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Dermoscopic Features of Skin Lesions in Patients with Mastocytosis

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Dermoscopic Features of Skin Lesions in Patients with Mastocytosis STUDY Dermoscopic Features of Skin Lesions in Patients With Mastocytosis Sergio Vano-Galvan, MD, PhD; Iva´n A´ lvarez-Twose, MD; Elena De las Heras, MD, PhD; J. M. Morgado, Msc; Almudena Matito, MD; Laura Sa´nchez-Mun˜oz, MD, PhD; Maria N. Plana, MD, PhD; Pedro Jae´n, MD, PhD; Alberto Orfao, MD, PhD; Luis Escribano, MD, PhD Objectives: To evaluate dermoscopic features in a group factors for more symptomatic forms of the disease ac- of 127 patients with mastocytosis in the skin and to in- cording to the need for daily antimediator therapy. vestigate the relationship between different dermo- scopic patterns and other clinical and biological charac- Results: Four distinct dermoscopic patterns were ob- teristics of the disease. served: yellow-orange blot, pigment network, reticular vascular pattern, and (most frequently) light-brown blot. Design: Clinical and laboratory data were compared A reticular vascular pattern was identified in all telangi- among patients with mastocytosis grouped according to ectasia macular eruptiva and some maculopapular mas- the different dermoscopic patterns. tocytosis. In turn, all patients with mastocytoma dis- played the yellow-orange blot pattern. The reticular Setting: Patients were selected from the Instituto de Es- vascular dermoscopic pattern was associated with the need tudios de Mastocitosis de Castilla La Mancha and the De- for daily antimediator therapy; this pattern, together with partment of Dermatology of Hospital Universitario Ramo´n serum tryptase levels and plaque-type mastocytosis, rep- y Cajal from April 1 through September 30, 2009. resented the best combination of independent factors to predict the need for maintained antimediator therapy. Patients: Overall, 127 consecutive patients (70 fe- males [55.1%] and 57 males [44.9%]; median age, 17 Conclusions: Dermoscopy is a feasible method for the years; range, 0-81 years) with mastocytosis in the skin subclassification of mastocytosis. Of note, a reticular vas- were included in the study. cular pattern is more frequently associated with the need for antimediator therapy. Main Outcome Measures: Evaluation of dermo- scopic patterns and investigation of potential predictive Arch Dermatol. 2011;147(8):932-940 KIN IS THE MOST COMMONLY tion, telangiectasia macularis eruptiva per- involved tissue in mastocy- stans (TMEP) also has been reported as a tosis, being affected in virtu- rare subvariant of MIS characterized by red- Author Affiliations: ally all pediatric and most dish macules that occur due to underly- Department of Dermatology adult cases.1-6 Because a com- ing, dilated, dermal, thin-walled blood ves- (Drs Vano-Galvan, De las Heras, Splete bone marrow study is required to es- sels; it is typically seen in adults.8-10 More and Jae´n) and Unidad de tablish the systemic nature of the dis- recently, (multiple) nodular and plaque- Bioestadı´stica Clı´nica (Dr Plana), ease, the term mastocytosis in the skin (MIS) type skin lesions also have been de- Hospital Universitario Ramo´ny recently has been proposed to define those scribed.11 They have been proposed as new Cajal, Madrid, Instituto de patients (eg, children with proven cuta- variants of MIS on the basis of the under- Estudios de Mastocitosis de Castilla La Mancha, Hospital neous mastocytosis) in whom screening lying clinical and pathogenic mechanisms. Virgen del Valle, Toledo for systemic involvement has not been Although careful inspection of skin le- (Drs A´ lvarez-Twose, Matito, performed. sions frequently suffices to identify MIS, Sa´nchez-Mun˜ oz, Jae´n, and According to the World Health Organi- a skin biopsy followed by histologic evalu- Escribano and Mr Morgado), zation, MIS may be subclassified into 3 vari- ation plus immunohistochemistry for Red Espan˜ ola de Mastocitosis ants: maculopapular cutaneous mastocyto- tryptase and c-kit are required to reach a (Dr A´ lvarez-Twose, Matito, sis (MPCM) (also known as urticaria final diagnosis.12 Histologic criteria for the Sa´nchez-Mun˜ oz, Orfao, and pigmentosa [UP]), diffuse cutaneous mas- diagnosis of MIS include the presence of Escribano and Mr Morgado), tocytosis, and solitary mastocytoma of the large aggregates (Ͼ15 cells per cluster) of Servicio General de Citometrı´a, skin.7 The most common variant is MPCM, tryptase-positive mast cells (MCs) or scat- Centro de Investigacio´n del Ca´ncer/Instituto de Biologı´a which is found in children and adults with tered MCs exceeding 20 cells per micro- ϫ 13,14 Molecular y Celular del Ca´ncer, mastocytosis; diffuse cutaneous mastocy- scopic high-power ( 40) field. In a and Department of Medicine, tosis and solitary mastocytoma of the skin variable percentage of cases, c-kit muta- Universidad de Salamanca, are less frequently observed and typically are tion at codon 816 also is detected in le- Salamanca (Dr Orfao), Spain. restricted to pediatric patients. In addi- sional skin.15,16 ARCH DERMATOL/ VOL 147 (NO. 8), AUG 2011 WWW.ARCHDERMATOL.COM 932 ©2011 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Corrected on August 23, 2011 Dermoscopy is a noninvasive technique based on in Table 1. Classification of Cutaneous Mastocytosis vivo epiluminescence microscopy, which provides rapid 11 and easy evaluation of the colors and microstructure of by Hartmann and Henz the epidermis, the dermoepidermal junction, and the pap- illary dermis, none of which are visible to the naked eye. Classification of Cutaneous Mastocytosis In recent years, dermoscopy has emerged as a simple and 1. Maculopapular cutaneous mastocytosis useful tool for the diagnosis of melanocytic and nonme- 2. Plaque-type cutaneous mastocytosis 3. Nodular cutaneous mastocytosis/mastocytoma (ie, solitary lanocytic skin lesions, and it has proven to be especially or multiple) 17 useful for early recognition of malignant melanoma. Sev- 4. Diffuse cutaneous mastocytosis eral diagnostic algorithms based on the use of dermos- 5. Telangiectatic cutaneous mastocytosis copy have been developed for melanocytic and nonme- lanocytic skin lesions. However, currently, information regarding the dermoscopic patterns of skin lesions in mas- tocytosis is scanty and restricted to individual case re- ferent dermoscopic patterns were identified, and each pa- ports or very small patient series.18,19 In this study, we tient’s lesions were classified into 1 of these 4 subgroups. report on the dermoscopic patterns of a large series Subsequently, the potential relationship between the dermo- (n=127) of patients with mastocytosis and the relation- scopic patterns identified and specific clinical variants of the disease, sBt levels, and the severity of MC mediator symptoms ship between those patterns and the clinical and biologi- was investigated. cal characteristics of the disease. The interobserver and intraobserver reproducibility was as- sessed for each dermoscopic pattern evaluated for a lesion in METHODS all 127 patients. The evaluation of dermoscopic patterns was masked from clinical data. Digital dermoscopic images were ob- tained from all patients by 4 investigators (S.V.-G., I.A´ .-T., A.M., PATIENTS AND CLINICAL and L.E.), and the dermoscopic images were evaluated by 3 der- AND LABORATORY EXAMINATIONS matologists (S.V.-G., E.D.L.H, and P.J.) who established the pre- dominant dermoscopic pattern in each case. Regarding intra- Overall, 127 consecutive patients (70 females [55.1%] and 57 observer reproducibility, 1 of the dermatologists (S.V.-G.) males [44.9%]; median age, 17 years; range, 0-81 years) with evaluated each lesion and reevaluated all of them 3 months later. MIS who were referred to the Instituto de Estudios de Mas- Regarding interobserver reproducibility, 2 dermatologists tocitosis de Castilla La Mancha or the Department of Derma- (E.D.L.H. and S.V.-G.) independently evaluated the same le- tology of Hospital Ramo´n y Cajal from April 1 through Sep- sions, and the results were compared. tember 30, 2009, were included in the study. Among the 127 patients, 61 (48.0%) were younger than 14 years and 66 (52.0%) were adults (median [range] age at the time of dermoscopy, 3 STATISTICAL ANALYSES years [0-11 years] and 38 years [15-81 years], respectively). In all patients except those with mastocytoma who had typical For all continuous variables, median and range were calcu- lated but for categorical variables, frequencies were reported. clinical features at arrival, the diagnosis of MIS was estab- ␹2 lished on the basis of a skin biopsy specimen. Median (range) The Mann-Whitney and tests were used to assess the statis- tical significance of differences observed between groups for time from disease onset to inclusion in the study was 11 years ␬ (3-60 years) in adults and 1 year (0-8 years) in children continuous and categorical variables, respectively. The sta- Ͻ tistics were calculated to assess interobserver and intraob- (P .001). The study was approved by the local ethics com- ␬ mittees, and each participant gave informed consent before en- server agreement. With regard to the interpretation of sta- tering the study, according to the tenets of the Declaration of tistics, a value of 1.00 indicates perfect agreement, values greater Helsinki. than 0.80 are considered excellent, values between 0.61 and Classification of MIS was performed in all patients after care- 0.80 are good, values between 0.40 and 0.60 are fair, and val- ful examination of the skin following the criteria proposed by ues less than 0.40 are poor. To identify the best combination of independent factors associated with each subgroup of pa- Hartmann and Henz (Table 1).11 Serum baseline tryptase (sBt) measurement was performed using a commercially available tients, multivariate (ie, logistic regression) analysis was per- technique (CAP; Phadia AB, Uppsala, Sweden); clinical symp- formed. For multivariate analyses, only those variables that showed statistically significant differences in the univariate study toms suggesting the release of MC mediators (eg, pruritus, flush- Յ ing, abdominal cramping, diarrhea, nausea or vomiting, and were included in the model.
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