Cse 18 A 63-yer-old womn from Pthumthni. Chief complint: Abnorml skin color extremities for 20 yers. F les Phs. res Sk top sm fee Hi leg Present illness: The ptient first noticed drkening of her skin the dors of feet pproximtely 20 yers go. The pigmentti slowly spred upwrd to her shins, while her hnds nd forerms were lso grdully involved. Over the lst few yers, she developed numerous smll white spots top of the drk ptches. She never experienced ny itch, pin, tenderness, redness, bruising, bleeding, or swelling over the skin lesis, except moderte itching loclized to the dors of her nkles nd feet, which she often scrtched with her fingernils or other hrd objects to find some relief. She denies history of prolged fever, weight loss, joint pin or swelling, be pin, ftigue, bnorml bleeding, or sensitivity to sunlight. Pst history: She hs no other medicl cditis nd denies history of ny drug or chemicl use, either orlly or topiclly. ms he M es. me ss, Ept Di et, Tr cts creht pg, Pres Co 108
es hs. es k op m ee i eg Fmily history: Ne of other fmily members hve similr skin lesis. Physicl exminti: Her vitl signs, HEENT, crdiovsculr, respirtory, nd bdominl exmintis were norml. Skin exminti: Ill-defined, brownish, hyperkertotic plques topped with multiple well-defined hypopigmented mcules nd smll ptches the extensor spect of forerms, hnds, legs, nd feet. Histopthology (S12-17717A, hypopigmented mcule right leg; S12-17717B, brownish plque right leg) ms he M es. ss, e Ept et, i cts r reht pg, res o Mild epiderml hyperplsi with bundnt epiderml melnin Multiple, smll, eosinophilic globules dmixed with some melnophges within the ppillry dermis Ech globule highlighted by crystl violet nd cgo red stining Dignosis: Dyschromic myloidosis Tretment: 10% ure crem pplied twice dily, 10% lctic cid crem pplied twice dily, nd 0.05% clobetsol propite crem pplied twice dily to itchy res Presenter: Kunlwt Thdnip, M.D. Csultnt: Chnitwn Wichychkorn, M.D. Crystl violet 109
Discussi Dyschromtoses re group of pigmentry disorders chrcterized cliniclly by the combinti of hypopigmented nd hyperpigmented lesis, which cn be presentti of genodermtoses, inflmmtory skin diseses, infectis, drug nd chemicl use, or nutritil disorders. The mjor cuses of dyschromtoses with in childhood re genodermtoses. In dults, the cuses of dyschromtosis, s detiled in figure 1, my comprise exposure to drugs nd chemicls, utoimmune diseses, infectis, nd other miscellneous cditis, including dyschromic myloidosis which my hve n in either childhood or dulthood. 1, 2 Skin biopsy my help in the dignosis of some of these cditis. Figure 1: Approch to dult- dyschromtoses. 2 Amyloidosis is group of severl diseses chrcterized by bnorml extrcellulr depositi of fibrillr proteinceous substnce, known s myloid, of which over 20 types hve been identified. Loclized cutneous myloidosis my be clssified into 2 typ wi ers PUnd deof inv nd noof poin Ovy no es, imic mor dyof m b to de h m fre Mo ret dis be ep lm c b fin mby whus men 2 110
yp ers i Und eof nv nd oof oin vy es, o mic mor yof m o e m re o et is e p lm in n mby hus men 2 types, primry nd secdry. The ltter is found in ssociti with skin tumors, inflmmtory disese (e.g. porokertosis), nd PUVA tretment. 3 Primry cutneous myloidosis refers to the depositi of myloid in previously norml skin without systemic involvement. It csists of 3 clssic forms, i.e. mculr, lichen, nd nodulr, 4 s well s mny other rre vrints, e.g. dyschromic, poikiloderm-like, bullous, vitiliginous, nd noscrl forms. 5 Overlps mg different forms hve lso been reported. 5, 6 In nodulr myloidosis, the myloid deposits re derived from immunoglobulin light chins, similr to primry systemic myloidosis observed in multiple myelom nd other plsm cell dyscrsis. By ctrst, the myloid of mculr nd lichen myloidosis is minly derived from cytokertin 5, which comes from bsl kertinocytes. 3 Our ptient is dignosed with dyschromic myloidosis, due to the dyschromtosis s the clinicl presentti nd the myloid deposit in the ppillry dermis, s shown by histopthology. There hve been number of published cses of primry cutneous myloidosis in ssociti with dyschromtosis. The most frequently reported form is myloidosis cutis dyschromic. Morishim first defined the cditi in 1970 s hving (i) dotted, reticulr hyperpigmentti with hypopigmented mcules distributed over nerly ll of the body, (ii) no or little itch, (iii) before puberty, nd (iv) focl myloid depositi under the epidermis. 7 Subsequently, there hve been severl reports of lmost exclusively Asin ptients, some of whom re fmilil cses, 8-10 cforming to these criteri. Nevertheless, the genetic bsis of myloidosis cutis dyschromic is still unknown. 7 The clinicl findings of our ptient re similr to those cses, except for the in dulthood. A few cses of dult- dyschromic myloidosis hve lso been reported. 11 Therefore, it is not cler whether the peditric- nd dult- forms of dyschromic myloidosis represent the sme disese spectrum or different 111
entities. However, these cditis seem to be benign, without extrcutneous involvement, s the myloid deposits re epiderml-derived nd not ssocited with systemic myloidosis. Tretment of primry cutneous myloidosis is often unstisfctory. Topicl corticosteroids, kertolytics, dimethyl sulfoxide, cpsicin, nd crb dioxide lser hve been tried with vrible success. 10 Only topicl tretment ws given to our ptient. 9, 12 Orl citretin hve been reported to be effective in some cses. Thus, it my be csidered in the future if the current tretment gives unstisfctory results. ut re en hyl th nt. 12 nt References 1. Vchirm V, Thdnip K, Chnprpph K. Infncy- nd childhood- dyschromtoses. Clin Exp Dermtol 2011;36:833-8, quiz 9. 2. Vchirm V, Thdnip K, Rttnkemkorn P. Adult- dyschromtoses. Clin Exp Dermtol 2012;37:97-103. 3. Fernndez-Flores A. Cutneous myloidosis: ccept review. Am J Dermtopthol 2012;34:1-14; quiz 5-7. 4. Brethnch SM. Amyloid nd myloidosis. J Am Acd Dermtol 1988;18:1-1 5. Chndrn NS, Goh BK, Lee SS, Goh CL. Cse of primry loclized cutneous myloidosis with proten clinicl mnifesttis: lichen, poikiloderm-like, dyschromic nd bullous vrints. J Dermtol 2011;38:1066-71. Cho TH, Lee MH. A cse of lichen myloidosus ccompnied by vesicles nd dyschromi. Clin Exp Dermtol 2008;33:291-3. 7. Qio J, Fng H, Yo H. Amyloidosis cutis dyschromic. Orphnet J Rre Dis 2012;7:95. 8. Chohkrn C, Wittychnypg S. Fmilil myloidosis cutis dyschromic: six cses from three fmilies. J Dermtol 2002;29:439-42. 9. Krdğ AS, Şimşek GG. Fmilil myloidosis cutis dyschromic. Turk J Med Sci 2010;40:151-4. 10. Grg T, Chnder R, Jbeen M, Brr M, Mittl K, Jin M et l. Amyloidosis cutis dyschromic: rre pigmentry disorder. J Cutn Pthol 2011;38:823-11. Ho MS, Ho J, Tn SH. Hypopigmented mculr myloidosis with or without hyperpigmentti. Clin Exp Dermtol 2009;34:e547-51. 12. Ozcn A, Senol M, Aydin NE, Krc S. Amyloidosis cutis dyschromic: cse treted with citretin. J Dermtol 2005;32:474-7. J ous ke, nd Dis utis ed sis out se 112
ut re en hyl th nt. 12 nt J ous ke, nd Dis utis ed sis out se 113