- Email: [email protected]
Multiple cutaneous leiomyoma
P513
P515
The perplexing progression of paraneoplastic pemphigus in a patient with CLL Thomas Busick, MD, Scott & White Hospital, Texas A&M Health Science Center, Temple, TX, United States; Daniel Bennett, MD, Scott & White Hospital, Texas A&M Health Science Center, Temple, TX, United States; Ronald Grimwood, MD, Scott & White Hospital, Texas A&M Health Science Center, Temple, TX, United States Background: Paraneoplastic pemphigus is a bullous disorder most commonly associated with a lymphoproliferative cancer; most commonly non-Hodgkins lymphoma (NHL), chronic lymphocytic leukemia (CLL), or a Castleman’s tumor. The classic presentation includes severe mucosal and genital erosions, a polymorpous pruritic eruption on the trunk with bullae, and often severe palmar/plantar ulcerations. Skin findings are often not correlated with the clinical stage of the malignancy. Diagnosis is made with a combination of biopsy, direct immunofluorescence (DIF), and indirect immunofluorescence (IIF) on rat bladder. Case report: We present the history and a photographic series of a 55-year-old white male with CLL and an indolent presentation of paraneoplastic pemphigus. His rash evolved through multiple stages clinically, histologically, and by immunofluorescence over a 5-month period. Initial presentation was clinically and histologically most consistent with a lichenoid drug eruption. After failing prednisone therapy, the patient returned erythrodermic with islands of sparing and was thought to have pityriasis rubra pilaris, but a repeat biopsy was unchanged. Next, he developed oral, periocular, and genital erosions to accompany the erythroderma. Paraneoplastic pemphigus was suspected, but DIF was initially negative. Several days later, a repeat DIF demonstrated a pemphigus pattern, but IIF was negative. One month later, the IIF became positive only on rat bladder, and the patient had developed impressive ulcerations of his fingertips with shedding of several nails. Throughout the course of his rash, the patient’s CLL was well controlled. His paraneoplastic pemphigus was improving on cyclosporine at time of submission. Comment: Although this patient’s final eruption fit the classic definition of paraneoplastic pemphigus, his rash took several months to evolve into that state. The biopsies and immunofluorescence studies followed a similar evolution. Our case illustrates the difficulty in diagnosing this rare entity as well as the importance of close follow up and reevaluation of the difficult patient.
Multiple cutaneous leiomyoma Mouna Jamali, MD, UHC, Ibn Rochd, Casablanca, MO, Morocco; Khadija Khadir, PhD, UHC, Ibn Rochd, Casablanca, MO, Morocco; Sofia Azzouzi, PhD, UHC, Ibn Rochd, Casablanca, MO, Morocco; Hakima Benchikhi, PhD, UHC, Ibn Rochd, Casablanca, MO, Morocco Cutaneous leiomyomas are rare benign, smooth muscle tumors characterized by painful nodules. They can be classified according to the derivation, as piloleiomyomas, angioleiomyomas, and genital leiomyomas. We describe a patient who has multiple cutaneous leiomyomas. A 44-year-old woman was referred for multiple lesions of the trunk. They began as small reddish-brown papules 10 years earlier and had slowly increased in size and in number over this period. There was no history of trauma, and no familial similar lesions. The lesions became recently painful, spontaneously or when palpated. Physical examination revealed multiple, painful, firm, non-tender, red nodules and papules, 2 cm to 3 cm in size on her trunk and 3 mm to 6 mm on her right upper arm. She had no other lesions elsewhere. Histopathologic examination of a nodule provided the diagnosis of cutaneous leiomyoma. An ultrasound scanning of pelvis had not revealed others lesions as uterine leiomyoma. Surgical removal was performed for only the bigger and painful lesions of the trunk. Our patient has multiple cutaneous leiomyoma. They are benign tumour that derived from the erector pili muscle. They arise in young adults as multiple, firm dermal papules and sometimes nodules that are classically pink or red in color. They can be painful when palpated, exposed to a cold environment, or as a result of emotional stress. The cause of the pain was initially proposed to be caused by the leiomyoma pressing on the cutaneous nerves. However, others have felt it to be more likely secondary to contraction of the muscle fibres making up the tumor itself. There have been several reports of the familial form of cutaneous leiomyomia and it is now thought to be an autosomal dominantly inherited disorder with incomplete penetrance. The association of cutaneous and uterine leiomyomia is not rare; this why all female patients with cutaneous lesions should have a pelvic ultrasound scan. The treatment of cutaneous leiomyoma is difficult and surgical excision is practicable only when lesions are painful and few; also, recurrences after excision are frequent. Electro-coagulation, carbon dioxide laser, cryotherapy, and radiotherapy are generally ineffective. Commercial support: None identified.
Commercial support: None identified.
P516 Electronmicroscopic investigation into the possible etiology of rosacea and the implication for treatment Richard Burroughs, MD, National Capital Consortium (Walter Reed Army Medical Center), Washington, DC, United States; Kurt Maggio, MD, Walter Reed Army Medical Center, Washington, DC, United States
Human head lice have become increasingly resistant to pyrethroids and lindane. Malathion resistance is still rare. Malathion is the only therapy that is consistently ovicidal. The most favorable circumstance for head lice propagation assumes the maximum time as a nit and the shortest possible time to mature from newly hatched nymph to egg-laying adult. The former is 11 days, the latter is 7 days. Treatment with an agent to which there is genetic resistance is unproductive. In the case of pediculicides that are non-ovicidal (eg, lindane and the pyrethroids), 3 rounds of treatment are necessary to ensure lice eradication, which is inconsistent with the package inserts for these products. For ovicidal therapies (malathion), a maximum of 2 treatments are necessary, consistent with the malathion package insert. The implication is that malathion should be used as a first line agent in the treatment of head lice, with abandonment of pyrethroids and lindane. Furthermore, no nit policies in schools can and should be rendered obsolete with the requirement of proof of treatment with malathion. As malathion has no resistance in the United States, treatment provides for a safe return to school the following day.
The presence of Demodex folliculorum has been implicated in the pathogenesis of rosacea, but never proven to be a causative agent. Most individuals in the general population have Demodex folliculorum present in their skin, yet only a fraction of the normal population develops rosacea. We hypothesize that cutaneous changes of rosacea are caused by an inflammatory response to intracellular bacteria within Demodex folliculorum. Candidate intracellular organisms include Wolbachia, other Rickettsia-like bacteria, g-proteobacteria, cytophaga-flavobacterium-bacteroides bacteria, and spiroplasmas. Our poster depicts the first known complete electron microscopic cross-section of Demodex folliculorum. This mite was obtained from sebum expressed from the face of a patient with rosacea, then imaged with electronmicroscopy. Perfect for presentation, the poster is comprised of 24 separate electron micrographs which are pieced together digitally to depict the entire mite and its contents. Inside Demodex there are several structures which have sizes consistent with intracellular bacteria and fungi for which we will perform PCR testing next summer. We presently have a grant from the National Rosacea Society for PCR and this poster depicts our preliminary microscopic investigation. This poster will be the first publication of our preliminary results. Our aim is to accurately identify the intracellular organism that may be responsible for Rosacea and thereby improve medical treatment for this disease. The intracellular bacteria we are investigating are uniformly sensitive to tetracyclines. This might clarify rosacea’s favorable response to tetracycline. Could the effects of antibiotics in rosacea be caused by their actions on intracellular bacteria of Demodex, rather than to a postulated anti-inflammatory mechanism? We believe so, and will demonstrate this in a first-of-its-kind poster.
Commercial support: None identified.
Commercial support: None identified.
P514 Therapeutic and public policy implications of head lice life cycle and resistance Jacob Levitt, MD, Mount Sinai School of Medicine, New York, NY, United States; Lily Clark, MD, Mount Sinai School of Medicine, New York, NY, United States; Mark Lebwohl, MD, Mount Sinai School of Medicine, New York, NY, United States
AB44
J AM ACAD DERMATOL
FEBRUARY 2007
P515
The perplexing progression of paraneoplastic pemphigus in a patient with CLL Thomas Busick, MD, Scott & White Hospital, Texas A&M Health Science Center, Temple, TX, United States; Daniel Bennett, MD, Scott & White Hospital, Texas A&M Health Science Center, Temple, TX, United States; Ronald Grimwood, MD, Scott & White Hospital, Texas A&M Health Science Center, Temple, TX, United States Background: Paraneoplastic pemphigus is a bullous disorder most commonly associated with a lymphoproliferative cancer; most commonly non-Hodgkins lymphoma (NHL), chronic lymphocytic leukemia (CLL), or a Castleman’s tumor. The classic presentation includes severe mucosal and genital erosions, a polymorpous pruritic eruption on the trunk with bullae, and often severe palmar/plantar ulcerations. Skin findings are often not correlated with the clinical stage of the malignancy. Diagnosis is made with a combination of biopsy, direct immunofluorescence (DIF), and indirect immunofluorescence (IIF) on rat bladder. Case report: We present the history and a photographic series of a 55-year-old white male with CLL and an indolent presentation of paraneoplastic pemphigus. His rash evolved through multiple stages clinically, histologically, and by immunofluorescence over a 5-month period. Initial presentation was clinically and histologically most consistent with a lichenoid drug eruption. After failing prednisone therapy, the patient returned erythrodermic with islands of sparing and was thought to have pityriasis rubra pilaris, but a repeat biopsy was unchanged. Next, he developed oral, periocular, and genital erosions to accompany the erythroderma. Paraneoplastic pemphigus was suspected, but DIF was initially negative. Several days later, a repeat DIF demonstrated a pemphigus pattern, but IIF was negative. One month later, the IIF became positive only on rat bladder, and the patient had developed impressive ulcerations of his fingertips with shedding of several nails. Throughout the course of his rash, the patient’s CLL was well controlled. His paraneoplastic pemphigus was improving on cyclosporine at time of submission. Comment: Although this patient’s final eruption fit the classic definition of paraneoplastic pemphigus, his rash took several months to evolve into that state. The biopsies and immunofluorescence studies followed a similar evolution. Our case illustrates the difficulty in diagnosing this rare entity as well as the importance of close follow up and reevaluation of the difficult patient.
Multiple cutaneous leiomyoma Mouna Jamali, MD, UHC, Ibn Rochd, Casablanca, MO, Morocco; Khadija Khadir, PhD, UHC, Ibn Rochd, Casablanca, MO, Morocco; Sofia Azzouzi, PhD, UHC, Ibn Rochd, Casablanca, MO, Morocco; Hakima Benchikhi, PhD, UHC, Ibn Rochd, Casablanca, MO, Morocco Cutaneous leiomyomas are rare benign, smooth muscle tumors characterized by painful nodules. They can be classified according to the derivation, as piloleiomyomas, angioleiomyomas, and genital leiomyomas. We describe a patient who has multiple cutaneous leiomyomas. A 44-year-old woman was referred for multiple lesions of the trunk. They began as small reddish-brown papules 10 years earlier and had slowly increased in size and in number over this period. There was no history of trauma, and no familial similar lesions. The lesions became recently painful, spontaneously or when palpated. Physical examination revealed multiple, painful, firm, non-tender, red nodules and papules, 2 cm to 3 cm in size on her trunk and 3 mm to 6 mm on her right upper arm. She had no other lesions elsewhere. Histopathologic examination of a nodule provided the diagnosis of cutaneous leiomyoma. An ultrasound scanning of pelvis had not revealed others lesions as uterine leiomyoma. Surgical removal was performed for only the bigger and painful lesions of the trunk. Our patient has multiple cutaneous leiomyoma. They are benign tumour that derived from the erector pili muscle. They arise in young adults as multiple, firm dermal papules and sometimes nodules that are classically pink or red in color. They can be painful when palpated, exposed to a cold environment, or as a result of emotional stress. The cause of the pain was initially proposed to be caused by the leiomyoma pressing on the cutaneous nerves. However, others have felt it to be more likely secondary to contraction of the muscle fibres making up the tumor itself. There have been several reports of the familial form of cutaneous leiomyomia and it is now thought to be an autosomal dominantly inherited disorder with incomplete penetrance. The association of cutaneous and uterine leiomyomia is not rare; this why all female patients with cutaneous lesions should have a pelvic ultrasound scan. The treatment of cutaneous leiomyoma is difficult and surgical excision is practicable only when lesions are painful and few; also, recurrences after excision are frequent. Electro-coagulation, carbon dioxide laser, cryotherapy, and radiotherapy are generally ineffective. Commercial support: None identified.
Commercial support: None identified.
P516 Electronmicroscopic investigation into the possible etiology of rosacea and the implication for treatment Richard Burroughs, MD, National Capital Consortium (Walter Reed Army Medical Center), Washington, DC, United States; Kurt Maggio, MD, Walter Reed Army Medical Center, Washington, DC, United States
Human head lice have become increasingly resistant to pyrethroids and lindane. Malathion resistance is still rare. Malathion is the only therapy that is consistently ovicidal. The most favorable circumstance for head lice propagation assumes the maximum time as a nit and the shortest possible time to mature from newly hatched nymph to egg-laying adult. The former is 11 days, the latter is 7 days. Treatment with an agent to which there is genetic resistance is unproductive. In the case of pediculicides that are non-ovicidal (eg, lindane and the pyrethroids), 3 rounds of treatment are necessary to ensure lice eradication, which is inconsistent with the package inserts for these products. For ovicidal therapies (malathion), a maximum of 2 treatments are necessary, consistent with the malathion package insert. The implication is that malathion should be used as a first line agent in the treatment of head lice, with abandonment of pyrethroids and lindane. Furthermore, no nit policies in schools can and should be rendered obsolete with the requirement of proof of treatment with malathion. As malathion has no resistance in the United States, treatment provides for a safe return to school the following day.
The presence of Demodex folliculorum has been implicated in the pathogenesis of rosacea, but never proven to be a causative agent. Most individuals in the general population have Demodex folliculorum present in their skin, yet only a fraction of the normal population develops rosacea. We hypothesize that cutaneous changes of rosacea are caused by an inflammatory response to intracellular bacteria within Demodex folliculorum. Candidate intracellular organisms include Wolbachia, other Rickettsia-like bacteria, g-proteobacteria, cytophaga-flavobacterium-bacteroides bacteria, and spiroplasmas. Our poster depicts the first known complete electron microscopic cross-section of Demodex folliculorum. This mite was obtained from sebum expressed from the face of a patient with rosacea, then imaged with electronmicroscopy. Perfect for presentation, the poster is comprised of 24 separate electron micrographs which are pieced together digitally to depict the entire mite and its contents. Inside Demodex there are several structures which have sizes consistent with intracellular bacteria and fungi for which we will perform PCR testing next summer. We presently have a grant from the National Rosacea Society for PCR and this poster depicts our preliminary microscopic investigation. This poster will be the first publication of our preliminary results. Our aim is to accurately identify the intracellular organism that may be responsible for Rosacea and thereby improve medical treatment for this disease. The intracellular bacteria we are investigating are uniformly sensitive to tetracyclines. This might clarify rosacea’s favorable response to tetracycline. Could the effects of antibiotics in rosacea be caused by their actions on intracellular bacteria of Demodex, rather than to a postulated anti-inflammatory mechanism? We believe so, and will demonstrate this in a first-of-its-kind poster.
Commercial support: None identified.
Commercial support: None identified.
P514 Therapeutic and public policy implications of head lice life cycle and resistance Jacob Levitt, MD, Mount Sinai School of Medicine, New York, NY, United States; Lily Clark, MD, Mount Sinai School of Medicine, New York, NY, United States; Mark Lebwohl, MD, Mount Sinai School of Medicine, New York, NY, United States
AB44
J AM ACAD DERMATOL
FEBRUARY 2007