Dermoscopic Features of Skin Lesions in Patients With Mastocytosis

Vañó‐Galván, Sergio

doi:10.1001/archdermatol.2011.190

Cited by 73 publications

(82 citation statements)

References 51 publications

(27 reference statements)

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“…A recent study using dermoscopy in cm described four patterns: light brown blot, yellow-orange blot, pigment network, and reticular vascular. The reticular vascular pattern was seen in all patients with tmep and was associated with a more frequent need for anti-mediator therapy 13 , also consistent with the severity of the symptoms seen in our patient.…”

Section: Discussionsupporting

confidence: 89%

Symptomatic Response to Imatinib Mesylate in Cutaneous Mastocytosis Associated with Chronic Myelomonocytic Leukemia

et al. 2013

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1 major and 1 minor, or 3 minor World Health Organization diagnostic criteria, which are listed in Table ii 4 . Although cutaneous and systemic involvement can occur together in adults, children rarely experience systemic manifestations and often have self-limiting disease [5][6][7] . The treatment of sm in adults remains a challenge, in part because of its chronicity and heterogeneity. CASE DESCRIPTIONA 67-year-old white woman presented with progressive and debilitating pruritus of 5 years' duration.Her symptoms had begun with intermittent pruritus of her arms, which then progressed to involve unexposed areas. The condition was unresponsive to treatment with histamine antagonists and ultraviolet B phototherapy. Two years later, she was noted to have peripheral blood monocytosis. Bone marrow (bm) examination demonstrated hypercellularity, dysplastic erythroid precursors and megakaryocytes, and increased monocyte precursors (Figure 1), but no increase in blasts. Blood and marrow findings were diagnostic of chronic myelomonocytic leukemia (cmml) 1. Mast cell numbers and morphology were normal.When the patient presented to our tertiary care centre, her pruritus had become constant and involved her entire body. She was unable to sleep unless medicated with sedatives. She was struggling to work and was having suicidal thoughts. Hot showers exacerbated her pruritus, but symptoms persisted even with avoidance of triggers. She noted a chronic truncal rash and erythematous patches on her arms. She denied flushing, wheezing, and diarrhea. Her medical history was otherwise unremarkable.Physical examination revealed a fine maculopapular rash with tan lesions over the patient's anterior trunk, concentrated near the umbilicus and under the breasts, and erythematous plaques on the left arm. She did not have dermatographia.White blood cell count was 6.8×10 3 /μL, with 19% monocytes and 1% eosinophils. Hemoglobin ABSTRACTMastocytosis is an uncommon disorder defined by increased and abnormal mast cells in one or more tissues. Cutaneous mastocytosis (cm) is limited to the skin, with varying degrees of rash, pruritus, and disfigurement. Systemic mastocytosis (sm) typically involves the bone marrow, sometimes in association with other bone marrow disorders, including chronic myelomonocytic leukemia (cmml). Mastocytosis has been associated with somatic mutations in the gene encoding the tyrosine kinase Kit, leading to identification of Kit as a therapeutic target. The Kit inhibitor imatinib mesylate is approved for aggressive sm. We present an unusual patient with disabling pruritus from telangiectasia macularis eruptiva perstans, a subtype of cm, and cmml, but with no evidence of systemic mast cell disease. She was treated with imatinib and experienced marked improvement in her pruritus. Concomitant cm and cmml have not previously been reported, and the present report is the first of successful imatinib therapy in an adult patient with cm.

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Section: Discussionsupporting

confidence: 89%

Symptomatic Response to Imatinib Mesylate in Cutaneous Mastocytosis Associated with Chronic Myelomonocytic Leukemia

et al. 2013

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“…All of them agreed that maculopapular CM, is dermatoscopically presented with brown reticular lines in combination with reddish to brown blot. [1,3,4,8] Dermatoscopic analysis of pigmented lesions in the presented patient, revealed irregular reticular lines with no typical regular holes as they can be usually seen in epidermal melanocytic nevi. Therefore, the term pigment networklike structure is more suitable to use in the presented case than pigment network.…”

Section: Discusionmentioning

confidence: 78%

“…[4] Although it can affect adults, usually it begins in childhood. Urticaria pigmentosa in adults is more likely to progress to systemic disease and its severity is related to the age of onset and to the size of cutaneous involvement.…”

Section: Discusionmentioning

confidence: 99%

“…[2] In recent years, dermatoscopy has emerged as a simple and useful tool for the diagnosis of melanocytic and nonmelanocytic skin lesions, providing rapid and easy evaluation of the colors and microstructure of the epidermis, the dermoepidermal junction, and the papillary dermis, not visible to the naked eye. [3,4] Several diagnostic algorithms based on the use of dermatoscopy have been developed for melanocytic and nonmelanocytic skin lesions. One of the main criteria for the diagnose of melanocytic lesions is the presence of the brown reticular lines (pigmented network).…”

Section: Introductionmentioning

confidence: 99%

“…[5][6][7] Skin lesions of mastocytosis represent one more nonmelanocytic lesion that may exhibit a pigment network. [1,3,4,8] …”

Section: Introductionmentioning

confidence: 99%

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Dermatoscopy in diagnosing maculopapular cutaneous mastocytosis

Popadić¹

2016

Praxis Med

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The diagnosis of cutaneous mastocytosis is based on the clinical finding of the pigmented macules or papules, positive Darier's sign and a dermal infiltrate composed of mast cells that can be better visualized by the use of special stains (Giemsa or Toluidine blue). Clinical, dermatoscopic and histopathological presentations of new appearing pigmented lesions in a female patient were presented. Dermatoscopic findings of pigment network-like structure combined with parallel pigmented lines and central reddish background in a adult patient with new appearing pigmented lesions should led us to searching for cutaneous mastocytosis.

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Risk of solid cancer, cardiovascular disease, anaphylaxis, osteoporosis and fractures in patients with systemic mastocytosis: A nationwide population‐based study

et al. 2016

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In patients with systemic mastocytosis (SM), several aspects of morbidity remain poorly understood. We assessed the risk of solid cancers, cardiovascular disease, anaphylaxis, osteoporosis, and fractures in SM patients. Using Danish medical registries, we conducted a nationwide population-based cohort study including 687 adult (≥15 years) SM patients diagnosed during 1997-2012. A comparison cohort of 68,700 subjects from the general Danish population who were alive and without SM at the given SM subject's diagnosis were age- and gender-matched. Outcomes were a new diagnosis of solid cancer, venous thromboembolism (VTE), myocardial infarction (MI), stroke, anaphylaxis, osteoporosis, or fracture. For solid cancers the hazard ratio (HR) was 2.4 (95% confidence interval [CI] 1.9-2.8) with a 10-year absolute risk (AR) in the SM-cohort of 12.6% (95% CI 9.4-16.3). Specifically, we found a HR of 7.5 (95% CI 4.4-13.0) for melanoma and a HR of 2.5 (95% CI 1.7-3.5) for non-melanoma skin cancers (NMSCs). For VTE we found a HR of 1.9 (95% CI 1.2-3.0), with a 10-year AR of 3.9% (95% CI 2.3-6.1); for MI a nonsignificant increased HR of 1.4 (95% CI 0.9-2.3), with a 10-year AR of 1.8% (95% CI 0.9-3.2); and for stroke a HR of 1.6 (95% CI 1.1-2.3) with a 10-year AR of 4.6% (95% CI 2.8-6.9). The HR for anaphylaxis was 7.2 (95% CI 5.3-9.9), and the 10-year AR was 3.1% (95% CI 1.9-4.9). For osteoporosis the HR was 3.6 (95% CI 2.7-4.6) with a 10-year AR of 7.2% (95% CI 5.2-9.8). For fractures the HR was 1.2 (95% CI 0.9-1.6) and the 10-year AR was 5.9% (95% CI 3.9-8.4). SM patients are at increased risk of solid cancers - especially melanoma and NMSC-and cardiovascular disease. The risk of anaphylaxis and osteoporosis is clearly increased in SM, though absolute risk was low in this population-based study. The fracture-risk was only slightly increased. Am. J. Hematol. 91:1069-1075, 2016. © 2016 Wiley Periodicals, Inc.

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Dermoscopic Features of Skin Lesions in Patients With Mastocytosis

Cited by 73 publications

References 51 publications

Symptomatic Response to Imatinib Mesylate in Cutaneous Mastocytosis Associated with Chronic Myelomonocytic Leukemia

Symptomatic Response to Imatinib Mesylate in Cutaneous Mastocytosis Associated with Chronic Myelomonocytic Leukemia

Dermatoscopy in diagnosing maculopapular cutaneous mastocytosis

Risk of solid cancer, cardiovascular disease, anaphylaxis, osteoporosis and fractures in patients with systemic mastocytosis: A nationwide population‐based study

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