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Multicentric Bowen disease in linear porokeratosis

European Journal of Dermatology. Volume 17, Number 5, 439-40, September-October 2007, Clinical report

DOI : 10.1684/ejd.2007.0244

Summary

Author(s) : Emmanuella Guenova, Wolfram Hoetzenecker, Gisela Metzler, Martin Röcken, Martin Schaller , Department of Dermatology, Eberhard Karl University – Tübingen, Liebermeisterstr. 25, 72076 Tübingen, Germany.

Summary : Linear porokeratosis is a genodermatosis, caused by a clonal proliferation of keratinocytes along the lines of Blaschko. This type of porokeratosis is particularly susceptible to malignant degeneration e.g. to Bowen disease, a squamous cell carcinoma (SCC) in situ with the potential for significant lateral spread. Here we report a case of linear porokeratosis on the left leg of a 56-year-old man, complicated with two lesions of multicentric Bowen disease.

Keywords : Bowen disease, linear porokeratosis, malignant degeneration

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ARTICLE

Auteur(s) : Emmanuella Guenova, Wolfram Hoetzenecker, Gisela Metzler, Martin Röcken, Martin Schaller

Department of Dermatology, Eberhard Karl University – Tübingen, Liebermeisterstr. 25, 72076 Tübingen, Germany

accepté le 17 Avril 2007

Porokeratosis represents a group of genetically heterogenous disorders of keratinization, characterized by the histopathological feature of the cornoid lamella, which represents a column of tightly fitted parakeratotic cells with pyknotic basophilic nuclei [1]. The multiple clinical variations of porokeratosis include plaque type (Mibelli), disseminated actinic superficial porokeratosis, porokeratosis palmoplantaris et disseminata, porokeratosis punctata palmoplantaris, porokeratosis filiformis palmoplantaris, giant porokeratosis and linear porokeratosis (LP) [2-4]. The increased potential of porokeratosis to malignant transformation is a well-known feature showing an association of epithelial tumors (e.g. SCC, Bowen disease and basal cell carcinoma) and porokeratosis between 7% and 11.6% [5, 6]. The linear type possesses the highest malignant potential [7, 8]. In this case we report of multicentric Bowen disease arising in hyperkeratotic LP.

Case report

A 56-year-old man presented with a lifelong history of a scaly linear lesion extending from the left buttock to the top of the left foot. The lesion was not pruritic but had become irritated over the past ten years – initially only around the ankle but now in the gluteal region as well. Serous fluid and blood intermittently stained his clothes, which stimulated him to seek medical help. Occasional exposure to sun with no blistering sunburn was recalled. The patient had no history of other skin diseases and the family history revealed neither porokeratosis nor any cancer of the skin.

Physical examination showed an extensive linear lesion lateral on the left leg perfectly matching the lines of Blaschko. It consisted of multiple, well-demarcated, confluent patches, several cm in size with silvery scale and red-brown rim (figure 1A). Two eroded plaques were noted within this larger lesion – a 10 × 12 cm lesion over the left fifth metatarsal bone (figure 1B) and a 3 × 3 cm one on the left buttock. The remaining clinical examination showed no pathological findings.

Histological examination of a typical scaly lesion showed prominent cornoid lamellae on a background of ortho- and parahyperkeratosis and irregular acanthosis, confirming the diagnosis of LP (figure 1C). Punch biopsies from the erosive plaques revealed an abundance of atypical epithelial cells with hyperchromatic nuclei and loss of polarity. The acanthotic epidermis showed a classic “windblown appearance”, consistent with Bowen disease (figure 1D). A computer-tomography scan of the thorax, abdomen and pelvis revealed no pathological findings.

The two plaques of Bowen disease were completely excised and the defects covered with split-thickness skin grafts taken from the left thigh. The surgical procedure and the postoperative period were uneventful. Follow-up at six months revealed no evidence of recurrence.

Discussion

LP is a rare variant of porokeratosis caused by a clonal proliferation of keratinocytes along the lines of Blaschko. Occurrence of LP is usually sporadic with no definitive pattern of inheritance established. However, LP has been observed concurrently with disseminated superficial actinic porokeratosis (DSAP) [9-13]. Recently, Freyschmidt-Paul et al. and Boente et al. described 3 cases of LP associated with DSAP, which is known to have an autosomal dominant trait [9, 10]. In general, as postulated by Happle, two types of segmental manifestations of autosomal dominant disorders can be distinguished [14]: type 1, which represents a linear appearance in an otherwise healthy person and which can be explained by postzygotic novel heterozygous mutation. Type 2 reflects loss of heterozygosity (LOH) for the same allele and displays severe involvement due to homo/hemizygosity for the underlying gene. As a characteristic feature, these patients show severe linear manifestation along the lines of Blaschko (e.g. LP), superimposed on a milder, nonsegmental, heterozygous involvement of the some disorder (e.g. DSAP).

The patient described in this report stated a life-long history of LP. This feature would be in line with LOH. However, as there was no further pathological involvement of the patient’s skin beside LP and family history revealed no evidence of porokeratosis, we suggest a type 1 segmental manifestation.

In this case, multicentric Bowen carcinoma developed on the background of a life-long LP. Interestingly, cancer-proneness of LP may be explained by allelic loss [8]. Furthermore, investigations have demonstrated an increased expression of the p53 tumor suppressor gene in all types of porokeratosis, a prerequisite for premalignant conditions [15, 16]. Malignant transformation has been reported in all five clinical variants of porokeratosis, however the rare linear type is particularly susceptible to carcinogenic degeneration [6, 8]. Treatment options for porokeratosis include topical 5-FU, retinoids and imiquimod [17, 18]. When malignant change occurs, surgery is required and micrographic surgery offers a precise way to separate the tumor from its porokeratotic background.

We describe a case of multicentric Bowen disease, arising in a lesion of hyperkeratotic LP, thus providing further evidence of the increased oncogenic potential of LP. Patients with this variant of porokeratosis should be monitored regularly to insure that malignant changes are detected as soon as possible and to minimize the risk of invasive and potentially metastatic tumors.

Acknowledgements

Financial support: none. Conflict of interest: none.

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