Zosteriform Lesions in an Elderly Man-Look Beyond Herpes Zoster.

Cutaneous metastasis in a zosteriform pattern is a very rare entity being reported only in 63 patients worldwide. Cutaneous metastases usually presents late in the course of the disease or sometimes after the treatment of the primary when it indicates recurrence of a treated malignancy. We report a case of zosteriform cutaneous metastases masquerading as lymphangioma without prior presentation of the primary malignancy.

What was known?

Cutaneous metastasis can present in various morphological forms.

It usually presents after the diagnosis of primary malignancy but at times may present before the primary malignancy.

Introduction

Cutaneous metastatic disease may be the first manifestation of an undiscovered internal malignancy or the first indication of recurrence of a supposedly adequately treated malignancy.[1] It commonly presents as solitary or widespread papules and nodules.[2] Rarely it may present in a zosteriform pattern or grow in a botryoid fashion.[3] According to a recent meta-analysis, only 63 cases of zosteriform cutaneous metastasis have been reported.[2] Here we report a case of zosteriform cutaneous metastatic disease resembling lymphangioma circumscriptum.

Case Report

A 55-year-old male presented with grouped, fluid-filled lesions over the right side of the neck, shoulder and upper chest since 3 months. The lesions started on the nape of neck. They increased in size and number to form larger lesions resembling cluster of grapes and further spread to involve the right upper chest and shoulder in a month. The patient developed severe pain, redness and swelling over the affected area and swelling over the right side of the face with dysphagia and odynophagia since 15 days. This was associated with eruptions of few fluid-filled lesions on the neck and infra-axillary area. The patient gave history of loss of weight, loss of appetite and frequent upper respiratory tract infections for the past 1 year.

On examination, bilateral cervical, submandibular and supraclavicular lymph nodes were enlarged, tender and firm to hard in consistency. On cutaneous examination, a large ill-defined tender, erythematous and indurated plaque studded with grouped papulovesicular lesions resembling a bunch of grapes varying in size from 0.5 × 1 to 5 × 3 cm was present in a zosteriform fashion extending from the neck to the upper chest including the shoulder on the right side in C3, C4 and C5 dermatomes [Figures 1 and 2]. Few discrete vesicles on an erythematous base were present on right side of neck and right infra-axillary area. A single firm to hard tender subcutaneous nodule measuring 2 × 3 cms was present on the right upper back.

Figure 1

Ill-defined erythematous and indurated plaque studded with grouped papulovesicular lesions

Figure 2

Papulovesicular lesions resembling a bunch of grapes on the neck

A differential diagnosis of adult onset lymphangioma circumscriptum superimposed with herpes zoster and zosteriform cutaneous metastasis was considered.

Complete hemogram was normal except for an ESR of 32 mm at the end of 1 hour. Blood sugars, liver function tests, renal function tests, and serum electrolytes were normal. VDRL was non-reactive and HIV test was negative. Tzanck smear from vesicles did not show any multinucleated giant cells.

A skin biopsy from papulovesicular lesions and from vesicles showed clusters of large, atypical round to oval cells having a high nuclear cytoplasmic (N/C) ratio with vesicular, hyperchromatic nuclei and prominent nucleoli [Figure 3]. They were arranged in sheets along with other inflammatory cells suggesting metastatic deposits of poorly differentiated carcinoma. Vascular and lymphatic invasion by tumor emboli was seen [Figure 4]. An ultrasound scan of the neck showed bilateral lymph nodal masses.

Figure 3

Cluster of atypical cells having a high N/C ratio with vesicular, hyperchromatic nuclei and prominent nucleoli, in sheets along with other inflammatory cells

Figure 4

Vascular invasion seen as tumor emboli

MRI of the head and neck showed bilateral thyroidomegaly, multiple enlarged submandibular, cervical and supraclavicular lymph nodes on both sides of the neck. Enlarged sub-carinal lymph nodes were seen. A solid focal lesion at nape of neck was seen indicative of a tumor deposit.

Based on these findings, a diagnosis of zosteriform cutaneous metastasis to an unknown primary was made. Immunohistochemistry with a panel of antigens was done from the biopsy specimen to look for the unknown primary malignancy. HMWCK, Pan CK, p63, and desmoglein markers were positive and HMB 45, S 100, CEA, and Napsin markers were negative. Immunohistochemistry markers favored a diagnosis of squamous cell carcinoma of pulmonary origin.

Unfortunately, the patient expired in 1 week after admission, so a detailed work up for the primary malignancy was not possible.

Discussion

Cutaneous metastasis from various primary internal malignancies varies from 0.2% to 10.4% in various studies.[4] Malignancies frequently developing skin metastases are malignant melanoma, followed by adenocarcinoma of the breast, lung, colon and ovary.[5] Frequency of metastatic skin disease in men is highest with lung carcinoma, followed by large intestine and melanoma.[3] Typical locations of metastases are chest wall, followed by the face and lower extremities.[6]

Cutaneous metastasis may present in various morphological forms. The common morphological forms are solitary or widespread papules and nodules. Various other morphological forms have been described and include inflammatory, sclerotic, bullous or vesicular lesions.[2] Rarely, it may present in a zosteriform pattern or grow like a cluster of grapes.[3] According to a recent meta-analysis, only 63 cases of zosteriform cutaneous metastasis have been reported.[2] Zosteriform cutaneous metastasis can be seen with lung carcinoma, gastric adenocarcinoma, carcinoma of renal pelvis, prostate, bladder, breast and ovary.[37] Metastases resembling lymphangiomas can rarely be seen with carcinoma lung, breast, cervix and ovary.[3]

Cutaneous metastasis is not an uncommon manifestation of visceral malignancy. In most cases, it develops after initial diagnosis of primary internal malignancy and late in the course of disease. In extremely rare cases it can be the presenting sign of underlying primary malignancy.[89] Literature search revealed that cutaneous metastases of carcinomas of the lung and kidney may be identified before the diagnosis of the primary tumor.[3] Most of the cutaneous metastases from lung cancer appear like cluster of cutaneous nodules and very rarely they may present in a zosteriform fashion.[3] Zosteriform metastasis can occur at the site of prior herpes zoster infection due to koebner-like reaction at the site (’locus minoris resistentiae’- site of lessened resistance). Other mechanisms could be due to perineural lymphatic spread, spread via fenestrated vessels of the dorsal root ganglion and accidental surgical implantation.[7]

Our patient presented with cutaneous metastatic disease without any signs and symptoms of a primary malignancy. In an elderly patient presenting with zosteriform lesions, metastatic carcinoma must be kept as a differential diagnosis along with other dermatoses presenting in a linear fashion. Metastatic carcinoma can present with various unusual clinical presentation and lesions resembling lymphangioma is one such unusual presentation rarely reported in the literature.

What is new?

Zosteriform lesions in elderly need not be herpes zoster always and needs careful evaluation.

In elderly patients, cutaneous metastasis may have a lymphangiomatous appearance clinically and should be considered in the differential diagnosis of zosteriform lesions.