Progressive kaposiform hemangioendothelioma and sirolimus-related severe thrombocytopenia.

Kaposiform hemangioendothelioma is a locally invasive tumor and we were unable to find any previous reports of multifocal progression. Sirolimus, a mammalian target of rapamycin inhibitor, has been widely used to treat kaposiform hemangioendothelioma. Herein, we report a case of multifocal progressive kaposiform hemangioendothelioma, wherein sirolimus treatment caused severe thrombocytopenia. A 12-year-old East Asian girl presented with indurated dark-purple masses on her back. The patient had received three surgical interventions following the first appearance of the masses in 2012 and subsequent reappearances in 2014 and 2016. Kaposiform hemangioendothelioma was diagnosed based on radiological and pathological findings. Two more masses appeared in the following year. The patient was treated with oral sirolimus (2.5 mg/ m2/day) and developed grade 3 thrombocytopenia 8 days later. The patient was uneventfully relieved 5 days later after the withdrawal of sirolimus and the administration of appropriate medications. This rare case indicated that kaposiform hemangioendothelioma could be progressive with local metastatic characteristics in children. Besides, the severe sirolimus-induced complication highlights the importance of serum drug level monitoring during treatment. Physicians should be extremely cautious while treating kaposiform hemangioendothelioma patients with sirolimus.

Novel Diagnostics for Kaposi Sarcoma and Other Skin Diseases in Resource-Limited Settings.

In resource-limited settings, point-of-care diagnostic devices have the potential to reduce diagnostic delays and improve epidemiologic surveillance of dermatologic conditions. We outline novel-point-of care diagnostics that have recently been developed for dermatologic conditions that primarily affect patients living in resource-limited settings, namely, Kaposi sarcoma, cutaneous leishmaniasis, leprosy, Buruli ulcer, yaws, onchocerciasis, and lymphatic filariasis. All of the technologies described in this article are prototypes, and some have undergone field testing. These devices still require validation in real-world settings and effective pricing to have a major impact on dermatologic care in resource-limited settings.

The diagnostic challenge of mapping elephantiasis in the Tigray region of northern Ethiopia.

BACKGROUND: In Ethiopia, lymphatic filariasis and podoconiosis are the two neglected tropical diseases planned to be mapped together within the recently launched Ethiopian neglected tropical diseases master plan (2013-2015). However, other disorders cause tropical lymphedema, and this report aims to identify clinical epidemiological aspects of limb swelling in northern Ethiopia and to provide an algorithm orienting the clinical diagnosis. METHODS: Medical records of patients with lower limb elephantiasis attending the Italian Dermatological Centre of Mekele, Tigray capital city, over a 4-year period (2005-2009) were retrospectively analyzed. Nine variables were collected from the charts comprising demographic data, job, origin, literacy, clinical, histopathologic, microscopic, and cultural findings. RESULTS: Over a total of 511 patients, lymphedema resulted from trauma (40.7%), chronic venous insufficiency (12.5%), deep mycoses (10.8%), lymphatic filariasis (9.2%), elephantiasis nostras verrucosa (7.0%), tropical ulcer (6.3%), leprosy (4.9%), recurrent infections (3.1%), podoconiosis (1.8%), tuberculosis (1.0%), malignancy (1.3%), Kaposi's sarcoma (1.0%), leishmaniasis (0.2%), and neurofibromatosis (0.2%). CONCLUSIONS: Advanced-stage elephantiasis, chronic osteomyelitis, and podoconiosis not previously reported in Tigray were observed. Further epidemiological investigation and training programs addressed to healthcare providers at the peripheral level are needed to detect elephantiasis early, prevent disabilities, and improve patients' quality of life.

Kaposi's sarcoma: a presenting sign of HIV.

Kaposi's sarcoma (KS) is a multifocal cutaneous and extra cutaneous vascular proliferative disorder. In India, there are only a few cases of HIV-associated KS in published literature. A 26-year-old married man presented with asymptomatic elevated skin lesions over the face, trunk, both upper limbs and lower limbs with a duration of one-and-a-half months. Cutaneous examination revealed multiple violaceous papules and nodules, on the face (right upper and lower eyelids, upper lip), trunk and proximal part of both limbs. Oral cavity showed erythematous plaques, two in number, on the hard palate. Skin biopsy showed proliferation of thin walled capillaries with formation of slit like spaces, spindle cell proliferation, abundant extravasation of RBCs and moderately dense inflammatory infiltrate in the dermis. Thus a clinical diagnosis of cutaneous KS was confirmed. On testing with ELISA for HIV, the patient was for the first time diagnosed as HIV reactive. Thus KS was the presenting manifestation of HIV disease.

Leprosy and Kaposi sarcoma presenting as an immune reconstitution inflammatory syndrome in a patient with AIDS.

The simultaneous presence of infectious organisms within cutaneous lesions of Kaposi sarcoma in persons with AIDS has been demonstrated. We describe a patient with concurrent leprosy and Kaposi sarcoma presenting as an immune reconstitution inflammatory syndrome in the setting of AIDS.

Kaposi's sarcoma: a presenting manifestation of HIV infection in an Indian.

Kaposi's sarcoma (KS) is a multifocal neoplastic proliferation of endothelial cells predominantly involving skin and other organs. HIV-associated Kaposi's sarcoma has been rarely reported from India. A 38-year-old male presented with persistent swelling on the left lower limb for one year along with multiple erythematous to dusky papules and plaques of one and half months duration and swelling and black discoloration of right lower limb for one month. Cutaneous examination revealed numerous skin colored and erythematous papules and plaques distributed on the left lower limb on the anteromedial aspect and verrucous plaque on the left sole. Multiple erythematous, grouped papules were present over the soft palate. Skin biopsy showed numerous slit like spaces dissecting into the collagen of the upper and mid-dermis along with 'promontory sign' suggestive of Kaposi's sarcoma. Patient was found to be HIV-positive by ELISA test.

Kaposi's sarcoma in an HIV-positive person successfully treated with paclitaxel.

Epidemic Kaposi's sarcoma is one of the malignant neoplasms, which can develop in HIV-infected patients. Although the prevalence of HIV infection is reported to be high in Asian countries, Kaposi's sarcoma is rarely reported. We report a case of Kaposi's sarcoma involving the skin and oral mucosa along with extensive bilateral lymphedema of lower extremities, treated successfully with paclitaxel and antiretrovirals.

Skin cancer in skin of color / Skin cancer in skin of color

Skin cancer is less common in persons with skin of color than in light-skinned Caucasians but is often associated with greater morbidity and mortality. Thus, it is crucial that physicians become familiar with skin cancer in persons of color so as to maximize the likelihood of early detection of these tumors. In dark-skinned ethnic groups, squamous cell carcinoma is most common; squamous cell carcinoma and melanoma usually occur on nonsun-exposed sites; and ultraviolet radiation is not an important etiologic factor for skin cancer with the exception of basal cell carcinoma. Races of intermediate pigmentation, such as Hispanics and Asians, share epidemiologic and clinical features of dark-skinned ethnic groups and Caucasians. Skin cancers pose a significant risk in skin of color and clinicians should focus on preventive measures in these groups such as regular skin exams, self-examination, public education, and screening programs. LEARNING OBJECTIVE: At the completion of this learning activity, participants should be familiar with the epidemiology and unique clinical features of skin cancer in skin of color and be aware of strategies to prevent skin cancer in skin of color.

Thalidomide: dermatological indications, mechanisms of action and side-effects.

Thalidomide was first introduced in the 1950s as a sedative but was quickly removed from the market after it was linked to cases of severe birth defects. However, it has since made a remarkable comeback for the U.S. Food and Drug Administration-approved use in the treatment of erythema nodosum leprosum. Further, it has shown its effectiveness in unresponsive dermatological conditions such as actinic prurigo, adult Langerhans cell histiocytosis, aphthous stomatitis, Behçet's syndrome, graft-versus-host disease, cutaneous sarcoidosis, erythema multiforme, Jessner-Kanof lymphocytic infiltration of the skin, Kaposi sarcoma, lichen planus, lupus erythematosus, melanoma, prurigo nodularis, pyoderma gangrenosum and uraemic pruritus. This article reviews the history, pharmacology, mechanism of action, clinical uses and adverse effects of thalidomide.

The promise of thalidomide: evolving indications.

Thalidomide was first used in the late 1950s but it was withdrawn from the market in the 1960s for its notorious teratogenic effects. This drug was more recently rediscovered as a powerful immunomodulatory and antiinflammatory agent and was approved by the FDA in 1998 for treatment of erythema nodosum leprosum. Thalidomide has shown great promise in advanced or refractory multiple myeloma either alone or in combination with other agents. It has also demonstrated benefits in a wide variety of disparate conditions such as aphthous and genital ulcers, cancer cachexia, HIV, tuberculosis and chronic graft versus host disease. Thalidomide is being investigated for treatment of renal cell carcinoma, and liver and thyroid cancers. Better understanding of its many mechanisms of action has provoked great interest in its potential use for treatment of various disorders. This review focuses on thalidomide's mechanisms of action, biochemistry, pharmacokinetics and its use in erythema nodosum leprosum as well as multiple myeloma, graft versus host disease, and renal cell carcinoma.

Thalidomide for erythema nodosum leprosum and other applications.

Thalidomide, administered as a sedative and antiemetic decades ago, was considered responsible for numerous devastating cases of birth defects and consequently was banned from markets worldwide. However, the drug remarkably has resurfaced with promise of immunomodulatory benefit in a wide array of immunologic disorders for which available treatments were limited. It is approved by the Food and Drug Administration for erythema nodosum leprosum (ENL). Although the relative paucity of leprosy and ENL worldwide may perceivably limit interest in and knowledge about thalidomide, increasing numbers of new and potential uses expand its applicability widely beyond ENL. Thalidomide, an inhibitor of tumor necrosis factor a, is the best known agent for short-term treatment of ENL skin manifestations, as well as postremission maintenance therapy to prevent recurrence. For this indication, it is effective as monotherapy and as part of combination therapy with corticosteroids. Studies of thalidomide in chronic graft-versus-host disease showed benefit in children and adults as treatment, but not as prophylaxis. The agent has been administered successfully for treatment of cachexia related to cancer, tuberculosis, and human immunodeficiency virus infection, although evidence of efficacy is inconclusive. Thalidomide monotherapy effectively induced objective response in trials in patients with both newly diagnosed and advanced or refractory multiple myeloma. Combination therapy with thalidomide and corticosteroids was also effective in these patients, as well as in treatment of aphthous and genital ulcers. Limited evidence supports the drug's benefit in treatment of Kaposi's sarcoma. Other thalidomide applications include Crohn's disease, rheumatoid arthritis, and multiple sclerosis. Somnolence, constipation, and rash were the most frequently cited adverse effects in studies, but thalidomide-induced neuropathy and idiopathic thromboembolism were critical causes for drug discontinuation. Thalidomide is still contraindicated in pregnant women, women of childbearing age, and sexually active men not using contraception. Clinicians should be conversant with thalidomide in ENL (its primary application) in the natural course of leprosy, as well as in the agent's other applications.

Thalidomide for erythema nodosum leprosum and other applications

Thalidomide, administered as a sedative and antiemetic decades ago, was considered responsible for numerous devastating cases of birth defects and consequently was banned from markets worldwide. However, the drug remarkably has resurfaced with promise of immunomodulatory benefit in a wide array of immunologic disorders for which available treatments were limited. It is approved by the Food and Drug Administration for erythema nodosum leprosum (ENL). Although the relative paucity of leprosy and ENL worldwide may perceivably limit interest in and knowledge about thalidomide, increasing numbers of new and potential uses expand its applicability widely beyond ENL. Thalidomide, an inhibitor of tumor necrosis factor a, is the best known agent for short-term treatment of ENL skin manifestations, as well as postremission maintenance therapy to prevent recurrence. For this indication, it is effective as monotherapy and as part of combination therapy with corticosteroids. Studies of thalidomide in chronic graft-versus-host disease showed benefit in children and adults as treatment, but not as prophylaxis. The agent has been administered successfully for treatment of cachexia related to cancer, tuberculosis, and human immunodeficiency virus infection, although evidence of efficacy is inconclusive. Thalidomide monotherapy effectively induced objective response in trials in patients with both newly diagnosed and advanced or refractory multiple myeloma. Combination therapy with thalidomide and corticosteroids was also effective in these patients, as well as in treatment of aphthous and genital ulcers. Limited evidence supports the drug's benefit in treatment of Kaposi's sarcoma. Other thalidomide applications include Crohn's disease, rheumatoid arthritis, and multiple sclerosis. Somnolence, constipation, and rash were the most frequently cited adverse effects in studies, but thalidomide-induced neuropathy and idiopathic thromboembolism were critical causes for drug discontinuation. Thalidomide is still contraindicated in pregnant women, women of childbearing age, and sexually active men not using contraception. Clinicians should be conversant with thalidomide in ENL (its primary application) in the natural course of leprosy, as well as in the agent's other applications.