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Neutrophilic fixed drug eruption
P533
P535
Waldenstrom’s macroglobulinemia presenting with purpuric patches and leg ulcers Michael Conroy, MD, MS, Mayo Clinic Scottsdale, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic Scottsdale, Scottsdale, AZ, United States; Karen Warschaw, MD, Mayo Clinic Scottsdale, Scottsdale, AZ, United States
Evaluation of potential for tachyphylaxis to topically applied dandruff and seborrheic dermatitis treatments based on pyrithione zin James Schwartz, PhD, Procter & Gamble, Cincinnati, OH, United States; Heather Rocchetta, PhD, Procter & Gamble, Cincinnati, OH, United States; Fangyi Luo, PhD, Procter & Gamble, Cincinnati, OH, United States
History & physical: 84-year-old female with a 2-month history of non-healing ulcers on the anterior lower legs. The patient denied a history of trauma. The ulcers began as spontaneous ‘‘bruises’’ which subsequently broke down.
Background: Dandruff and seborrheic dermatitis of the scalp are conditions that affect nearly half of the population. Symptoms include flaking and itching, which can lead to quality of life issues, such as frustration and embarrassment. The etiology includes causative factors associated with the commensal organism Malassezia, sebaceous lipids and human response factors. Most common forms of treatment rely on anti-fungal materials to reduce the population of Malassezia and allow the scalp skin to normalize. Once the condition is brought under control, patients must continue to utilize the anti-fungal product to prevent the Malassezia population from returning. Thus, effective treatment requires long term prophylactic product usage. There is a widespread belief that tachyphylaxis (accommodation to treatment leading to decreasing benefit) commonly occurs in dandruff treatment and that patients must switch between treatment types (ie, products containing different active ingredients) to maintain efficacy.
Past medical history: Lymphoma of unknown type, for which she had received no treatment. Physical exam: Left and right anterior lower legs showed 6-cm purpuric patches with central 2-cm shallow ulcers and overlying black eschar. Pathology/laboratory studies: Punch biopsy revealed a hyalinizing non-inflammatory vasculopathy. The superficial and deep dermal vessels showed total occlusion with homogeneous eosinophilic material. Labs: Peripheral blood smear showed marked RBC agglutination and clumped proteinaceous material. Cryoglobulins were identified in the peripheral blood. Serum protein electrophoresis revealed a monoclonal IgM kappa paraprotein and polyclonal IgG. Urinalysis showed large numbers of RBCs. Course: Oncology records revealed a prior diagnosis of IgM monoclonal gammopathy with lymphoma and Waldenstrom’s macroglobulinemia (WM). There had been an increase in viscosity and IgM level in the preceding 9 months. Patient was treated with chlorambucil and prednisone and exhibited a significant drop in her IgM level after 1 dose. Discussion: WM is a rare lymphoproliferative disorder, in which IgM-producing lymphoplasmacytic cells proliferate in the bone marrow, lymph nodes or spleen. The majority of signs and symptoms are secondary to the increased viscosity of the blood caused by the high levels of IgM protein. CNS manifestations include dizziness, confusion, and stroke. Renal and cardiac function may also be compromised. Cutaneous findings most commonly show purpura and leg ulceration. Pathology typically shows a non-inflammatory vasculopathy. Asymptomatic patients may not require specific treatment. If symptomatic, multiple regimens may be employed such as chlorambucil and prednisone, cladribine monotherapy, or rituximab with chemotherapy. Although our patient’s only symptoms were leg ulcers and purpura, these cutaneous findings may be an important clue to severe hyperviscosity. Recognition of hyperviscosity is critical because of the risk of catastrophic stroke and other complications.
Objective: To assess whether tachyphylaxis really occurs in treatment of dandruff and seborrheic dermatitis when treatment is conducted with a pyrithione zinc-based anti-fungal shampoo. Methods: Two long-term placebo-controlled clinical evaluations (6-month and 12-month) were conducted using dermatologist-assessed flaking as end points. Statistical analyses were conducted on mean population difference from placebo control as well as individual panelist responses over time to evaluate the proportion of individuals that experience an early benefit but do not maintain it through the end of the study. Results: Evaluation of the mean population responses demonstrate statistically significant differences between treatment and placebo (treatment more effective) across all time points for both studies. This data suggests that, on average, tachyphylaxis is not an issue for pyrithione zinc-based anti-dandruff shampoos. Data will also be presented utilizing the evaluation of individual responses as a function of treatment time. Other explanations will be considered to explain the perception amongst clinicians that treatment efficacy decreases over time. 100% is sponsored by Procter & Gamble.
Commercial support: None identified.
P534 Neutrophilic fixed drug eruption Temitope Soares, PharmD, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States; Karen Warschaw, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States We present a 71-year-old white female with a recurrent eruption on the right hip. The night before presentation, the patient had a low grade fever and took naproxen. The following morning, she developed a rash on the right hip/buttock. This rash has occurred intermittently 6 times over the past 3 years in the exact same location. The rash eventually resolves over 10 to 14 days and is totally absent between episodes. She describes an association with low-grade fever, joint aches, and malaise. Past medical history is significant for hypothyroidism, degenerative arthritis leading to hip replacement, and impaired glucose tolerance. Patient takes levothyroxine, multivitamins, and aspirin daily, and naproxen intermittently as needed for aches and pains. She denies taking any other medication. On the right hip extending to the buttock is a 7 cm 3 8 cm, fairly well-marginated, bright pink to red plaque with no surface epidermal changes. A biopsy showed marked papillary dermal edema. In the dermis there was a diffuse inflammatory infiltrate composed predominantly of neutrophils, epidermal exocytosis of neutrophils, and no vasculitis. The patient had a previous biopsy with similar histology. The differential diagnosis in this case includes the neutrophillic dermatoses, with drug-induced Sweet’s syndrome being the most apparent initially. While she did report low-grade fevers associated with her lesion, the recurrent nature of the lesion at the same precise site makes a fixed drug eruption more likely, despite the histopathological picture. A review of her medications reveals that the only intermittent medication is naproxen. There have been 2 previous reports of neutrophilic fixed drug eruption. Agnew and Oliver reported a case involving amoxicillin-clavulanic acid in 2000, and Ozkaya et al reported a case involving naproxen in 2005. In both cases, the patients were afebrile, and the rash was reproduced upon re-challenge. She has been advised to avoid naproxen in the future. We present this case to further stimulate discussion on whether the neutrophilic fixed drug eruption is indeed a distinct entity. Commercial support: None identified.
FEBRUARY 2007
P536 Maffucci’s syndrome Cecilia Laguna, MD, General Universitary Hospital of Valencia, Valencia, Spain; Blanca Martin, MD, General Hospital of Valencia, Valencia, Spain; Gerard Pitarch, MD, General Hospital of Valencia, Valencia, Spain; Juan Vilata, MD, General Hospital of Valencia, Valencia, Spain Maffucci’s syndrome is a non-hereditary congenital mesodermal hyperplasia characterized by the coexistence of multiple enchondromas (possibility of malignant transformation in 20-100% of cases), soft tissue hemangiomas and other mesenchymatous injuries. We describe the case of a 53-year-old male who presented with tumoral lesions on his left hand and forearm with bony appearance, and mobile bluish-coloured nodules. The patient had presented similar lesions since adolescence and some of then had been removed. Radiologic study revealed enchondromas without malignant transformation signs and histologic examination of a cutaneous nodule with vascular appearance showed a cavernous hemangioma. The gammagraphy showed multiple hypercaptant focus and angiography detected subcutaneous fistules affecting left extremities. The syndrome was first coined by Maffucci in 1881. The etiology is unknown and the patients are generally normal at birth. The skeletal changes often affects metacarpals, hand phalanges, and bones of the feet. Hemangiomas may be situated in the brain, eyes, and gastrointestinal tract. Sarcomatous transformation is seen in bone and soft tissue lesions, but the risk is more often in enchondromas. Pathologic fractures are commonly seen, with an incidence rate of 26%. Other complications include hemorrhages from hemangiomas or shortness of stature. Commercial support: None identified.
J AM ACAD DERMATOL
AB49
P535
Waldenstrom’s macroglobulinemia presenting with purpuric patches and leg ulcers Michael Conroy, MD, MS, Mayo Clinic Scottsdale, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic Scottsdale, Scottsdale, AZ, United States; Karen Warschaw, MD, Mayo Clinic Scottsdale, Scottsdale, AZ, United States
Evaluation of potential for tachyphylaxis to topically applied dandruff and seborrheic dermatitis treatments based on pyrithione zin James Schwartz, PhD, Procter & Gamble, Cincinnati, OH, United States; Heather Rocchetta, PhD, Procter & Gamble, Cincinnati, OH, United States; Fangyi Luo, PhD, Procter & Gamble, Cincinnati, OH, United States
History & physical: 84-year-old female with a 2-month history of non-healing ulcers on the anterior lower legs. The patient denied a history of trauma. The ulcers began as spontaneous ‘‘bruises’’ which subsequently broke down.
Background: Dandruff and seborrheic dermatitis of the scalp are conditions that affect nearly half of the population. Symptoms include flaking and itching, which can lead to quality of life issues, such as frustration and embarrassment. The etiology includes causative factors associated with the commensal organism Malassezia, sebaceous lipids and human response factors. Most common forms of treatment rely on anti-fungal materials to reduce the population of Malassezia and allow the scalp skin to normalize. Once the condition is brought under control, patients must continue to utilize the anti-fungal product to prevent the Malassezia population from returning. Thus, effective treatment requires long term prophylactic product usage. There is a widespread belief that tachyphylaxis (accommodation to treatment leading to decreasing benefit) commonly occurs in dandruff treatment and that patients must switch between treatment types (ie, products containing different active ingredients) to maintain efficacy.
Past medical history: Lymphoma of unknown type, for which she had received no treatment. Physical exam: Left and right anterior lower legs showed 6-cm purpuric patches with central 2-cm shallow ulcers and overlying black eschar. Pathology/laboratory studies: Punch biopsy revealed a hyalinizing non-inflammatory vasculopathy. The superficial and deep dermal vessels showed total occlusion with homogeneous eosinophilic material. Labs: Peripheral blood smear showed marked RBC agglutination and clumped proteinaceous material. Cryoglobulins were identified in the peripheral blood. Serum protein electrophoresis revealed a monoclonal IgM kappa paraprotein and polyclonal IgG. Urinalysis showed large numbers of RBCs. Course: Oncology records revealed a prior diagnosis of IgM monoclonal gammopathy with lymphoma and Waldenstrom’s macroglobulinemia (WM). There had been an increase in viscosity and IgM level in the preceding 9 months. Patient was treated with chlorambucil and prednisone and exhibited a significant drop in her IgM level after 1 dose. Discussion: WM is a rare lymphoproliferative disorder, in which IgM-producing lymphoplasmacytic cells proliferate in the bone marrow, lymph nodes or spleen. The majority of signs and symptoms are secondary to the increased viscosity of the blood caused by the high levels of IgM protein. CNS manifestations include dizziness, confusion, and stroke. Renal and cardiac function may also be compromised. Cutaneous findings most commonly show purpura and leg ulceration. Pathology typically shows a non-inflammatory vasculopathy. Asymptomatic patients may not require specific treatment. If symptomatic, multiple regimens may be employed such as chlorambucil and prednisone, cladribine monotherapy, or rituximab with chemotherapy. Although our patient’s only symptoms were leg ulcers and purpura, these cutaneous findings may be an important clue to severe hyperviscosity. Recognition of hyperviscosity is critical because of the risk of catastrophic stroke and other complications.
Objective: To assess whether tachyphylaxis really occurs in treatment of dandruff and seborrheic dermatitis when treatment is conducted with a pyrithione zinc-based anti-fungal shampoo. Methods: Two long-term placebo-controlled clinical evaluations (6-month and 12-month) were conducted using dermatologist-assessed flaking as end points. Statistical analyses were conducted on mean population difference from placebo control as well as individual panelist responses over time to evaluate the proportion of individuals that experience an early benefit but do not maintain it through the end of the study. Results: Evaluation of the mean population responses demonstrate statistically significant differences between treatment and placebo (treatment more effective) across all time points for both studies. This data suggests that, on average, tachyphylaxis is not an issue for pyrithione zinc-based anti-dandruff shampoos. Data will also be presented utilizing the evaluation of individual responses as a function of treatment time. Other explanations will be considered to explain the perception amongst clinicians that treatment efficacy decreases over time. 100% is sponsored by Procter & Gamble.
Commercial support: None identified.
P534 Neutrophilic fixed drug eruption Temitope Soares, PharmD, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States; Karen Warschaw, MD, Mayo Clinic Arizona, Scottsdale, AZ, United States We present a 71-year-old white female with a recurrent eruption on the right hip. The night before presentation, the patient had a low grade fever and took naproxen. The following morning, she developed a rash on the right hip/buttock. This rash has occurred intermittently 6 times over the past 3 years in the exact same location. The rash eventually resolves over 10 to 14 days and is totally absent between episodes. She describes an association with low-grade fever, joint aches, and malaise. Past medical history is significant for hypothyroidism, degenerative arthritis leading to hip replacement, and impaired glucose tolerance. Patient takes levothyroxine, multivitamins, and aspirin daily, and naproxen intermittently as needed for aches and pains. She denies taking any other medication. On the right hip extending to the buttock is a 7 cm 3 8 cm, fairly well-marginated, bright pink to red plaque with no surface epidermal changes. A biopsy showed marked papillary dermal edema. In the dermis there was a diffuse inflammatory infiltrate composed predominantly of neutrophils, epidermal exocytosis of neutrophils, and no vasculitis. The patient had a previous biopsy with similar histology. The differential diagnosis in this case includes the neutrophillic dermatoses, with drug-induced Sweet’s syndrome being the most apparent initially. While she did report low-grade fevers associated with her lesion, the recurrent nature of the lesion at the same precise site makes a fixed drug eruption more likely, despite the histopathological picture. A review of her medications reveals that the only intermittent medication is naproxen. There have been 2 previous reports of neutrophilic fixed drug eruption. Agnew and Oliver reported a case involving amoxicillin-clavulanic acid in 2000, and Ozkaya et al reported a case involving naproxen in 2005. In both cases, the patients were afebrile, and the rash was reproduced upon re-challenge. She has been advised to avoid naproxen in the future. We present this case to further stimulate discussion on whether the neutrophilic fixed drug eruption is indeed a distinct entity. Commercial support: None identified.
FEBRUARY 2007
P536 Maffucci’s syndrome Cecilia Laguna, MD, General Universitary Hospital of Valencia, Valencia, Spain; Blanca Martin, MD, General Hospital of Valencia, Valencia, Spain; Gerard Pitarch, MD, General Hospital of Valencia, Valencia, Spain; Juan Vilata, MD, General Hospital of Valencia, Valencia, Spain Maffucci’s syndrome is a non-hereditary congenital mesodermal hyperplasia characterized by the coexistence of multiple enchondromas (possibility of malignant transformation in 20-100% of cases), soft tissue hemangiomas and other mesenchymatous injuries. We describe the case of a 53-year-old male who presented with tumoral lesions on his left hand and forearm with bony appearance, and mobile bluish-coloured nodules. The patient had presented similar lesions since adolescence and some of then had been removed. Radiologic study revealed enchondromas without malignant transformation signs and histologic examination of a cutaneous nodule with vascular appearance showed a cavernous hemangioma. The gammagraphy showed multiple hypercaptant focus and angiography detected subcutaneous fistules affecting left extremities. The syndrome was first coined by Maffucci in 1881. The etiology is unknown and the patients are generally normal at birth. The skeletal changes often affects metacarpals, hand phalanges, and bones of the feet. Hemangiomas may be situated in the brain, eyes, and gastrointestinal tract. Sarcomatous transformation is seen in bone and soft tissue lesions, but the risk is more often in enchondromas. Pathologic fractures are commonly seen, with an incidence rate of 26%. Other complications include hemorrhages from hemangiomas or shortness of stature. Commercial support: None identified.
J AM ACAD DERMATOL
AB49