Dermoscopy of papillomatosis cutis lymphostatica: a report of two cases

INTRODUCTION

Papillomatosis cutis lymphostatica (PCL) is a rare skin condition characterized by the presence of verrucous and papulo-nodular lesions in the area of lower limbs [1]. Its cause is chronic edema of the lower extremities caused either by primary causes such as hereditary lymphedema, or secondary factors, such as lymphatic vessels damage, venous insufficiency, tumors or radiotherapy [2, 3]. All of them lead to chronic inflammatory condition, hyperproliferation of epidermal cells, and abnormal keratinization process [1]. There are no guidelines regarding the management of PCL, however, besides treatment of the primary cause, compression, surgery or laser therapy, beneficial use of vitamin A derivatives have been described [1, 4]. So far, to the best of our knowledge, there have been only 10 articles regarding this dermatosis published and there are no actual specific reports on dermoscopic findings.

OBJECTIVE

Hereby we present for the first time detailed dermoscopic features and pictures of this rare entity such as PCL.

CASE REPORT

Two adult men presented to the dermatologic admission room at the University of University of Campania, Naples, Italy. The patients were 30 and 52 years old, both had morbid obesity according to body mass index (BMI). They presented with pink and flesh-colored papulo-nodular-papillomatous firm and exudative lesions, located on both legs. Lesions were covered with honey-colored crusts indicating bacterial superinfection. Moreover, severe edema of lower limbs was observed in both patients (figs. 1. A, B). On dermoscopy examination, we found non-focused dotted vessels clustered in squares, resembling red rosettes, distributed within the whole lesion, in some cases with a glomerular aspect; in other lesions vessels were separated by white lines resembling the inverse network of the Spitz naevus. Moreover, we noticed slight whitish-yellowish scales of peripheral arrangement. We also found shiny white structures – blotches and strands with no specific arrangement. Lesions had a pink structureless background. In one of the patients prominent scaling was observed (figs. 2. A, B). Both patients were advised to visit a vascular surgeon and were prescribed antibiotics to treat the superinfection.

CONCLUSIONS

Papillomatosis cutis lymphostatica is a rare cutaneous disorder that has not been widely studied so far. Besides its clinical picture, dermoscopy may support the diagnosis, especially in cases of atypical clinical manifestations. Since this is the first full description of dermoscopic features of PCL, we cannot determine the prevalence, sensitivity, or specificity of its findings. However, in most of our patients’ lesions, the vascular pattern was quite similar, with non-focused, clustered dotted and glomerular vessels, and shiny white blotches and strands. Further reports on this matter will help to determine if these characteristics are typical of this entity.
In the differential diagnosis of the pink nodules on the lower limbs, we could take into consideration Kaposi sarcoma, Merkel cell carcinoma (MCC), or cutaneous metastases. Kaposi sarcoma is characterized by the ‘rainbow pattern’, blue or red background, and scales on the surface [5]. In MCC, the lesion is usually solitary and we can observe a milky red background with linear or polymorphous vessels [6]. Skin metastases exhibit an increased density of polymorphous vessels but other features may vary depending on the point of origin of the primary neoplasm [7].

FUNDING

No external funding.

ETHICAL APPROVAL

Not applicable.

CONFLICT OF INTEREST

The authors declare no conflict of interest.

References