Focal Epithelial Hyperplasia (Heck Disease)in a Black Child

Barbara Binder, M.D.,* Ulrike Wieland, M.D.,� and Josef Smolle, M.D.,*

*Department of Dermatology, University Graz, Graz, Austria, �Institute of Virology, University Koln, Koln, Germany

Abstract: We describe a 3-year-old African male child with focal epithelialhyperplasia (Heck disease). The clinical diagnosis was confirmed histolog-ically and by the finding of human papillomavirus type 32.

Focal epithelial hyperplasia (FEH) is a rare skin dis-ease caused by human papillomavirus (HPV), whichmainly affects the oral mucosa, and, rarely, the genitaland anal mucosa. This disease is chronic, but in mostinstances a self-limited disease, most commonly found inchildren. It exists in numerous populations and ethnicgroups, especially in Eskimos, South American Indians,and SouthAfricans (1).We report FEH in a black boy ofAfrican ancestry.

CASE REPORT

A 3-year-old male child of African origin presented witha fewmonths history of enlarging lesions on themucosalside of the upper and lower lips. Clinical examinationfound soft, asymptomatic, round, flat-topped, pale pa-pules on the upper and lower labial mucosa (Fig. 1) andon the tongue (Fig. 2). Whole body examination re-vealed no other skin lesions. The boy’s mental andphysical development was normal and he had no historyof infectious, immunosuppressive or metabolic disease.

Laboratory investigations, including HIV and syph-ilis, had normal results. Histology showed a noncorni-fied, highly acanthotic epithelium with elongated reteridges, numerous mitoses, koilocytosis with perinuclearvacuolation, and intracytoplasmic basophilic granules.The underlying connective tissue displayed elongatedpapillae with dilated capillaries (Fig. 3).

In paraffin-embedded biopsy material, HPV 32 wasidentified by sequence analysis of a HPV group-specificPCR product generated with primers A6/A8, as des-cribed previously (2). We suggested topical imiquimodtherapy three times a week, but this was refused.

DISCUSSION

Focal epithelial hyperplasia is a rare disease of themucous membranes in children, first described byArchand et al in 1965 (3). They reported Inuit andIndian children from North and South America withpapillomatous lesions on the oral mucosa, sometimesalso affecting the genital and anal mucosa. Since then,FEH has been described in children from most conti-nents.

The clinical picture is characterized by multiple, cir-cumscribed, sessile, soft, elevated papules and nodules ofwhitish color or color similar to that of adjacentmucosa.The lesions are usually painless and the patients are ingood general condition; only a few patients have painand scalding sensations (4).

Histologic findings are also distinct, and suggestive ofHPV infection. Marked acanthosis with koilocytosis isusually seen. Connective tissue shows elongated papillae,dilated capillaries, and a mild lymphohistiocytic infil-trate. The cause of FEH is an infection with HPV.The most common HPV types isolated from biopsy

Address correspondence to Barbara Binder, M.D., Departmentof Dermatology, University Graz, Auenbruggerplatz 8, A-8036Graz, Austria, or e-mail: barbara.binder@klinikum-graz.at.

DOI: 10.1111/j.1525-1470.2007.00435.x

� 2007 The Authors. Journal compilation � 2007 Blackwell Publishing, Inc. E31

Pediatric Dermatology, E31–E32, 2007

specimens of themucosa are 32 and 13 (5). In our patientHPV type 32 was detected.

Usually, spontaneous remission of FEH is observedbyadolescence,but in some instances thepapillomasmaycontinuously progress and lead to significant functionaldisturbances, and for these patients treatment is recom-mended. Several treatment regimes have been describedin the literature. Surgical techniques such as excision,electrocauterization, cryotherapy and curettage areused, especially for localized lesions. Widespread lesionsmay require systemic treatment: acitretin, etretinate,interferon-a2, and methotrexate have been successfullyadministered, but recurrences are not infrequent.Another possibility is destruction by carbondioxide laser(6). All these treatment modalities, however, are associ-ated with well-known side effects and risks. Therefore,topical treatment such as interferon-b and imiquimodshould be preferred, especially in children. Recently,successful therapywith imiquimodhas been reported (7).Unfortunately, our little patient was noncompliant andwould not tolerate topical therapy.

Focal epithelial hyperplasia is a rare and distinctivedisorder of childhood. Diagnosis can be made clinicallyand confirmed by histologic findings and detection ofHPV-DNA type 13 or 32. It is a benign disease whichoften leads to spontaneous remission within severalmonths or years. No risk of malignancy associated withthis disorder is known, and therefore, treatment is onlynecessary in case of pain and/or feeding problems.

REFERENCES

1. Segura-Saint-Gerons R, Toro-Rojas M, Ceballos-Salobrena A et al. Focal epithelial hyperplasia. A raredisease in our area. Med Oral Patol Oral Cir Bucal2005;10:128–131.

2. WielandU,RitzkowskyA,StoltidisMet al. PapillomavirusDNA in basal cell carcinomas of immunocompetentpatients: an accidental association J Invest Dermatol2000;115:124–128.

3. Archand HO, Heck JW, Stanley HR. Focal epithelialhyperplasia: an unusual oral lesion found in Indian children.Oral Surg 1965;20:201–212.

4. Steinhoff M, Metze D, Stockfleth E et al. Successfultopical treatment of focal epithelial hyperplasia (Heck’sdisease) with interferon-b. Br J Dermatol 2001;144:1069.

5. Jayasooriya PR, Abeyratne S, Ranasinghe AW et al.Focal epithelial hyperplasia (Heck’s disease): report oftwo cases with PCR detection of human papillomavirusDNA. Oral Dis 2004;10:240–243.

6. Akyol A, Anadolu R, Anadolu Y et al. Multifocal papil-lomavirus epithelial hyperplasia: successful treatment withCO2 laser therapy combined with interferon alpha-2b. Int JDermatol 2003;42:733–735.

7. Maschke J, Brauns TC,GoosM. Imiquimod-Creme fur dietopische Behandlung der fokalen epithelialen Hyperplasie(M. Heck) bei einem Kind. JDDG 2004;2:848–850.

Figure 3. Epthelial hyperplasia, elongated papillae, dilatedcapillaries, and a mild lymphocytic infiltrate (H&E).

Figure 1. Numerous papillomatous lesions on the mucosalsurface of the lower lip.

Figure 2. Papillomatous lesions on the tongue.

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