pain or tingling; spontaneous resolution within afew minutes; and the ‘‘hand in the bucket’’ sign.2

Histologic examination shows dilated eccrine ductsand orthokeratotic hyperkeratosis. The cause of thisrare condition remains unclear, but it may be relatedto an acquired sweat gland abnormality or a primarykeratoderma. The condition may also be associatedwith functional and structural changes in the stratumcorneum during puberty.5 A total of 16 cases ofacquired aquagenic syringeal acrokeratoderma havebeen reported in the European and American liter-ature. This number has increased since the last timethe same literature was studied.3,6

We present a new case that contributes to theknowledge of this disease. We believe that thecondition has been underdiagnosed thus far, giventhe clinical presentation of the lesions and thespontaneous resolution.

Cristina Serrano Falcon, MD,a and Salvio SerranoOrtega, MDb

Dermatology, Hospital de Alta Resolucion de Gua-dix, Guadix, Granada, Spain,a and Departmentof Dermatology, Facultad de Medicina, Univer-sidad de Granada, Spainb

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Cristina Serrano Falcon, MD,Dermatologıa, Avenida Mariana Pineda S/N18500 Guadix, Granada, Spain

E-mail: Crisefa77@hotmail.com

REFERENCES

1. Pardo J, Sanchez-Motilla JM, Latasa JM. Atypical aquagenic

keratoderma [in Spanish]. Actas Dermosifiliogr 2005;96:540-2.

2. Yan AC, Aasi SZ, Alms WJ, James WD, Heymann WR, Paller AS,

et al. Aquagenic palmoplantar keratoderma. J Am Acad

Dermatol 2001;44:696-9.

3. Baldwin BT, Prakash A, Fenske N, Messina JL. Aquagenic

syringeal acrokeratoderma: report of a case with histologic

findings. J Am Acad Dermatol 2006;54:889-902.

4. English JC, McCollough ML. Transient reactive papulotranslu-

cent acrokeratoderma. J Am Acad Dermatol 1996;34:686-7.

5. Betlloch I, Vergra G, Albares MP, Pascual JC, Silvestre JF, Botella

R. Aquagenic keratoderma. J Eur Acad Dermatol Venereol

2003;17:306-7.

6. Sais G, Bigata X, Admella C. Aquagenic syringeal acrokerato-

derma [in Spanish]. Actas Dermosifiliogr 2007;98:69-70.

doi:10.1016/j.jaad.2008.04.033

Fig 1. Lesions on the palmar surface of the patient’s hand.

J AM ACAD DERMATOL

VOLUME 59, NUMBER 5

Letters S113

Treatment of juvenile pityriasis rubra pilariswith etanercept

To the Editor: Pityriasis rubra pilaris (PRP) is a rarepapulosquamous disorder characterized by circum-scribed follicular papules, varying degrees of eryth-roderma, islands of uninvolved skin, andpalmoplantar keratoderma.

We report the case of a 16-year-old girl with a 4-year history of a pruritic, scaly eruption which beganon her scalp and spread to the trunk and extremitiesafter 1 year. Another physician diagnosed psoriasisand initiated treatment with etanercept 50 mgsubcutaneously twice weekly after she failed torespond to topical steroids, topical retinoids, andcalcipotriene.

She was first evaluated in our clinic 4 weeks afterstarting etanercept. The physical examination wasnotable for erythroderma with islands of uninvolvedskin, thick hyperkeratotic plaques on her shins, fol-licular papules on the extremities, and desquamationof the palms and soles. A skin biopsy was performedand confirmed the diagnosis of PRP. Because thepatient reported improvement on etanercept, wecontinued the medication at the same dose.

Four weeks later, the hyperkeratotic plaques onher shins were thinner and the erythroderma hadresolved. After 4 months of therapy, she had nearcomplete clearance (Fig 1). The dose of etanerceptwas tapered to 50 mg weekly for 2 months, 25 mgweekly for an additional 2 months, and then dis-continued. A complete remission was maintained for6 months before the skin disease flared. Etanercept50 mg twice a week was restarted and near clearancewas achieved after 2 months of therapy. The patientis currently maintained on etanercept 50 mg weekly.She has experienced no adverse effects.

The treatment of PRP in both adults and childrenremains challenging. There is no universally ac-cepted treatment because the response rate to

Primary pyoderma gangrenosum of the lungs

To the Editor: An 82-year-old white female with ahistory of osteoarthritis, osteoporosis, and hyperlip-idemia presented with a cough of 5 months’ dura-tion. A chest radiograph revealed a suspicious massin the right upper lobe of her lung, and a computedtomography scan identified a 7-cm lesion (Fig 1, A).A computed tomographyeguided needle aspirationwas performed, revealing a single fragment ofatypical squamous epithelium suspicious for squa-mous cell carcinoma. A bronchoscopy revealedclusters of neutrophils but was negative for tumor.Another needle aspiration and two separate biopsiesshowed nonspecific inflammatory changes with anegative acid-fast stain. After a negative mediastino-scopy, the patient underwent an exploratorythoracotomy.

A large mass in the posterior right upper lobe wasencountered, and biopsies showed changes consis-tent with bronchioalveolar cancer with negativelymph nodes. She was started on oral chemotherapy,and the dermatology department was consulted toevaluate nonhealing of the thoracotomy and media-stinoscopy sites. The physical examination revealeda 19-cm long, 2-cm wide, and 0.3-cm deep ulcer onthe right side of her back, and a 1 cm 3 0.6 cmhealing mediastinoscopy site at the mid-upper chest(Fig 2). A punch biopsy of the back revealed ulcer-ation and diffuse neutrophils and lymphocytes con-sistent with pyoderma gangrenosum (PG; Fig 3).With this diagnosis in mind, we reviewed the

J AM ACAD DERMATOL

NOVEMBER 2008

S114 Letters

different therapies varies widely. Oral retinoids andmethotrexate are the most frequently used systemicmedications for PRP. A review of the literature revealsa number of case reports of adult PRP patients whoresponded to treatment with infliximab1 and etaner-cept both with2 and without3 acitretin.

There are two previous case reports of juvenilePRP treated with a tumor necrosis factorealfa (TNF-a) inhibitor. Ruzetti et al4 demonstrated successfultreatment of juvenile PRP with infliximab, andGomez et al5 reported clinical improvement withefalizumab. To our knowledge, this report is thefirst to examine treatment of juvenile PRP withetanercept.

The etiology of PRP remains unclear. Some be-lieve it is a manifestation of abnormal vitamin Ametabolism, because PRP patients commonly re-spond to retinoid therapy. Others link PRP to im-mune dysregulation. A third theory is that PRP,specifically juvenile PRP, is a superantigen-mediateddisease, similar to staphylococcal scalded skin syn-drome or guttate psoriasis.

Controlled studies are needed to establish the effi-cacy of TNF-a inhibitors in the treatment of PRP, butthey may be difficult to perform because of the rarity ofthe disease. Our case report and those discussed abovedemonstrate that TNF-a inhibitors may be a promisingtreatment option for both adult and juvenile PRP.

Veronica Cox, MD, Erin B. Lesesky, MD, BarbaraD. Garcia, MD, and Terence C. O’Grady, MD

Division of Dermatology, University of Califor-niaeSan Diego, San Diego, California

Funding source: None.

Conflicts of interest: None declared.

Presented at the 2006 Summer American Academyof Dermatology live patient discussion session,San Diego, CA, July 29, 2006.

Fig 1. Right arm pretreatment (A) and 4 months posttreat-ment (B) with etanercept.

Correspondence to: Erin B. Lesesky, MD, Divisionof Dermatology, University of CaliforniaeSanDiego, 200 W Arbor Dr, #8420, San Diego, CA92103

E-mail: erinbialas@hotmail.com

REFERENCES

1. Liao WC, Mutasim DF. Infliximab for the treatment of adult-

onset pityriasis rubra pilaris. Arch Dermatol 2005;141:423-5.

2. Davis KF, Wu JJ, Murase JE, Rosenberg FR, Sorenson EP,

Meshkinpour A. Clinical improvement of pityriasis rubra pilaris

with combination etanercept and acitretin therapy. Arch

Dermatol 2007;143:1597-9.

3. Seckin D, Tula E, Ergun T. Successful use of etanercept in type I

pityriasis rubra pilaris. Br J Dermatol 2008;158:642-4.

4. Ruzzetti M, Saraceno R, Carboni I, Papoutsaki M, Chimenti S.

Type III juvenile pityriasis rubra pilaris: a successful treat-

ment with infliximab. J Eur Acad Dermatol Venereol 2008;

22:117-8.

5. Gomez M, Ruelas ME, Welsh O, Arcaute HD, Ocampo-Candiani J.

Clinical improvement of pityriasis rubra pilaris with efalizumab

in a pediatric patient. J Drugs Dermatol 2007;6:337-9.

doi:10.1016/j.jaad.2008.06.016