Are Hyperlinear Palms and Dry Skin Signs of a Concomitant Autosomal Lchthyosis Vulgaris in Atopic Dermatitis?

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Acta Derm Venereol (Stockh) I 989; Suppl 144: 143-145 Are Hyperlinear Palms and Dry Skin Signs of a Concomitant Autosomal lchthyosis Vulgaris in Atopic Dermatitis?

MANJGE FARTASCH, THOMAS L. DIEPGEN and OTTO PAUL HORNSTEIN Departmenl af Dermalology, Uniuersily of Erlangen, F.R.G.

In 30 % to 40 % of cases atopic dermatitis (AD) is ton-Lamprecht ( 12) demonstrated a severe disturb believed to be associated with autosomal dominant ance of kcratohyalin (KH) synthesis resulting in fewer ichthyosis vulgaris (ADI). The diagnosis of ADI can be and abnorma! KH-granules. This abnorma! KH is proved by the ultrastructural demonstration of a defec present in all AD! patients also in clinically unaffect tive keratohyalin (KH) synthesis, resulting in minute ed skin (12-14). Thus, the defective KH of ADI can granules of crumbly appearence in only one layer of be uscd as a genetic marker to control the presence of granular cells. To investigate the suggested frequent association of ADI with AD, ultrastructural examina the ADI gene( I3). tion of dry skin of 49 AD patients was performed. Only In order to investigate the suggested frequent asso in 2 patients abnormal KH was demonstrated by elec ciation ultrastructural analysis of AD patients was tron microscopy. 17 patients, including the 2 patients performed. with abnorma! KH, showed hyperlinear palms. The present study shows that hyperlinear palms and dry PATIENTS AND METHODS skin are in most cases a phenotypic marker of AD alone and not a sign of concomitant ADI. A histologi Noneczematous bu! dry skin of 49 atopic patients (31 males. cally one-layered or absent stratum granulosum may 18 f'emales)aged 15-36 years was invcstigated using Iight and electron microscopy. The diagnosis of AD was established occur in thc dry skin of patients with only AD and does according to Hanilin and Rajkas (I). Further requirements not indicatc a manifestation of concomitant ADI in all for inclusion were a history of persistent dry skin and cases. Key words: Ultrastructural analysis; Atopic der Lhe absence of eczema on thc lateral aspcc1 of the bu nock. All matitis; lchthyosis. patients were cxamined for the presence of hyperliner palms Dr med. M. Fartasch. Department of Dermatology, (i.e., dccp linear grooves crossing perpendicular to thc thenar University of Erlangen, Hanmannstr. 14, D-8520 Er and/or hypothcnar emincnccs ( 15)) and soles. langen, West-Germany. Punch biopsy specimens (4 mm) were taken from the skin ofthe uppcr outer quadrant ofthe buttock and fixed in 2.5% glutaraldehyde and postfixed with osmium tetraoxide. The Atopic dermatitis (AD) and autosomal dominant samples were then dehydrated in a gradcd series of ethanol ichthyosis (ADI) is believed to be frcquently associat and thcn embedded in Epon 812. Semithin sections of 1-2 µm were cut and stained with I% methylene blue and cxam ed. The incidence of ichthyosis in atopic dermatitis ined by light rnicroscopy. Ultrathin scctions were stained in ranges widely, from 2% to 40%, according to differ uranyl acctatc plus lead citrate prior to the examination in a ent studies (1-6). Some atopic features are seen in at JEOL 100 CX transmission electron microscopc. least 50% of patients with AD! (7-9). Recent studies (2, 4-6) concluded that in 30% to 40% of the cases RESULTS AD is associated with AD!. In these studies determi nation of the percentage of patients with both AD and Light microscopic examination of semithin sections ADI was based on clinical signs such as hyperlinear showcd a normal thickness (I 6) of the granular laycr palms.keratosis pilaris and dry skin, as well as on thc histological featurcs of a reduced granular layer thick Table I. Light microscopical examination ness(2, 4-6. I 0). Depending on the time of year when the patients were examined. clinical features of ich Str. granulosum Number of patients thyosis vulgaris may be inapparent, but palmar hyper (layers) 11=49 linearity is usually a persistent finding. This feature has becn described in one-third to one-halfofpatients 2-3 21 17 with AD(I0.11). 1-2 0--1 Il Ultrastructural analyses of ADI performed by An-

Autosoma/ ichthyosis uu/garis 145 influence of AD on keratinization and KH synthesis. nanl and sex linkcd ichthyosis in an English population. This is supported also by the observation that bonc Br Med J 1966; I: 947-950. I 0. Mevorah B, Marazzi A. Frenk E. The prcvalence of ac marrow transplantation in children with Wiskott-Al centuated palmoplantar markings and keratosis pilaris in drich syndrome caused a simultaneous disappearance atopic derrnatitis. amosomal dominant ich1hyosis and of AD and xeroxis associated wilh skin lesions ( 18). control dermatological pa1ien1s. Br J Dcrmaiol 1985: Thus. dry skin in AD appears, at leasl partly, lo be 112: 679-685, related lo the immune dysfunction and is not due to a 11. Hoyer H. Agda! N. Munkvad M. Palmar hyperlinearity in atopic dermatitis. Acia Denn Vcnereol (Stockholm) primary defect of kcratinization. 1982: 62; 346--347, 12. Anton-Lamprcch1 I. Zur Ultrastruktur hereditärer Vcr hornungsstörungen. 111. Autosomal-dominantc lchthyo sis vulgaris. Arch Dermatol Forsch 1973; 248: 149-172. 13. Bernd1-Schumann C. Ncurodermitis atopica und au10s0- REFERENCES mal-dominan1e lchthyosis vulgaris: eine 11ltras1rukturelle I. Hanifin JM. Rajka G. Diagnostic features of atopic der S1udie zur genetischcn Korrelation beider Dcrmatosen matitis. Ac1a Dem, Venereol (Swckholm) 1980: Suppl anhand eines ausgewähl1cn Patien1en-Kollek1ivs. Disser 92: 44-47. tation 1982, University of Heidelberg, Heidelberg. De 2. Leutgcb CH. Bandmann HJ. Breit R. Handlinienmuster, partment of Dennawlogy. lchthyosis vulgaris und Derma1i1is atopica. Arch Derma 14. Papst B. Yergleichende ul1ras1rukturelle Untersuchun lOI Forsch 1972; 244: 354-356. gen an bcfallener und nicht befallener Haut von Patien 3. Fartasch M, Hancke E, Anton-Lamprecht I. Ultrastruc ten mil autosomal-dominanter lch1hyosis vulgaris und tural study of thc occurrence of au1osomal dominan1 primärem S1rukturdefekt des Kcratohyalins. Dissertation ichthyosis vulgaris in atopic eczema. Arch Dermalol Res 1979, University of Heidelberg, Heidelberg, Depanmelll 1987;279: 270-272. of Dermatology. 4. Uehara M, Hayashi S. Hypcrlinear palms. Arch Derma- 15. Leung DJ\1, Rhodes AR. Geha SR. Atopic dcrmatitis. 101 I 981; 117:490-491. In: Fi1zpatrick TB, ed. Dennatology in general medicine. 5. Uehara M, Miyauchi H. The morphologic charac1eristics 3rd ed. New York: McGraw-Hill. 1987; Vol. I: of dry skin in atopic dcrmatitis. Arch Dermatol 1984: I 385-1408. 120: I 186-1190. 16. Erickson L, Major MC, Kahn U, Kahn G. The granular 6. Uehara M. Clinical and hiswlogical features of dry skin layer thickness in atopy and ichthyosis vulgaris. J lnvest in atopic dermatitis. Acta Dcrm Venereol (Stockh) 1985; Derma1ol 1970; 54: 11-12. Suppl I 14: 82-86. I 7. Finlay AY. Nicholls S, King CS, Marks R. The dry non 7. Frost P, van Scott EJ. lch1hyosiform dermatoses. Classi eczema1ous skin associated with atopic eczema. Br J fica1ion based 011 anatomic and biomctric observations. Derma10I 1980; 102: 249-256. Arch Dermatol 1966; 94: 113. 18. Saurat JH. Woodley D, Helfer N. Cutaneous symptoms 8. Rajka G. Atopic dermatitis. Saunders. London I 975. in primat)· immunodcficiencies. Curr Probl Derm l 985; 9. Wells RS. Kerr CB. Clinical featurcs of au1osomal-domi- 13; 50-9 I.