Cutaneous Collagenous Vasculopathy

Home , Microangiopathy

source: https://doi.org/10.7892/boris.76592 | downloaded: 30.9.2021 Helmut Beltraminelli b a University ofGenoa, Genoa , and University ofTurin, Turin , Italy ture. options availablewithasurvey of thelitera- our knowledgeaboutCCVand therapeutic of thewidespreadlesions.Weherereview in astrikingandalmostcompleteclearance never beenreportedforCCVsofar,resulted mized pulsedlight.Thisapproach,whichhas 595-nm/Nd:YAG 1,064-nmlaserandopti- limbs. ShewastreatedwithMultiplexPDL CCV involvingthetrunk,upperandlower tient presentingwithanextensiveformof in theupperdermis.Wereportanelderlypa- thickened wallscontaininghyalinematerial blood vesselswithflatendothelialcellsand logically bythepresenceofdilatedsmall cutaneous vasculaturecharacterizedhisto- is arareidiopathicmicroangiopathyofthe Cutaneous collagenous vasculopathy(CCV) Abstract Multiplex laser·Optimizedpulsedlight Cutaneous collagenousvasculopathy· Words Key Basso Davide Literature the and OptimizedPulsed Light withaReview of Treated withaCombination ofMultiplexLaser A Rare Form ofMicroangiopathy Successfully Cutaneous Collagenous Vasculopathy: www.karger.com/drm E-Mail [email protected]

Department ofDermatology,UniversityHospitalInselspitalBern, Bern , and Skinpulse Dermatology,LaserandBeautyCenters, Geneva , Switzerland;

a AlbertA.Ramelet d

Section ofDermatology,DepartmentMedicalSciences,

a, d ClaudiaBlazek available therapeuticoptions. and wereviewpreviouscasesof CCVand tial PDL595-nm/Nd:YAG1,064-nm laser, (OPL) combinedwithamultiplexsequen- the firsttimewithanoptimizedpulsedlight patients. Theseweresuccessfullytreatedfor skin lesionsandthequalityoflifeaffected considered tosignificantlyimproveboth gressive condition,vascularlasershouldbe is nocureforthisusuallychronicandpro- sult ofunknownfactors by veilcellsintheoutervesselwallsasare- due totheproductionofabnormalcollagen The perivascularfibrosisisthoughttobe asymptomatic butsometimesdisfiguring. upper limbs.Cutaneouslesionsareusually neous telangiectasiaonthetrunk,lowerand develops inmiddle-agedpatientswithcuta- cutaneous vasculature known origin,whichinvolvesthesuperficial (CCV) isararemicroangiopathyofun- DI 10.1159/000439126 DOI: 2016;232:107–111 Dermatology Case and Review Cutaneous collagenousvasculopathy Introduction

a LucaBorradori

a NathalieDietrich EMi simone.ribero E-Mail Via Cherasco 23,IT–10100Torino(Italy) Department of MedicalSciences,UniversityofTurin Section ofDermatology Simone Ribero, MD,PhD [1] [1] c

Section ofDermatology, MauriceAdatto . CCVtypically . Althoughthere

unito.it a

D.B., S.R., L.B.andM.A.contributedequally. lesions wereraised,slightlyhypertrophic rically onthelegs,armsandchest.Some spread telangiectasiasdistributedsymmet- and levocetirizine. procoumon, acetylsalicylicacid,oxazepam eralized itch.Thepatientwasonphen- and chronicrenalinsufficiencywithgen- ischemic heartdiseasewitharrhythmias surgery forovariancysticadenocarcinoma, markable. Themedicalhistoryrevealed and chest.Thefamilyhistorywasunre- subsequently spreadontothethighs,arms the ageof20yearsonlowerlegsand ( fig. 1 a). Thelesionshadfirstdevelopedby generalized asymptomatictelangiectasias

a, b Physical examinationshowedwide- A 77-year-oldwomanwasevaluatedfor t r o p e R e s a C

Published online:November4,2015 Accepted afterrevision:July31,2015 Received: April21,2015

Downloaded by: Universitätsbibliothek Bern 198.143.58.1 - 3/9/2016 1:54:59 PM as well as an OPL. The following parameters were used, as spot tests first, with the laser, on the hypertrophic lesions: 595-nm pulsed dye laser, a 7-mm spot at 8.5 J/cm 2 and a 10-ms pulse duration followed se- quentially by a 500-ms interval, then a pulse of 1,064-nm Nd:YAG laser at 55 J/ cm 2 and a 15-ms pulse duration (Cynergy Multiplex, Cynosure, Westford, Mass., USA). This was delivered in a single pass with no overlap. Forced chilled air was used for parallel cooling. For flat lesions, we used an OPL (Limelight Cutera, Bris- a b bane, Calif., USA) with a 520-nm filter, a 10 × 30 mm spot, fluence range of 16–18 J/cm2 , 8–9 ms of pulse width, with contact cooling and 2 perpendicular passes. The response of these tests gave a remarkable improvement. Therefore all lesions of the upper limbs and trunk were subsequently treated using the same parameters. Almost complete clearance of the telangiectasia on both arms and the chest was obtained after a total of 7 combined laser/intense pulsed light treatment sessions, performed every 4–8 weeks (fig. 1 b). c d

Discussion Fig. 1. a Diffuse acquired telangiectasias over the arms and chest. b Close-up view of the c right arm. Postintervention resolution of the telangiectasias over the arms and the chest. Our patient had clinical and histopath- d Postintervention close-up view of the right arm. ological features typically described as CCV, a distinctive primary cutaneous microangiopathy first described in 2000 [1, 2] . This entity is rare since only 25 cases have been described so far in the English literature (table 1 ). CCV has almost exclusively been reported in middle-aged subjects [3] , even though 1 pediatric case has been reported [4]. In our patient, cutaneous telangiectasia originally developed on the lower legs and progressed subsequently to the thighs, trunk and the upper extremities in accor- dance with data reported in the literature a b [5]. Usually lesions are almost invariably asymptomatic although some itching may Fig. 2. a Telangiectasia of dermal capillaries with hyalinized thickening of the vessel wall. occur [5] . Lesions remain then relatively b The hyaline material within the thick vessel wall is positive for collagen type IV. stable or may occasionally worsen during the summer months [2] . No mucosal involvement has been reported so far. Light microscopy studies are essential and more of a purple color, while some oth- IV was visible at immunohistochemistry for the diagnosis of CCV. This typically ers were completely flat and bright red. ( fig. 2 b). shows dilated small blood vessels in the su- There was no mucosal or nail involvement. The patient desired to improve the dis- perficial dermis and, more rarely, in the Light microscopy studies of a biopsy turbing cosmetic appearance of these ex- midreticular dermis. Sparse lymphocytes specimen obtained from the leg revealed tensive red-blue lesions, especially on the scattered around the vessel walls may be dilated superficial cutaneous vessels with arms and forearms. We proposed laser found. Electron microscopy studies dis- perivascular deposits of hyaline materi- treatment using a combination of a Multi- close dilated postcapillary venules with flat al (fig. 2 a). A staining with collagen type plex PDL 595-nm/Nd:YAG 1,064-nm laser endothelial cells and a thickened laminat-

108 Dermatology 2016;232:107–111 Basso/Ribero/Blazek/Dietrich/ DOI: 10.1159/000439126 Beltraminelli/Ramelet/Borradori/Adatto

Downloaded by: Universitätsbibliothek Bern 198.143.58.1 - 3/9/2016 1:54:59 PM Table 1. Survey of the literature: cases of CCV and their management

No. Authors Age, Sex Race Medical history Distribution Evolution Treatment years

1 Salama and 54 M Caucasian Depression Trunk, upper and lower 5 years No Rosenthal [1], limbs 2000

2 Davis et al. [6], 59 M Caucasian Diabetes mellitus, hypercholesterolemia, Forearm, chest, abdomen 7 months No 2008 hypertension 6 M Caucasian Diabetes mellitus, hypertension, psoriasis Thigh Unclear No 80 M Caucasian Atrial fibrillation, gastroesophageal reflux, venous Abdomen, thigh, back, Unclear No insufficiency hands

3 Kanitakis et al. 65 M Caucasian Hypertension, coronary infarction, prostatic Lower extremities, 5–6 years No [8], 2010 adenoma abdomen, buttocks, back

4 Monteagudo 68 M Caucasian Hypertension, hypercholesterolemia, prostatic Forearm, abdomen, 15 years No et al. [11], 2010 adenoma, hyperuricemia lower limbs

5 Perez et al. [7], 51 F Caucasian Hypothyroidism, psoriasis Trunk, neck, upper 16 years No 2010 and lower limbs, retroauricular area 71 F Caucasian Pituitary tumor, osteoporosis Feet, knees, upper limbs, 7 years No chest, cheeks

6 Lloyd et al. [4], 16 F Caucasian Mood disorders Trunk, legs 3 years No 2011

7 González 83 F Caucasian Atrial fibrillation, mitral valve disease, hepatitis C, Upper and lower limbs, >20 years No Fernández hypertension abdomen et al. [3], 2012 74 F Caucasian Discoid lupus, venous insufficiency Lower limbs No

8 Echeverría 42 F Caucasian – Upper and lower limbs, 7 years PDL, et al. [14], 2012 abdomen successful

9 Burdick et al. 68 M Unknown Macular degeneration since childhood, Legs, abdomen Several weeks No [2], 2012 hypertension, hyperlipidemia, gastroesophageal reflux disease 59 M Unknown Diabetes mellitus, hypertension, hyperlipidemia, Arms, legs Several No osteoarthritis, depression months 70 M Unknown Hypertension, diabetes mellitus, Right leg 10 years No hypercholesterolemia 41 F Unknown Basal cell carcinoma, inactive hepatitis Arms Several years No

10 Bernard et al. 47 F Unknown Diabetes mellitus type 1, psoriasis, hypertension, Lower limbs, abdomen 1–2 years No [21], 2012 hypothyroidism

11 Salama et al. [5], 84 M Caucasian Myelodysplastic syndrome, diabetes mellitus, celiac Lower limbs and trunk 3 years No 2014 disease, tuberculosis, tachycardia

12 Salama [22], 68 F Unknown Mild chronic renal failure, Lower limbs Unknown – 2015 cryofibrinogenemia 85 F Unknown Diabetes mellitus Lower limbs 10 years No 50 F Unknown – Lower limbs and upper 2 years No limbs 69 F Unknown Diabetes mellitus Lower limbs and upper Unknown No limbs, abdomen 56 F Unknown – Lower limbs and upper 25 years No limbs, trunk 42 F Unknown Raynaud phenomenon Lower limbs >20 years No 73 M Unknown – Lower limbs and upper 2.5 years No limbs 13 Bardazzi et al. 57 F Caucasian Uveitis, hypertension Legs, abdomen, buttocks, 9 years No [23], 2014 hips, trunk, arms, malar region, fingers

14 This study, 77 F Caucasian Renal insufficiency, venous insufficiency, ovarian Upper and lower limbs, >20 years PDL/ 2015 adenocarcinoma, ischemic heart disease chest Nd:YAG

Laser for Cutaneous Collagenous Dermatology 2016;232:107–111 109 Vasculopathy DOI: 10.1159/000439126 Downloaded by: Universitätsbibliothek Bern 198.143.58.1 - 3/9/2016 1:54:59 PM ed basement membrane consisting of an to the production of abnormal collagen source. Our OPL, due to its large spot size amorphous eosinophilic hyaline material and reparative fibrosis [4] . The intake of (10 × 30 mm) allowed speed, comfort and [2, 3]. By immunohistochemistry, vascular drugs, encompassing corticosteroids, lithi- also very good cosmetic results in treating cells of endothelial origin are CD31 and um, tiotixene, interferon, isotretinoin, cal- large areas when compared with a laser CD34 positive. The amorphous eosino- cium channel blockers, antibiotics or anti- with a small spot size (7–10 mm). A few philic hyaline material is stained using an- depressants, has been thought to act as trig- cases of essential telangiectasias have been tibodies directed against laminins, fibro- ger [7, 10] . published using an intense pulsed light nectin and, characteristically, type IV col- Conditions such as diabetes mellitus or [17–20] . To our knowledge, our case is the lagen [5–7]. No amyloid deposits by Congo hypertension may further contribute. Most first describing a case of CCV successfully red staining are found. likely CCV represents a response pattern treated with an OPL combined with a Mul- CCV has to be differentiated from a triggered by multiple factors [2, 3, 11] . tiplex laser within the same session. number of other vascular disorders pre- The therapy of CCV is challenging. If In conclusion, we here describe the first senting with cutaneous telangiectasias, in- the patient wants a cosmetic improvement, successful case of CCV treated with an OPL cluding benign hereditary telangiectasia, lasers and other light sources represent the combined with a multiplex sequential PDL angioma serpiginosum and telangiectasia first therapeutic option. The latter are 595-nm/Nd:YAG 1,064-nm laser within macularis eruptiva perstans [3] . General- working according to the principle of selec- the same session. This approach led to an ized essential telangiectasia (GET) [8] is tive photothermolysis [12] . When treating impressive aesthetical improvement de- another benign condition that should be vascular lesions, the primary target is oxy- spite the very large affected surface. Even if differentiated from CCV. At variance with hemoglobin [13]. Two cases of CCV have a causal treatment of this difficult skin dis- CCV, GET is more common in women and been treated by a pulsed dye laser [14] . We ease has not already been discovered, the can rarely also involve the oral mucosa and opted for a combination of Multiplex PDL/ combination approach used here seems conjunctiva. In contrast to CCV, in GET YAG laser on thicker, purple lesions, to- very effective. there are dilated vessel walls, which consist gether with an OPL on the flatter, redder of thickened endothelial cells, but without ones. The advantages of this combination any deposition of amorphous hyaline ma- of energy-based devices during the same Acknowledgment terial [2, 9] . session are: (a) the Multiplex laser, which The question remains whether the pe- combines PDL at 595 nm followed by YAG The authors kindly acknowledge the culiar histopathological findings of CCV at 1,064 nm, is ideal for treating hypertro- support of Dr. Nathalie Irla and Dr. Ivan are sufficient to distinguish CCV from phic vascular lesions; this specific device Hegyi for their help in the evaluation of the GET as a distinct disease or whether both has already been used successfully in treat- patient. conditions represent different faces of a ing various vascular conditions, with better single disease. We included in the study vessel clearance than with a single pulsed only the certified CCV. laser [15]; (b) the OPL is an evolution of Disclosure Statement The mechanisms leading to the devel- the intense pulsed light, developed in 1992 opment of CCV remain unknown. Some [16], as it brings true square pulse emis- The authors have no conflicts of interest authors have discussed a nonimmunologi- sion. This peculiarity gives a very precise to disclose. cally related injury of the endothelial cells control of delivered fluencies, similar to by unknown factors, which ultimately lead a laser, even being a noncoherent light

References

1 Salama S, Rosenthal D: Cutaneous collage- 5 Salama S, Chorneyko K, Belovic B: Cutaneous 10 Requena L, Sangueza OP: Cutaneous vascular nous vasculopathy with generalized telangi- collagenous vasculopathy associated with in- anomalies. II. Hamartomas, malformation ectasia: an immunohistochemical and ultra- travascular occlusive fibrin thrombi. J Cutan and dilatation of preexisting vessels. J Am

structural study. J Cutan Pathol 2000; 27: Pathol 2014; 41: 386–393. Acad Dermatol 1997; 37: 523–549. 40–48. 6 Davis TL, Mandal RV, Bevona C, et al: Col- 11 Monteagudo B, Perez-Valcarcel J, Ramirez- 2 Burdick LM, Losher S, Somach SC, et al: Cu- lagenous vasculopathy: a report of three cases. Santos A, et al: Cutaneous collagenous vascu-

taneous collagenous vasculopathy: a rare cu- J Cutan Pathol 2008; 35: 967. lopathy: a case report and review of the litera-

taneous microangiopathy. J Cutan Pathol 7 Perez A, Wain ME, Robson A, et al: Cutane- ture. Actas Dermosifiliogr 2010; 101: 444.

2012; 39: 741–746. ous collagenous vasculopathy with general- 12 Anderson RR, Parrish JA: Selective photo- 3 González Fernández D, Gómez Bernal S, et ized telangiectasia in two female patients. J thermolysis: precise microsurgery by selective

al: Cutaneous collagenous vasculopathy: de- Am Acad Dermatol 2010; 63: 882. absorption of pulsed radiation. Science 1983;

scription of two new cases in elderly women 8 Kanitakis J, Faisant M, Wagschal D, et al: Cu- 220: 524–527. and review of the literature. Dermatology taneous collagenous vasculopathy: ultrastruc- 13 Baumler W, Vural E, Landthaler M, et al: The

2012; 225: 1–8. tural and immunohistochemical study of a effects of intense pulsed light (IPL) on blood

4 Lloyd BM, Pruden SJ 2nd, Lind AC, et al: Cu- new case. Am J Clin Dermatol 2010; 11: 63. vessels investigated by mathematical model-

taneous collagenous vasculopathy: report of 9 Becket SW: Generalized telangiectasia: clini- ing. Lasers Surg Med 2007; 39: 132–139. the first pediatric case. Pediatr Dermatol cal study with special consideration of the eti-

2011; 28: 598. ology and pathology. Arch Dermatol 1926;

14: 387–426.

110 Dermatology 2016;232:107–111 Basso/Ribero/Blazek/Dietrich/ DOI: 10.1159/000439126 Beltraminelli/Ramelet/Borradori/Adatto

Downloaded by: Universitätsbibliothek Bern 198.143.58.1 - 3/9/2016 1:54:59 PM 14 Echeverría B, Sanmartín O, Botella-Estrada R, 17 Babilas P, Schreml S, Szeimies RM, et al: In- 21 Bernard S, Cawet B, Theate Y, et al: Cutane- et al: Cutaneous collagenous vasculopathy tense pulsed light (IPL): a review. Lasers Surg ous collagenous vasculopathy: a rare cause of

successfully treated with pulsed dye laser. Int Med 2010; 42: 93–104. generalized telangiectasia. Ann Dermatol Ve-

J Dermatol 2012; 51: 1359–1362. 18 Fernández-Torres R, del Pozo J, de la Torre C, nereol 2012; 139: 381–386. 15 Li G, Sun J, Shao X, et al: The effects of 595- et al: Generalized essential telangiectasia: a re- 22 Salama SS: Cutaneous collagenous vasculopa- and 1,064-nm lasers on rooster comb blood port of three cases treated using an intense thy: a new case series with clinicopathologic vessels using dual-wavelength and multipulse pulsed light system. Actas Dermosifiliogr and ultrastructural correlation, literature re-

techniques. Dermatol Surg 2011; 37: 1473– 2010; 101: 192–193. view, and insight into the pathogenesis. Am J

1479. 19 Raulin C, Weiss RA, Schönermark MP: Treat- Dermatopathol 2015; 37: 368–375. 16 Wat H, Wu DC, Rao J, et al: Application of ment of essential telangiectasias with an in- 23 Bardazzi F, Virdi A, Odorici G, et al: Cuta- intense pulsed light in the treatment of der- tense pulsed light source (PhotoDerm VL). neous collagenous vasculopathy: report of a

matologic disease: a systematic review. Der- Dermatol Surg 1997; 23: 941–945. case. Clin Exp Dermatol 2014; 39: 228–230.

matol Surg 2014; 40: 359–377. 20 Wenzel SM, Hohenleutner U, Landthaler M: Progressive disseminated essential telangiectasia and erythrosis interfollicularis colli as examples for successful treatment with a

high-intensity flashlamp. Dermatology 2008;

217: 286–290.

Laser for Cutaneous Collagenous Dermatology 2016;232:107–111 111 Vasculopathy DOI: 10.1159/000439126 Downloaded by: Universitätsbibliothek Bern 198.143.58.1 - 3/9/2016 1:54:59 PM