Large maculopapular cutaneous lesions are associated with favorable outcome in childhood-onset mastocytosis Tim Wiechers, MD, Anja Rabenhorst, PhD, Tina.

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Date of download: 6/22/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Dermoscopic Features of Skin Lesions in Patients.

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Large maculopapular cutaneous lesions are associated with favorable outcome in childhood-onset mastocytosis Tim Wiechers, MD, Anja Rabenhorst, PhD, Tina Schick, MD, Liane M. Preussner, MD, Anja Förster, PhD, Peter Valent, MD, Hans-Peter Horny, MD, Karl Sotlar, MD, Karin Hartmann, MD Journal of Allergy and Clinical Immunology Volume 136, Issue 6, Pages 1581-1590.e3 (December 2015) DOI: 10.1016/j.jaci.2015.05.034 Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Patients with childhood-onset mastocytosis present with heterogeneous cutaneous lesions. According to the type of skin lesion, patients were categorized into those with MPCM, DCM, and mastocytoma. Patients with MPCM were further subdivided into 3 groups: MPCM-small, MPCM-large, and MPCM-other. Journal of Allergy and Clinical Immunology 2015 136, 1581-1590.e3DOI: (10.1016/j.jaci.2015.05.034) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 Serum tryptase levels differ between cutaneous subforms and variants. A and B, Tryptase levels were measured in patients with different cutaneous subforms (Fig 2, A) and in patients with MPCM-large lesions subdivided according to the number of skin lesions (Fig 2, B). Data are presented as box plots (minimum to maximum) with medians (left) and scatter dot plots with means (right). C, Representative course of tryptase levels over 7 to 11 years in 2 patients with sporadic DCM and decrease in cutaneous involvement (patient 1 and 2 of Table III; solid squares) and in 2 patients with familial DCM and no decrease in cutaneous involvement (patients 9 and 10 of Table III, open squares). Journal of Allergy and Clinical Immunology 2015 136, 1581-1590.e3DOI: (10.1016/j.jaci.2015.05.034) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 3 Disease duration differs between cutaneous subforms and correlates with tryptase levels. A, Disease duration was recorded in patients with different cutaneous subforms. Data are presented as box plots (minimum to maximum) with medians (left) and scatter dot plots with means (right). B, Number of patients grouped according to disease duration. C-E, Tryptase levels (y-axis) positively correlate with disease duration (x-axis) in all patients with childhood-onset mastocytosis (Fig 3, C), all patients with MPCM (Fig 3, D), and patients with MPCM-small lesions (Fig 2, E). Journal of Allergy and Clinical Immunology 2015 136, 1581-1590.e3DOI: (10.1016/j.jaci.2015.05.034) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 4 Spontaneous regression of skin lesions occurs more frequently in patients with MPCM-large lesions. A, Number of patients with either a decrease (white bars) or no decrease (dotted bars) of skin lesions. B, Representative patient with MPCM-large lesions (upper panel) showing spontaneous improvement of skin lesions within 5 years (left, year 2005; middle, year 2008; right, year 2010) compared with a representative patient with MPCM-small lesions (lower panel) with no decrease in skin lesions over 9 years (left, year 2004; middle, year 2010; right, year 2013). C, Number of patients with MPCM-large lesions grouped according to disease duration from diagnosis until the start of decrease in skin lesions. Journal of Allergy and Clinical Immunology 2015 136, 1581-1590.e3DOI: (10.1016/j.jaci.2015.05.034) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 Representative pictures of dermatologic terms used within the text. Journal of Allergy and Clinical Immunology 2015 136, 1581-1590.e3DOI: (10.1016/j.jaci.2015.05.034) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E2 Number of patients grouped according to disease onset. Journal of Allergy and Clinical Immunology 2015 136, 1581-1590.e3DOI: (10.1016/j.jaci.2015.05.034) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E3 Mutational and histologic characteristics in a subset of patients participating in this study. large, MPCM-large lesions; n.a., not applicable; n.d., not detected; other, MPCM-other lesions; small, MPCM-small lesion; wt, wild type. The blue-red color scale illustrates hierarchic comparisons: blue, minimum; red, maximum. MC density is as follows: 1, low; 2, middle; 3, high. MC localization is as follows: pa, periadnexal (around hair follicles/sweat glands); pb, papillary body; pv, perivascular; rb, reticular body. MC infiltration pattern is as follows: a, perivascular in the papillary body and upper reticular dermis; b, sheet-like within the papillary body and upper reticular dermis; c, interstitial; d, nodular. Journal of Allergy and Clinical Immunology 2015 136, 1581-1590.e3DOI: (10.1016/j.jaci.2015.05.034) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions