significant for a 30-pack per year cigarette smoking habit was referred for a slowly enlarging intraoral lump. This was painless and had been present for 5 years. Examination revealed a solitary 3- 3 3-mm freely mobile, smooth, firm nodule on the labial mucosa of the upper lip. The clinical diagnosis was a mucocele. Histopathologic examination revealed salivary gland polymorphous low-grade adenocarcinoma. Mohs micrographic surgery was performed and no residual carcinoma was identified in the Mohs stage. On follow-up, she is doing well without evidence of further disease.
AB40
J AM ACAD DERMATOL
MARCH 2009
P1104
Cutaneous sinus histiocytosis (RosaieDorfman disease) presenting histologicly as subcutaneous panniculitis-like T-cell lymphoma versus lupus panniculitis (lupus erythematosus profundus) Regina Ramon, MD, PhD, Hospital General Alicante, Alicante, Spain; Irene Ballester, Hospital General Alicante, Alicante, Spain; Isabel Betlloch, Hospital General Alicante, Alicante, Spain; Jaime Guijarro, Hospital General Alicante, Alicante, Spain; Maria Perez, Hospital General Alicante, Alicante, Spain A 51-year-old female presented to our clinic with a 2-month history of a large, unique, asymptomatic nodule located on her right abdomen. She was otherwise healthy, with no history of fever, malaise, or weight loss. Biopsy specimens from the lesion were suggestive of subcutaneous panniculitis-like T-cell lymphoma versus lupus panniculitis (lupus erythematosus profundus), including lobular panniculitis with small fat lobules and thickened septa, with abundant mucin between collagen bundles of the reticular dermis. The dense infiltrate was mainly composed of small to medium atypical lymphocytes, sometimes showing an angiocentric distribution and forming rings around the adipose tissue lobules, plasma cells among necrotic adipocytes, and sclerotic collagen bundles in the thickened septa. No clonality was documented by T-cell receptor gene rearrangement studies. Routine blood investigations showed an elevated erythrocyte sedimentation rate and mild leukocytosis. Results of autoimmune screening for lupus erythematosus were negative. There was no lymphadenopathy or hepatosplenomegaly. With the histologic diagnostic doubt and because the nodule was quickly growing the lesion was fully excised. The histopathologic findings of the full lesion were strongly suggestive of cutaneous sinus histiocytosis (RosaieDorfman disease): pale-staining, large, polygonal histiocytes admixed with plasma cells, neutrophils, many lymphocytes, and occasional Touton giant cells. Emperipolesis of plasma cells with the typical halo were also seen.
Tuberculoid leprosy: An immune reconstitution inflammatory syndrome in an HIV-positive patient Carla Oliveira, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil; Bruno Aquino, MD, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil; Omar Lupi, PhD, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil; Ricardo Lima, MD, Unirio Universidade Federal Do Estado Do Rio De Janeiro, Rio De Janeiro, Brazil Background: The introduction of highly active antiretroviral therapy (HAART) has led to the emergence of a new clinical syndrome known as immune reconstitution inflammatory syndrome (IRIS). This syndrome has been described in association with many diseases; however, only recently has IRIS has been associated with leprosy. A paradox is that HIV coinfection does not affect the tuberculoidelepromatous clinical spectrum of leprosy. These patients might have more severe leprosy neuritis and an increased frequency of immune mediated reversal reaction (type I).
Commercial support: None identified.
Case report: A 31-year-old HIV-positive male from Nova Iguac¸u who has been on HAART for 18 months related a lesion on right forearm for about 8 months. Clinical examination revealed a single erythematous infiltrate plaque, measuring 2 cm, painless, and with a thick nerve emerging on it. This lesion had amendment of thermal sensitivity. A skin biopsy was performed and showed a granulomatosis inflammatory tuberculoid pattern, BAAR negative, confirming a tuberculoid leprosy with reversal reaction type1 diagnosis. The patient started prednisone 60 mg/day and a paucibacillary scheme therapy with rifampicin and dapsone. After 9 months of the beginning of HAART, the pacient developed several erythematous plaque with neuritis on the right forearm. Discussion: The temporal association between the development of these lesions and CD41 lymphocytopenia observed during HAART strongly suggests that this apresentation of leprosy was a result from immune reconstitution. Interations between HIV infection and leprosy in use of HAART may lead to increased cellmediated immunity to Mycobacterium leprae, triggering reversal reactions and making control of the disease a hard task to achieve. Although this case represents a pacient with a borderline tuberculoid leprosy, based on the Ridley and Jopling classification, a paucibacillary scheme was started according to the operational division of World Health Organization that classifies up to five lesions as a paucibacillary form. Systemic corticosteroids are indicated in severe leprosy reaction with neuritis and were prescribed in the present case. Commercial support: None identified.
P1103 Mucoceles that weren’t—Two case reports Kristyna Lee, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Daniel M. Siegel, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Laura Cepeda, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States
P1105
Discussion: Oral cysticercosis and salivary gland adenocarcinoma are rare diseases of the oral mucosa. It is important to consider these and other uncommon diagnoses when evaluating an oral nodule presumed to be a mucocele. We discuss the natural history and pathogenesis of mucoceles and these disease processes.
Purpura annularis telangiectodes of majocchi: Two case reports of this uncommon dermatoses Kristyna Lee, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Usha Alapati, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States At least five subtypes of pigmented purpuric dermatoses have been described. Purpura annularis telangiectodes of Majocchi is an uncommon pigmented purpuric eruption characterized by symmetrical, purpuric, telangiectatic, and atrophic patches with a predilection for the lower extremities and buttocks. We present two cases of this uncommon dermatoses and discuss the natural history and pathogenesis of this and other types of purpura. The first case is an 83-year-old male who presented complaining of a 1-week history of nonpruritic red spots on his lower extremities. The second case is a 79-year-old male who presented with a 2-month history of a pruritic patchy rash along his waistline. Both patients had no significant medical history. Both patients denied any history of trauma, preceding illness, prodromal symptoms, new medication use, or easy bruisability. Their reviews of symptoms were both noncontributory. In patient 1, there were multiple discrete annular and arcuate purpuric and erythematous patches extensively distributed along his bilateral proximal pretibial and posterior calves and ankles. In patient 2, there were multiple discrete annular purpuric and erythematous patches along the lower abdomen and posterior waistline. The patches in both patients were nonblanchable. Laboratory evaluation including antinuclear antibody titer, coagulation panel, and complete blood cell count with differential revealed no abnormalities in both patients. Given the lack of constitutional symptoms in either of these otherwise healthy patients with physical examinations unremarkable outside of classic annular purpura and unremarkable laboratory evaluations, the diagnoses of coagulation disorders, vasculitis, thrombocytopenic states, or purpuras secondary to acute infections were unlikely. Histopathologic examination for both patients revealed a superficial perivascular and interstitial infiltrate of lymphocytes admixed with red blood cells and mild edema in the papillary dermis. The diagnosis of purpura annularis telangiectodes of Majocchi was made for both patients. Supportive therapy was offered and all lesions in both patients gradually resolved with residual postinflammatory hyperpigmentation.
Commercial support: None identified.
Commercial support: None identified.
Background: A mucocele is one of the more commonly encountered disorders of the oral mucosa. It develops when a minor salivary duct is injured or blocked, resulting in the escape of mucus into the adjacent submucosal connective tissue. We present two cases that were initially diagnosed as mucoceles. Histologic examination, however, revealed dissimilar and medically significant pathologies. Case 1: A healthy 38-year-old male was referred for multiple intraoral nodules. They were firm, painless, and not related to any traumatic episode. They had been present for 1 year after traveling to Puerto Rico and 6 months after traveling to Costa Rica. A large solitary nodule arose first, followed by three smaller nodules 6 months later that developed around the primary lesion. The patient had no headaches, seizures, or any neurologic deficits. On examination, all nodules were smooth and well defined with intact overlying mucosa. An 8- 3 6-mm nodule and three surrounding 3- 3 3-mm nodules were seen on the lower labial mucosa. Clinically, the nodules resembled mucoceles. Histopathologic examination of the largest lesion revealed a cystic space containing a cysticercus larva. The diagnosis of oral cysticercosis was made. Magnetic resonance imaging scans showed no evidence of cysticerci in the brain or eye. An stool examination revealed no ova, cysts, or parasites. The lesions resolved after an oral course of albendazole 400 mg twice daily. Case 2: A 71-year-old female with a medical history significant for a 30-pack per year cigarette smoking habit was referred for a slowly enlarging intraoral lump. This was painless and had been present for 5 years. Examination revealed a solitary 3- 3 3-mm freely mobile, smooth, firm nodule on the labial mucosa of the upper lip. The clinical diagnosis was a mucocele. Histopathologic examination revealed salivary gland polymorphous low-grade adenocarcinoma. Mohs micrographic surgery was performed and no residual carcinoma was identified in the Mohs stage. On follow-up, she is doing well without evidence of further disease.
AB40
J AM ACAD DERMATOL
MARCH 2009