JAAD GRAND ROUNDS

Answer sheets are bound into the Journal for US, Canadian, and life members of the American Academyof Dermatology. Additional answer sheets may be obtained by contacting the Member Resource Centerof the American Academy of Dermatology by calling toll-free (866) 503-SKIN (7546), by calling (847)240-1280 (for international members), or by e-mailing mrc@aad.org.

The Self-Assessment examination of the American Academy of Dermatology is changing its name toJAAD Grand Rounds. The content will remain the same and still offer AMA PRA Category 1 Credit�.This change is being implemented to reduce confusion regarding which AAD activities fulfillComponent 2 (Life-long learning and Periodic Self-Assessment) of the ABD’s Maintenance ofCertification in Dermatology (MOC-D) Program.

JAAD Grand Rounds quiz*Identification No. 809-203

Learning objectives: At the conclusion of this self-assessment learning activity, physician participants shouldbe able to assess their own diagnostic and patient management skills with respect to those of theircolleagues in the field, use the results of this self-assessment to help determine personal learning needs thatcan be addressed through subsequent CME involvement, and enhance their ability to comply with therequirements for certification in the specialty of dermatology.

Instructions for Category I CME credit appear in the front advertising section. See last page of Contents forpage number.

Instructions: In answering each question, refer to the specific directions provided. Because it is oftennecessary to provide information occurring later in a series that give away answers to earlier questions,please answer the questions in each series in sequence. ( J Am Acad Dermatol 2009;60:532-8.)

A 69-year-old male with a pustular eruption

Lindsay Higgins, Jennifer Johnson, MPH, BrittanyOswald, MD, and Erin E. Boh, MD, PhDNew Orleans, Louisiana

A 69-year-old male was referred from a woundcare center for an evaluation of progression of atender lesion that first appeared 7 months earlier.The lesion emerged as pustules that involved theperiungual and distal nail of the right second digit.The patient had a history of chronic lymphocyticleukemia (CLL), diagnosed 10 years earlier, which

*The Self-Assessment Task Force of the American Academy of

Dermatology is led by Erin E. Boh, MD.

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had been stable until he developed lymphade-nopathy approximately 3 months after the lesionarose.

Initial evaluations by outside physicians includedseveral cultures, bone scans, debridement andwound care, intravenous and oral antibiotics, andantifungals without improvement of the lesion. Con-comitantly, the patient began fludarabine chemo-therapy treatment (Fludara, Berlex Laboratories,Montville, NJ) for the progression of CLL. After hisfirst round of fludarabine, he noted worsening of thelesions. Upon his fourth round of fludarabine withprednisone, the patient finally saw some mild im-provement despite the prednisone taper that wasstarted in anticipation of his dermatology clinicreferral.

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a. Intraepidermal spongiotic vesicles with littleinflammatory change

b. Aggregations of neutrophils between para-keratotic stratum corneum layers

c. Subcorneal cavities filled with neutrophilsand spongiform pustules of Kogoj

d. Spongiosis and intracellular edemae. Pale superficial keratinocytes

3. Which of the following can be used to treat thiscondition?a. Calcipotriolb. Psoralen plus ultraviolet A light phototherapyc. Retinoidsd. Corticosteroidse. All of the above

4. Which of the following is not a potential com-plication of this disease?a. Anonychiab. Malignant changec. Interphalangeal joint arthropathyd. Generalized pustular eruptionse. Syndactyly

5. The etiology of this condition is:a. malignancy.b. allergic reaction.c. Staphylococcus aureus.d. fungal infection.e. unknown.

DiscussionOriginally described in 1890 as a suppurative

condition, acrodermatitis continua of Hallopeau(ACH) is a rare and chronic skin disorder that isconsidered to be a form of acropustular psoriasis.Also known as dermatitis repens and acrodermatitisperstans, ACH has a greater prevalence amongmiddle-aged females, although it may occur in anyage group. Trauma is thought to play an initiatingrole and is associated with ACH, as are infection andheavy smoking, although the true etiology remainselusive.

ACH presents as a pustular eruption on the distalfingers and occasionally the toes, involving the acralareas and nails. Sterile pustules form on an erythem-atous and scaling base, rupture, and then arise anew.The whole process often begins at one or twofingertips, or less frequently toes. Symptoms mayremain limited to the original site or may spread toinvolve multiple digits or extend proximally toinvolve the dorsal aspects of the hands, forearms,or feet.

Throughout its chronic course, acute episodes ofpustulation occur intermittently, seemingly withoutreason. Over time, the pustules may coalesce to form

His medical history included childhood measlesand mumps, reactivated varicella zoster virus, upperrespiratory infection, oral and labial herpes simplexvirus, and CLL. His surgical history included tonsil-lectomy, appendectomy, cardiothoracic surgery re-pair of atrial septal defect, and interatrial aneurysm,bilateral cataract repair, and right index finger de-bridement. His family history was noncontributory.His current medications are Fludara once per monthand prednisone 30 mg daily (taper); he previouslyreceived treatment with minocycline, terbinafine,ertapenem, linezolid, and voriconazole.

The physical examination revealed sharply de-marcated erythematous, scaling, and pustular pla-ques on the second and third digits of the right hand,spanning from the distal fingertip to the area be-tween the proximal interphalangeal and metacarpal-phalengeal joints (Fig 1). Pustulosis of the secondnail was noted, while the nail on the third finger wasavulsed. No other lesions were noted on the hands.The patient did not have psoriasiform lesions onextensor joint surfaces, oral or conjunctival lesions,or juicy erythematous plaques on the face, back,chest, or arms.

Laboratory studies were undertaken with tissuecultures showing no growth, and potassium hydrox-ide preparations were negative. A complete bloodcell count (CBC) showed a normal white blood cellcount, slight anemia, and mild thrombocytopenia.The CBC differential and comprehensive metabolicpanel were within normal limits, while the absoluteneutrophil count was elevated at 3135 (normal,\550). A bone scan was negative for infection.

1. What is the most likely diagnosis?a. Acrodermatitis continua of Hallopeaub. Dyshidrotic hand eczemac. Acrodermatitis enteropathicad. Allergic contact dermatitise. Psoriasis arthropathica

2. Which of the following are histopathologicfeatures of this disease?

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lakes of pus and crust over. With long-term ACH,generalized pustular eruptions may develop.Periods of remission or resolution may arrive be-tween flares, although some cases are extremelyrecalcitrant.

Atrophic skin changes are often notable in thosewith ACH. Erythema, keratosis, and fissuring areseen with persistent pustulation that may eventuallylead to the destruction of nail bed and matrix andsevere onychodystrophy or anonychia. In somecases, the disease further affects the bones, causingosteolysis of the distal phalanx and arthropathy ofthe interphalangeal joints. In severe cases, patientsmay experience a chronic painful and disablingcondition with sclerosis of soft tissue, flexion con-tractures, or even syndactyly.

Histopathologic examination reveals epidermalacanthosis, subcorneal neutrophilic macropustules,and aggregated leukocytes between epidermal cells,which form spongiform pustules of Kogoj. In addi-tion, the papillary dermis may contain an intermedi-ate level of lymphohistiocytic infiltrate and localedema. Parakeratosis and hyperkeratosis may alsobe observed. However, necrosis and spongiosis ofthe epidermal cells are not present. In persistentlesions, epidermal thinning and papillary dermalatrophy may be remarkable.

The diagnosis of ACH is based on the aboveclinical and histopathologic findings. However, in apractical sense, it may also be considered one ofexclusion. Patients presenting with such lesionsshould have potassium hydroxide preparations per-formed and cultures made to rule out infectiouscauses. The differential diagnoses includes paro-nychia secondary to bacterial or fungal infection,digital herpes simplex virus infection, allergic con-tact dermatitis, dyshidrotic hand eczema, psoriasisarthropathica, acrodermatitis enteropathica, nonder-matophyte onychomycosis, and pyoderma.

In a small portion of patients, a generalizedpustular psoriasis may develop, contributing to theview of ACH as a variant form of psoriasis. One caseoutlines a patient who had symptoms of both ACHand psoriatic arthritis and was found to be humanleukocyte antigeneB27 positive. Such findings arehighly indicative of some relationship between ACHand psoriasis. Although classified as a form of pso-riasis because of its histologic similarities, ACH doesnot necessarily respond to antipsoriatic drugs andtherapies. Whatever treatment regimen is selected,the results are often disappointing.

ACH may be quite resistant to successful treat-ment, and because no therapy provides lastingremission, no definitive treatment guidelines exist.Many therapies have been attempted, but remission

is usually incomplete and recurrences occur rapidly,especially with treatment discontinuation or dosereduction. Only in the rare case does spontaneousimprovement occur.

Topical treatments include corticosteroids, tacro-limus 0.1% ointment under occlusion, vitamin Dderivatives, 5-fluorouracil, dithranol, and tar. Vita-min D derivatives, such as calcipotriol and calcitriol,work by reducing keratinocye hyperproliferationand improving the penetration of other topicalmedications. The two-compound ointment contain-ing betamethasone dipropionate and calcipotriolhas been found to be efficacious when appliedtwice daily for about a month, although it hasreportedly aggravated the condition for some pa-tients. Another option is intralesional injections withtriamcinolone.

For more resistant cases, topical calcitriol andtacrolimus 0.1% ointment has been employed withsome success. Tacromilus, derived from a fungalmetabolite of Streptomyces tsukubaensis, is an im-munomodulator that works by down-regulatinginflammatory cells in the skin. As a calcineurininhibitor, tacrolimus serves as an antiinflammatoryagent without the skin side effects observed withcorticosteroids. Topical treatments, which tend tohave fewer side effects, have been used alone and incombination with systemic treatments.

A variety of systemic therapies give the clinicianmany options for treating ACH. Corticosteroids, reti-noids (acitretin,methotrexate, cyclosporine, dapsone,and hydroxycarbamide), antibiotics (tetracycline andminocycline, colchicine, and hydroxyurea), and psor-alen plus ultraviolet A light phototherapy (PUVA)have all been helpful in some cases. Biologic agents,such as tumor necrosis factor-alfa (TNFa) antagonists,including etanercept, infliximab, and adalimumab,have also shown promise. TNFa, a proinflammatorycytokine, mediates leukocyte recruitment, migration,and activation, essentially triggering an inflammatorycascade. However, when the patient has a history ofcancer, the biologics should be avoided. Etanercepthas been found to be especially efficacious whencombined with acitretin.

Clinically, this patient was suspected to haveacrodermatitis continua of Hallopeau. With this inmind, his prednisone taper was continued. Twoweeks after the initial evaluation, diprolene ointmentafter each dressing change by wound care wasencouraged, and oral sulfasalazine 500 mg was takentwice daily. The response to the topical steroids andsulfasalazine was impressive, with regrowth of nor-mal skin and no evidence of pustules.

For this series, the recommended choices are asfollows: 1, a; 2, c; 3, e; 4, b; 5, e.

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6. What is the most likely diagnosis?a. Dermatofibromab. Dermatofibrosarcoma protuberansc. Infantile digital fibromatosisd. Juvenile xanthogranulomae. Keloid

7. What is/are the histopathologic characteristicfeature(s)?a. Compact, glassy, eosinophilic collagen bun-

dlesb. Eosinophilic cytoplasmic inclusion bodiesc. Spindle-cell or fusiform macrophages and

Touton giant cellsd. Hyperpigmented basal layer separated by a

clear (Grenz) zone from the tumor cells inthe dermis

e. Spindle cells arranged in a storiform pattern

8. Malignant transformation may occur in up towhat percent of patients?a. 5%b. 10%c. 15%d. 25%e. There is no evidence of malignant transfor-

mation

9. The treatment of choice for the aggressive typeof lesion is:a. radiotherapy.b. cryotherapy.c. cautery.d. intralesional steroid injection.e. surgery.

10. After surgical resection, the rate of recurrence isreported to be as high as ___.a. 5%b. 15%c. 30%d. 60%e. 90%

DiscussionInfantile digital fibromatosis (IDF), first described

by Reye in 1965, is a benign fibro/myofibroblasticproliferation that almost always occurs only on thefingers and toes. The condition is a rare, benign,spontaneously regressing, fibrous tissue tumor ofinfancy and childhood that commonly appears in thefirst year of life and may be present at birth in one-third of cases. In its characteristic from, it is notknown to occur in adulthood.

Clinically, it presents as 1- to 2-cm, firm nodules,single or multiple, the color being pink or similar tothe normal skin. It has predilection for the third,

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A large tumor on the toe

Mapar Mohammad Ali, MD, Hashem Zadeh Sara,MD, and Ayoobi Arash, MDAhvaz, Iran

A 5-month-old boy presented with a skin tumorover the extensor aspect of the third left toe. Thislesion was noted in the third month of life and hadgradually increased in size since that time. The full-term infant was the first and normal pregnancy anddelivery to young, unrelated parents. He was normalat birth without any problems.

The physical examination revealed a pink, firm,2 cm 3 2.5 cm erythematous dermal nodule with asmooth surface located primarily on the distal pha-lanx of the third toe (Figs 2 and 3). It was tender onpressure but did not have any joint deformity. Allother examinations were normal. Routine laboratorytests did not show any abnormalities.

An incisional biopsy was performed. It revealedinfiltration of the dermis with uniform spindle cells,collagen bundles arranging in interlacing fascicles,and a few eosinophilic intracytoplasmic inclusionbodies (Fig 4).