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Beau Lines

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61. Cutaneous Manifestations of HIV Disease (Diagnosis)

related to HIV-1 infection. [ ] Beau lines, telogen effluvium, and pallor of the nail beds are the general effects of the chronic illness. Elongation of the eyelashes and softening and straightening of the scalp hair may be observed in HIV disease, and proximal subungual onychomycosis is also usually a sign of HIV disease. The frequency of onychomycosis may be higher in men than in women. Generalized alopecia can occur in patients with HIV who are treated with indinavir, an antiretroviral protease

2014 eMedicine.com

62. Acute Myelogenous Leukemia (Follow-up)

(or no initial complete response) who had no response to first-salvage therapy and received a second or subsequent salvage therapy had a response rate of 0%. These data underscore the need to develop new treatment options for these patients. Response to third-line therapy is even worse. Giles et al studied 594 patients with AML undergoing second salvage therapy from 1980 to 2004. [ ] The patient median age was 50 years. Salvage therapy included allogeneic stem cell transplantation (SCT), standard-dose (...) cell count every 1–3 months for 2 years, then every 3–6 months up to 5 years. The NCCN recommends a bone marrow aspirate and biopsy only if a peripheral smear is abnormal or cytopenias develop. [ ] Previous References Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood . 2016 May 19. 127 (20):2391-405. . . Smith MT, Skibola CF, Allan JM, Morgan GJ. Causal models

2014 eMedicine.com

63. Acute Lymphoblastic Leukemia (Follow-up)

of imatinib to chemotherapy. [ , , ] Nilotinib Nilotinib is a tyrosine kinase inhibitor that has a higher binding affinity and selectivity for the ABL kinase than imatinib. [ ] Nilotinib has 20 to 50 times the inhibitory activity against imatinib-sensitive CML cell lines relative to imatinib. In a phase II study in patients with relapsed/refractory Ph+ ALL, complete responses were reported in 24% of patients treated with nilotinib. [ ] Although nilotinib is approved in the relapsed/refractory setting (...) of Relapsed ALL Patients with relapsed acute lymphoblastic leukemia (ALL) have an extremely poor prognosis. Most patients are referred for investigational therapies. Patients who have not previously undergone transplantation are referred for such therapy, preferably after obtaining a complete response to salvage therapy. Reinduction regimens include standard chemotherapy regimens (similar to the front-line setting), novel chemotherapeutic agents or immunotherapies (blinatumomab, inotuzumab ozogamicin

2014 eMedicine.com

64. Mastocytosis, Systemic (Diagnosis)

+ / KIT + pluripotent hematopoietic cells in the bone marrow. [ ] The neoplastic clone of mast cells express abnormal cell surface markers CD25 and/or CD2. Mueller et al reported that the adhesion molecule CD44 is expressed in systemic mastocytosis cell lines and correlates with the aggressiveness of the disorder. They found that serum levels of soluble CD44 were higher in advanced systemic mastocytosis compared with indolent systemic mastocytosis or cutaneous mastocytosis, and correlated with overall (...) ] Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood . 2016 May 19. 127 (20):2391-405. . . Tefferi A, Levine RL, Lim KH, Abdel-Wahab O, Lasho TL, Patel J, et al. Frequent TET2 mutations in systemic mastocytosis: clinical, KITD816V and FIP1L1-PDGFRA correlates. Leukemia . 2009 May. 23(5):900-4. . Butterfield JH. Survey of aspirin administration in systemic

2014 eMedicine.com

65. Alopecia Areata (Diagnosis)

and the patches coalesce Ophiasis - Hair loss is localized to the sides and lower back of the scalp Sisaipho (ophiasis spelled backwards) - Hair loss spares the sides and back of the head Alopecia totalis - 100% hair loss on the scalp Alopecia universalis - Complete loss of hair on all hair-bearing areas Nail involvement, predominantly of the fingernails, is found in 6.8-49.4% of patients, most commonly in severe cases. Pitting is the most common; other reported abnormalities have included trachyonychia, Beau (...) lines, onychorrhexis, onychomadesis, koilonychias, leukonychia, and red lunulae See for more detail. Diagnosis Diagnosis usually can be made on clinical grounds. A scalp biopsy seldom is needed, but it can be helpful when the clinical diagnosis is less certain. See for more detail. Management Treatment is not mandatory, because the condition is benign, and spontaneous remissions and recurrences are common. Treatment can be topical or systemic. [ ] Corticosteroids Intralesional corticosteroid therapy

2014 eMedicine.com

66. Psoriatic Arthritis (Treatment)

with psoriatic arthritis, may include the following: Beau lines Leukonychia Onycholysis Oil spots Subungual hyperkeratosis Splinter hemorrhages Spotted lunulae Transverse ridging Cracking of the free edge of the nail Uniform nail pitting Extra-articular features are observed less frequently in patients with psoriatic arthritis than in those with rheumatoid arthritis (RA) but may include the following: Synovitis affecting flexor tendon sheaths, with sparing of the extensor tendon sheath Subcutaneous nodules (...) of autoimmunity, perhaps mediated by complement activation. The inflammatory nature of the skin and joint lesions in psoriatic arthritis is demonstrated by synovial-lining cell hyperplasia and mononuclear infiltration, resembling the histopathologic changes of RA. However, synovial-lining hyperplasia is less, macrophages are fewer, and vascularity is greater in psoriatic arthritis than in RA synovium. The cytokine profile for psoriatic arthritis reflects a complex interplay between T cells and monocyte

2014 eMedicine.com

67. Acute Myelogenous Leukemia (Treatment)

(or no initial complete response) who had no response to first-salvage therapy and received a second or subsequent salvage therapy had a response rate of 0%. These data underscore the need to develop new treatment options for these patients. Response to third-line therapy is even worse. Giles et al studied 594 patients with AML undergoing second salvage therapy from 1980 to 2004. [ ] The patient median age was 50 years. Salvage therapy included allogeneic stem cell transplantation (SCT), standard-dose (...) cell count every 1–3 months for 2 years, then every 3–6 months up to 5 years. The NCCN recommends a bone marrow aspirate and biopsy only if a peripheral smear is abnormal or cytopenias develop. [ ] Previous References Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood . 2016 May 19. 127 (20):2391-405. . . Smith MT, Skibola CF, Allan JM, Morgan GJ. Causal models

2014 eMedicine.com

68. Acute Lymphoblastic Leukemia (Treatment)

of imatinib to chemotherapy. [ , , ] Nilotinib Nilotinib is a tyrosine kinase inhibitor that has a higher binding affinity and selectivity for the ABL kinase than imatinib. [ ] Nilotinib has 20 to 50 times the inhibitory activity against imatinib-sensitive CML cell lines relative to imatinib. In a phase II study in patients with relapsed/refractory Ph+ ALL, complete responses were reported in 24% of patients treated with nilotinib. [ ] Although nilotinib is approved in the relapsed/refractory setting (...) of Relapsed ALL Patients with relapsed acute lymphoblastic leukemia (ALL) have an extremely poor prognosis. Most patients are referred for investigational therapies. Patients who have not previously undergone transplantation are referred for such therapy, preferably after obtaining a complete response to salvage therapy. Reinduction regimens include standard chemotherapy regimens (similar to the front-line setting), novel chemotherapeutic agents or immunotherapies (blinatumomab, inotuzumab ozogamicin

2014 eMedicine.com

69. Psoriasis, Nails (Treatment)

or salmon patch of the nail bed This lesion is a translucent, yellow-red discoloration in the nail bed resembling a drop of oil beneath the nail plate. This patch is the most diagnostic sign of nail psoriasis. [ ] Pitting of the proximal nail matrix Pitting is a result of the loss of parakeratotic cells from the surface of the nail plate. Beau lines of the proximal nail matrix These lines are transverse lines in the nails due to intermittent inflammation causing growth arrest lines. Leukonychia (...) starts distally and progresses proximally, causing a traumatic uplifting of the distal nail plate. Secondary microbial colonization may occur. Nail plate crumbling Nail plate weakening due to disease of the underlying structures causes this condition. Splinter hemorrhage/dilated tortuous capillaries in the dermal papillae Splinter hemorrhages are longitudinal black lines due to minute foci of capillary hemorrhage between the nail bed and the nail plate. This is analogous to the Auspitz sign

2014 eMedicine.com

70. Polycythemia Vera (Treatment)

statistical significance at levels of 11,000/µL and above (HR 3.90, P = 0.02). An association between elevated WBC counts and thrombosis has also been found in studies of patients with essential thrombocythemia. These authors recommend including the WBC count when evaluating response to cytoreductive therapy. [ ] Initial research has suggested a role for interferon alpha as a first-line treatment, athough toxicity can be problematic. Interferon alpha is not yet approved for use in PV, but phase III (...) . The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Study Group: a survey of American Society of Hematology members' practice patterns. Blood . 2002 Feb 15. 99(4):1144-9. . . Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood . 2016 May 19. 127 (20):2391-405. . . James C, Ugo V, Le Couedic JP, et al. A unique clonal JAK2 mutation

2014 eMedicine.com

71. Psoriatic Arthritis (Overview)

with psoriatic arthritis, may include the following: Beau lines Leukonychia Onycholysis Oil spots Subungual hyperkeratosis Splinter hemorrhages Spotted lunulae Transverse ridging Cracking of the free edge of the nail Uniform nail pitting Extra-articular features are observed less frequently in patients with psoriatic arthritis than in those with rheumatoid arthritis (RA) but may include the following: Synovitis affecting flexor tendon sheaths, with sparing of the extensor tendon sheath Subcutaneous nodules (...) of autoimmunity, perhaps mediated by complement activation. The inflammatory nature of the skin and joint lesions in psoriatic arthritis is demonstrated by synovial-lining cell hyperplasia and mononuclear infiltration, resembling the histopathologic changes of RA. However, synovial-lining hyperplasia is less, macrophages are fewer, and vascularity is greater in psoriatic arthritis than in RA synovium. The cytokine profile for psoriatic arthritis reflects a complex interplay between T cells and monocyte

2014 eMedicine.com

72. Psoriatic Arthritis (Overview)

with psoriatic arthritis, may include the following: Beau lines Leukonychia Onycholysis Oil spots Subungual hyperkeratosis Splinter hemorrhages Spotted lunulae Transverse ridging Cracking of the free edge of the nail Uniform nail pitting Extra-articular features are observed less frequently in patients with psoriatic arthritis than in those with rheumatoid arthritis (RA) but may include the following: Synovitis affecting flexor tendon sheaths, with sparing of the extensor tendon sheath Subcutaneous nodules (...) of autoimmunity, perhaps mediated by complement activation. The inflammatory nature of the skin and joint lesions in psoriatic arthritis is demonstrated by synovial-lining cell hyperplasia and mononuclear infiltration, resembling the histopathologic changes of RA. However, synovial-lining hyperplasia is less, macrophages are fewer, and vascularity is greater in psoriatic arthritis than in RA synovium. The cytokine profile for psoriatic arthritis reflects a complex interplay between T cells and monocyte

2014 eMedicine.com

73. Psoriasis, Nails (Overview)

or salmon patch of the nail bed This lesion is a translucent, yellow-red discoloration in the nail bed resembling a drop of oil beneath the nail plate. This patch is the most diagnostic sign of nail psoriasis. [ ] Pitting of the proximal nail matrix Pitting is a result of the loss of parakeratotic cells from the surface of the nail plate. Beau lines of the proximal nail matrix These lines are transverse lines in the nails due to intermittent inflammation causing growth arrest lines. Leukonychia (...) starts distally and progresses proximally, causing a traumatic uplifting of the distal nail plate. Secondary microbial colonization may occur. Nail plate crumbling Nail plate weakening due to disease of the underlying structures causes this condition. Splinter hemorrhage/dilated tortuous capillaries in the dermal papillae Splinter hemorrhages are longitudinal black lines due to minute foci of capillary hemorrhage between the nail bed and the nail plate. This is analogous to the Auspitz sign

2014 eMedicine.com

74. Psoriatic Arthritis (Overview)

with psoriatic arthritis, may include the following: Beau lines Leukonychia Onycholysis Oil spots Subungual hyperkeratosis Splinter hemorrhages Spotted lunulae Transverse ridging Cracking of the free edge of the nail Uniform nail pitting Extra-articular features are observed less frequently in patients with psoriatic arthritis than in those with rheumatoid arthritis (RA) but may include the following: Synovitis affecting flexor tendon sheaths, with sparing of the extensor tendon sheath Subcutaneous nodules (...) of autoimmunity, perhaps mediated by complement activation. The inflammatory nature of the skin and joint lesions in psoriatic arthritis is demonstrated by synovial-lining cell hyperplasia and mononuclear infiltration, resembling the histopathologic changes of RA. However, synovial-lining hyperplasia is less, macrophages are fewer, and vascularity is greater in psoriatic arthritis than in RA synovium. The cytokine profile for psoriatic arthritis reflects a complex interplay between T cells and monocyte

2014 eMedicine.com

75. Alopecia Areata (Overview)

and the patches coalesce Ophiasis - Hair loss is localized to the sides and lower back of the scalp Sisaipho (ophiasis spelled backwards) - Hair loss spares the sides and back of the head Alopecia totalis - 100% hair loss on the scalp Alopecia universalis - Complete loss of hair on all hair-bearing areas Nail involvement, predominantly of the fingernails, is found in 6.8-49.4% of patients, most commonly in severe cases. Pitting is the most common; other reported abnormalities have included trachyonychia, Beau (...) lines, onychorrhexis, onychomadesis, koilonychias, leukonychia, and red lunulae See for more detail. Diagnosis Diagnosis usually can be made on clinical grounds. A scalp biopsy seldom is needed, but it can be helpful when the clinical diagnosis is less certain. See for more detail. Management Treatment is not mandatory, because the condition is benign, and spontaneous remissions and recurrences are common. Treatment can be topical or systemic. [ ] Corticosteroids Intralesional corticosteroid therapy

2014 eMedicine.com

76. Myelodysplastic Syndrome (Overview)

marrow mimicking may be seen. Bone marrow cells display aberrant morphology and maturation (dysmyelopoiesis), resulting in ineffective blood cell production. MDS affects hematopoiesis at the stem cell level, as indicated by cytogenetic abnormalities, molecular mutations, and morphologic and physiologic abnormalities in maturation and differentiation of one or more of the hematopoietic cell lines. [ , , ] See the image below. Blood film (1000× magnification) demonstrating a vacuolated blast (...) published in 2008 and 2016. The 2016 WHO classification of MDS is as follows [ ] : MDS with single-lineage dysplasia (MDS-SLD) – 1 or 2 blood cytopenias; in bone marrow, dysplasia in ≥ 10% of one cell line, < 5% blasts MDS with multilineage dysplasia (MDS-MLD) – 1-3 blood cytopenias, < 1 × 10 9 /L monocytes; in bone marrow, dysplasia in ≥ 10% of cells in ≥ 2 hematopoietic lineages < 15% ring sideroblasts (or < 5% ring sideroblasts if SF3B1 mutation present) < 5% blasts MDS with ring sideroblasts (MDS-RS

2014 eMedicine.com

77. Mastocytosis, Systemic (Overview)

+ / KIT + pluripotent hematopoietic cells in the bone marrow. [ ] The neoplastic clone of mast cells express abnormal cell surface markers CD25 and/or CD2. Mueller et al reported that the adhesion molecule CD44 is expressed in systemic mastocytosis cell lines and correlates with the aggressiveness of the disorder. They found that serum levels of soluble CD44 were higher in advanced systemic mastocytosis compared with indolent systemic mastocytosis or cutaneous mastocytosis, and correlated with overall (...) ] Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood . 2016 May 19. 127 (20):2391-405. . . Tefferi A, Levine RL, Lim KH, Abdel-Wahab O, Lasho TL, Patel J, et al. Frequent TET2 mutations in systemic mastocytosis: clinical, KITD816V and FIP1L1-PDGFRA correlates. Leukemia . 2009 May. 23(5):900-4. . Butterfield JH. Survey of aspirin administration in systemic

2014 eMedicine.com

78. Cutaneous Manifestations of HIV Disease (Overview)

related to HIV-1 infection. [ ] Beau lines, telogen effluvium, and pallor of the nail beds are the general effects of the chronic illness. Elongation of the eyelashes and softening and straightening of the scalp hair may be observed in HIV disease, and proximal subungual onychomycosis is also usually a sign of HIV disease. The frequency of onychomycosis may be higher in men than in women. Generalized alopecia can occur in patients with HIV who are treated with indinavir, an antiretroviral protease

2014 eMedicine.com

79. Nail Surgery (Treatment)

, cryotherapy is associated with high cure rates and minimal complications. The postoperative complications of freezing include pain, blistering, and transient neuropathy; the open wound heals by secondary intention within 2-5 weeks. [ ] Less common complications such as depigmentation, nail loss, Beau lines, transient neuropathy, and anesthesia may occur. Pain during the procedure is controlled by providing prophylactic analgesia with 600 mg of aspirin 2 hours before surgery. This regimen is continued 3 (...) at avulsion are associated with failed treatment outcomes and a greater risk of recurrence. Peridigital resection may be used to treat the soft tissue hypertrophy. When this approach is used, 2 incisions are made: the first incision is a curved line, hugging the lateral surface of the digit, and the second incision is made parallel to the first and is extended to the underlying subcutaneous fat to remove a wedge-shaped sample of tissue. At closure, the nail folds in the nail groove are oriented away from

2014 eMedicine.com

80. Cutaneous Manifestations of HIV Disease (Treatment)

related to HIV-1 infection. [ ] Beau lines, telogen effluvium, and pallor of the nail beds are the general effects of the chronic illness. Elongation of the eyelashes and softening and straightening of the scalp hair may be observed in HIV disease, and proximal subungual onychomycosis is also usually a sign of HIV disease. The frequency of onychomycosis may be higher in men than in women. Generalized alopecia can occur in patients with HIV who are treated with indinavir, an antiretroviral protease

2014 eMedicine.com

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