Romiti et al (2) first proposed papulolinear collagenomaas a variant of collagenoma within the umbrella ofconnective tissue nevi. In the original description,papular, and linear lesions occurred on the dorsal handand fingers in a 45-year-old Brazilian woman. A secondcase was reported by Girard et al (3) in a 3-year-old girl.The lesions occurred on the lower back and werearranged in a linear array which followed Blaschko’slines. They proposed it as a new variant of isolatedhamartoma of collagen type.

In our case, the individual lesions were similar to thepreviously reported cases but differed by the presenceof arborizing pattern in addition to the papulolineardistribution. To us, the distribution showed a resem-blance more with a tear in the skin rather than a Blas-chkoid distribution. Papulolinear collagenoma may bean underdiagnosed entity and this peculiar arborizingarrangement of the lesion may be another clinicalpresentation.

REFERENCES

1. Uitto J, Santa CruzDJ, Eisen AZ. Connective tissue nevi ofthe skin. J Am Acad Dermatol 1980;3:441–461.

2. RomitiR,RomitiN.Papulolinear collagenoma. JAmAcadDermatol 2004;50:797–798.

3. Girard C, Bessis D. Papulolinear collagenoma. J Am AcadDermatol 2006;54(Suppl. 5):S240.

LI-KAI LO, M.D.*TSENG-FANG TSAI, M.D.�YU-FU CHEN, M.D.*CHI-MING HUNG, M.D.*WANG-CHENGKO, M.D.**Department of Dermatology, Show Chwan MemorialHospital, Changhua, Taiwan, �Department of Pediatrics,National Taiwan University Hospital

PRIMARY CUTANEOUS T-CELL LYMPHOMA

FOLLOWING ORGAN TRANSPLANTATION IN

A 16-YEAR-OLD BOY

Abstract: Organ transplant recipients on immuno-suppressant therapy are at risk of developing lympho-proliferative disorders. Although rare, there have beenreported cases of primary cutaneous T-cell lymphomaoccurring in this population. We present a pediatricpatient who developed cutaneous T-cell lymphoma withmycosis fungoides histology after liver transplantation.

Organ transplant recipients on long-term immunosup-pression are predisposed to developing lymphoprolifer-ative disorders (1). There have been 23 reported cases ofposttransplant cutaneous T-cell lymphoma (CTCL) (2).Onlyone reported caseoccurred inapediatric patient (3).We present a boy who had a liver transplantation at age11 secondary to primary sclerosing cholangitis and sub-sequently (4 yrs) developed mycosis fungoides (MF).Our patient is the first reported pediatric case of post-transplant MF.

He was maintained on immunosuppressive therapythat included sirolimus, tacrolimus, and prednisone. Attheageof 15,hedevelopeda5 · 3 cmnonpruritic plaque(Fig. 1) that expanded in size over the next 6 months.Aninitial punch biopsy demonstrated spongiotic psoriasi-form dermatitis. Topical steroids were maximized with-out significant effect. A second punch biopsy 7 monthslaterwas consistentwithMF(Fig. 2).T-cell clonalitywasdetected in the biopsy tissue; blood was negative forEpsteinBarr virusDNA.Chest andpelvicCT scanswereunremarkable. Over the following 5 months, he dem-onstrated hypopigmented patches of alopecia withpetechiae on his anterior lower legs and forearms.

The patient was treated with narrowband ultravioletB (NBUVB), irradiation light for 5 months. No changeswere made in his immunosuppression regimen. Sixmonths after initiating NBUVB therapy, the lesion onthe back resolved. At discontinuation of phototherapy,clobetasol ointment was used as needed. Eighteenmonths after cessation of phototherapy, he continues tohave multiple areas of macular nonscarring alopecia onthe lower legs with a few small petechiae. His CT scanscontinue to be negative for systemic involvement.

It is uncertain whether immunosuppresion directlyinfluences the development of MF. However, two obser-vations from previous reports suggest that immuno-suppression is important: (a) some cases show disease

Figure 1. Clinical presentation of initial plaque located onpatient’s back.

Address correspondence to Lai-Kai Lo, Department ofDermatology, Show Chwan Memorial Hospital, 542, Sec 1Chung-shan Road, Changhua 500, Taiwan, or e-mail: billionaire@yahoo.com.tw.

112 Pediatric Dermatology Vol. 26 No. 1 January ⁄February 2009

regression after reduction of immunosuppression; (b) theprognosisofposttransplantCTCLinadults isconsiderablypoorer thanCTCL in the general population (2,4).Amongthe reported adult cases, 14 of the 23 cases developedaggressive disease and died within 2 years of diagnosis (2).There were no clear variables that could predict whichpatients had an aggressive or an indolent disease.

Studies demonstrate 0% to 33% of immunocompe-tent children with MF experience disease progressionover a span of 5 to 10 years. There is some indicationthat the subgroups of patients with hypopigmented orpoikilodermatous lesions generally have a better prog-nosis. However, because there is still not enough data onfactors that can predict the development of disseminateddisease, lifelong follow-up is recommended.

Although our patient responded to phototherapy, hislong-termrisk fordiseaseprogressionstill remainsunclearin the setting of life-long immunosuppressive therapy.The patientmay also be at risk for developing skin canceras there is evidence of increased carcinoma risk in patientson immunosuppressants and certain forms of UV lighttherapy (5). It is important that we continue to monitorfor progression of disease and also for the development ofother skin lesions that may be due to complications of hisimmunosuppression and ⁄or phototherapy.

REFERENCES

1. Penn I.Cancers complicatingorgan transplantation.NEnglJ Med 1990;323:1767–1769.

2. Ravat FE, SpittleMF, Russell-Jones R. Primary cutaneousT-cell lymphoma occurring after organ transplantation.J Am Acad Dermatol 2006;54:668–674.

3. Katugampola RP, Finlay AY, Harper JI et al. Primarycutaneous CD30+ T-cell lymphoproliferative disorder fol-lowing cardiac transplantation in a 15-year-old boy withNetherton’s syndrome. Br J Dermatol 2005;153:1041–1046.

4. Lok C, Viseux V, Denoeux JP et al. Post-transplant cuta-neous T-cell lymphomas. Crit Rev Oncol Hematol2005;56:137–145.

5. Marcil I, Stern RS. Squamous-cell cancer of the skin inpatients given PUVA and ciclosporin: nested cohort cross-over study. Lancet 2001;358:1042–1045.

AHMADAMIN, B.S.CRAIG BURKHART, M.D.PAMELA GROBEN, M.D.DEAN S. MORRELL, M.D.Department of Dermatology, The University of NorthCarolina at Chapel Hill School of Medicine, Chapel Hill,North Carolina

A DISTINCT TYPE OF PALMOPLANTAR

KERATODERMA

Abstract: Palmoplantar keratodermas (PPK) are adiverse group of disorders. We report a boy with PPK,grayish-blue hyperkeratotic lesions on the lips and peri-oral area, opacities on the lower portions of the corneas,mutilationofhisrightauricleandmanyotherskinlesions.

CASE REPORT

A 6-year-old Afghan boy was referred with palmo-plantar keratodermas (PPK) (Fig. 1). The patient wasborn after a normal pregnancy to very distant relatedparents. He was normal at birth. At the age of 2 yearshe had developed hyperkeratotic plaques on his shins,knees, elbows, and buttocks. The superior parts ofthese patches on the shins had reticular pattern. Onhis lips and peri-oral area was a narrow band of dis-coloration (Fig. 2). Also, verrucouse papules andnodules were on the soles and subungual areas. Thelesions on the shins became infected secondary toitching and excoriation. Narrow linear opacities on thelower portions of the corneas without any problem ofthe sight were observed. On slight lamp examinationthere were some vascularizations of the lower portionsof the corneas. Erythematous telangiectatic lesionswere seen on the malar area of the face. On the upperborder of the right auricle there was a triangularcartilage defect (Fig. 2). According to his father, thisdefect developed secondary to continuous manipula-tion by the patient. A mild reticular discoloration all

Figure 2. Haloed lymphocytes are lined up along the basalcell layer of the epidermis (original magnification · 400)(Hematoxylin–eosin stain).

Address correspondence to Dean S. Morrell, M.D., Depart-ment of Dermatology, 3100 Thurston Bowles, University of NorthCarolina at Chapel Hill, Chapel Hill, NC 27599, or e-mail:morrell@med.unc.edu.

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