Aschwanden, Markus

Daikeler, Thomas

Halter, Jörg P.

Jaeger, Kurt A.

Staub, Daniel

Tichelli, André

Tyndall, Alan

Walker, Ulrich A.

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Letters to the EditorRheumatology 2011;50:11681169doi:10.1093/rheumatology/ker125Advance Access publication 30 March 2011Nail fold capillaroscopy differs widely betweensystemic sclerosis and chronic graft vs hostdisease of the skinSIR, Skin fibrosis is a common feature in patients with sclero-derma (SSc) or chronic graft vs host disease (cGvHD) afterhaemopoietic stem cell transplantation (HCST) with a rele-vant impact on quality of life. Despite the similarities inskin fibrosis, both diseases show several differences intheir clinical manifestation.Nail fold capillaroscopy (NFC) is a simple and eleganttool to study microvascular architecture non-invasively.This well-established method is widely used in the diag-nosis and follow-up of SSc patients [1]. In SSc, NFCreveals a severe disarrangement of capillaries, giant capil-laries, atypical forms, microhaemorrhages and capillaryrarefaction up to avascular fields differing quantitativelyin the time course and depending on disease activityand therapy (Fig. 1). These morphological changes aredescribed as scleroderma pattern. Very often, but not ex-clusively [2], this pattern is seen in patients with SSc. Itreflects the microvascular aetiology of the disease. Therole of vascular pathology in cGvHD is less well studiedand much more controversial [3, 4]. The involvement ofnail-fold capillaries by means of standard NFC has not yetbeen investigated.Here, we report on standard NFC findings in 11 patientswith sclerodermoid cGvHD. Six men and five women witha median age of 43 years (range 2465 years) underwentallogeneic HSCT from a related (6) or unrelated (5) donorbecause of acute lymphoblastic (3) or acute myeloid (2) orchronic myeloid leukaemia (2), lymphoma (3) or pure whitecell aplasia (1). All but one had received peripheral bloodstem cells. Median time since HSCT was 72 months(range 28120 months), median time interval from diagno-sis of cGvHD to NFC was 30 months (range 10118 months).The severity of cGvHD according to the National Institutesof Health scale [5] was severe in 10 and moderate in 1patient. In cGvHD assessment of skin activity, the medianbody surface areas affected by erythematous rash, teth-ered and non-tethered sclerosis/fasciitis were 5% (range010%), 5% (040%) and 15% (563%), respectively. Allbut one patient were treated with calcineurin inhibitors orsirolimus. Two were still under treatment with CSs, threepatients had imatinib, one nilotinib and one was treatedwith rituximab 5 months before NFC. All patients hadstable disease at the time of examination. All patients under-went NFC (Wild M8 binocular microscope, Heerbrugg,Switzerland/JVC KY-F55B Videocamera, Japan) of fingersIIV on both hands after they spent at least 10 min in a tem-perate room (23C).Nine patients showed excellent transparency of theskin, whereas in two patients visualization of capillarieswas adequate. In general, a uniform pattern with a regularappearance concerning number and arrangement ofcapillaries was seen. Slightly tortuous capillaries wereseen in two and infrequent minor capillary haemorrhageswere seen in three individuals only. One patient showedsome isolated minimally enlarged capillaries. No giantcapillaries could be documented.Our observation of a nearly normal capillaroscopic pat-tern in patients with cGvHD is in contrast to the severecapillary pathologies in 18 patients with sclerodermoidcGvHD reported by Akay et al. [6]. There are several dif-ferences between the patient characteristics described byAkay et al. and our group, which may explain the differentfindings. The most important difference is that RP—whichis an early and frequent clinical feature in SSc patients—was present in 17% of Akay’s patients compared withnone in our series. Furthermore, none of their patientswas under immunosuppressive treatment at the time ofFIG. 1 (AC) Representative images of individuals withcGvHD after HSCT. (A) A 51-year-old man 8 years afterHSCT because of chronic myeloid leukaemia. (B) A23-year-old woman 3 years after HSCT because ofHodgkin’s disease. (C) A 38year-old woman 7 yearspost-HSCT due to acute myeloid leukaemia. All fingersshow a normal aspect with the exception of (C), featuringsparse capillary bleeds (*). (D) A 31-year-old man 1 yearafter the diagnosis of SSc, showing capillary bleeds, ca-pillary thromboses, giant capillaries and avascular fieldsas a typical sclerodermiform pattern, is shown forcomparison.! The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.comRHEUMATOLOGYLETTERSNFC and median duration of cGvHD before NFC wasmuch shorter in their patient group (3 vs 30 months).Fleming et al. [4] noted areas of microvascular prolifer-ations in some cGvHD biopsies, especially in those witha lichenoid histological picture. We do not know whetherthe NFC abnormalities described by Akay et al. were dif-ferent between patients with lichenoid changes (nine inAkay’s report) or not, but we did not observe any NFCabnormalities in our patients with lichenoid changes.Furthermore, these authors used dermatoscopy—amethod that showed inferior reliability in patients withSSc compared with standard NFC [7]. This fact mayhave additionally contributed to the different interpretationof the capillary morphology. On the other hand, our find-ings support those of Fleming et al. [4] who could dem-onstrate by histopathology only slight alterations insuperficial dermal microvessels in cGvHD in contrast topatients with SSc, in whom capillaries were significantlyreduced in number, showed fewer canonical endothelialmarkers and no microvascular endothelial proliferation.In summary, our results further support the hypothesisthat cGvHD of the skin and SSc are two similar-lookingphenomena of skin fibrosis, but with a different patho-physiology, and therefore may need different therapeuticapproaches. Thus, cGvHD patients and pathological find-ings on NFC may represent a different group and shouldbe carefully evaluated for new-onset collagenosis.Rheumatology key message. Patients with cGvHD of the skin after HCST presentnormal nail-fold capillary findings.Disclosure statement: The authors have declared noconflicts of interest.Markus Aschwanden1,*, Jo¨rg P. Halter2,*,Ulrich A. Walker3, Daniel Staub1, Andre´ Tichelli2,Thomas Daikeler3, Kurt A. Jaeger1 and Alan Tyndall31Department of Angiology, 2Department of Haematology and3Department of Rheumatology, University Hospital Basel,Basel, SwitzerlandAccepted 8 February 2011Correspondence to: Markus Aschwanden, Department ofAngiology, University Hospital Basel, Petersgraben 4,CH-4031 Basel, Switzerland. E-mail: aschwandenm@uhbs.ch*Markus Aschwanden and Jo¨rg P. Halter contributed equal-ly to this work.References1 Aschwanden M, Daikeler T, Jaeger KA et al. Rapidimprovement of nailfold capillaroscopy after intenseimmunosuppression for systemic sclerosis and mixedconnective tissue disease. Ann Rheum Dis 2008;67:10579.2 Gutierrez M, De Angelis R, Bertolazzi C, Grassi W.Capillaroscopic scleroderma-like pattern in patientswithout connective tissue disorders. Rheumatology 2010;49:19946.3 Biedermann BC, Sahner S, Gregor M et al. Endothelialinjury mediated by cytotoxic T lymphocytes and loss ofmicrovessels in chronic graft versus host disease.Lancet 2002;359:207883.4 Fleming JN, Shulman HM, Nash AR et al. Cutaneouschronic graft-versus-host disease does not have theabnormal endothelial phenotype or vascular rarefactioncharacteristic of systemic sclerosis. PLoS One 2009;4:e6203.5 Filipovich AH, Weisdorf D, Pavletic S et al. NationalInstitutes of Health consensus development projecton criteria for clinical trials in chronic graft-versus-host disease: I. Diagnosis and staging workinggroup report. Biol Blood Marrow Transplant 2005;11:94556.6 Akay BN, Sanli H, Topcuoglu P, Arat M, Akyol A.Nailfold capillary abnormalities are prevalent insclerodermoid graft-versus-host disease and readilydetected with dermatoscopy. Br J Dermatol 2010;162:107682.7 Baron M, Bell M, Bookman A et al. Office capillaroscopy insystemic sclerosis. Clin Rheumatol 2007;26:126874.Rheumatology 2011;50:11691171doi:10.1093/rheumatology/ker140Advance Access publication 10 April 2011Need for a consensus on the methods by which tomeasure joint mobility and the definition of normsfor hypermobility that reflect age, gender andethnic-dependent variation: is revision of criteria forjoint hypermobility syndrome and EhlersDanlossyndrome hypermobility type indicated?SIR, Joint mobility is a continuous trait that varies with jointlocation and is strongly influenced by age, gender andethnic origin. Variation in joint mobility probably beginsin utero and can be expressed, often most dramatically,in individuals with heritable syndromes, such as forms ofEhlersDanlos syndrome (EDS) and various conditions inwhich joint contracture is a conspicuous feature. In indi-viduals with these disorders, the diagnosis generally de-pends on the sum of the different features of thecondition, in a sense the gestalt of the presentation. Ingeneral, the ability to formulate these diagnoses dependsin large part on the experience of the clinician and aware-ness of the classical descriptions of the syndromes.Individuals with syndromic forms of abnormalities injoint mobility tend to fall into the diagnostic realm of thegeneticist, paediatrician, rheumatologist or other special-ists, depending on the national preferences for referral.Their clinical care is often assumed by rheumatologistsand physical therapists. A second and much largergroup of individuals occupies the attention of bothrheumatologists and geneticists—those who have jointmobility that seems out of the usual range (usuallyincreased) at some point in their lives. They may haveacute or chronic subluxation, may have joint-related painthat seems out of proportion to clinical signs and moreoften than not are female. In part, because of thewww.rheumatology.oxfordjournals.org 1169Letters to the Editor

Nail fold capillaroscopy differs widely between systemic sclerosis and chronic graft vs host disease of the skin

Nail fold capillaroscopy differs widely between systemic sclerosis and chronic graft vs host disease of the skin

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