A 3-day-old boy was referred to our department for the evaluation of skin finding presented at birth. The lesion showed erythema, linear vesiculations, and focal crust on the trunk and leg, predominantly over the left side (Fig. 1A, B).
At the time of birth, several vesicles and erythematous papules developed over the left trunk and thigh along Blaschko’s lines. Blistered skin was replaced by a crusted lesion on the fifth day after birth (Fig. 1C). Then, hyperkeratotic papules and verrucous lesions occurred consecutively on the fifteenth day (Fig. 1D).

INTRODUCTION
Incontinentia pigmenti (IP), also known as Bloch- Sulzberger’s disease, is a rare X-linked dominant genodermatosis caused by a mutation in nuclear factor- kB (NF- kB) essential modulator (NEMO) gene on the X chromosome, localized to Xq28. IP predominantly affects female infants (in excess of 37 : 1), and is usually lethal in males in utero. The survival of affected males is attributed to the presence of an extra X chromosome (Klinefelter’s syndrome), hypomorphic mutations, and somatic mosaicism. Only two cases of IP in male infants without karyotype study have been reported in the Korean dermatologic literature since 1982 . Read the rest of this entry »

Evaluation and management of lymph node metastasis should be performed in all patients with MCC, because lymph node metastasis develops in up to 75% of patients. But, Routine performance of elective lymph node dissection (ELND) is currently considered controversial because of potential morbidity, without proven survival benefit. Many recent studies suggest that, as alternative method, sentinel lymph node (SLN) mapping and biopsy could improve the ability to detect subclinical nodal metastases, sparing those patients with negative nodes the morbidity of lymph node dissection. SLN mapping and biopsy could provide a more accurate prognostic information for patients. Sheela G et al. recently suggested that SLN biopsy should be routinely included in the evaluation of patients with MCC.
Merkel cell carcinoma (MCC) typically presents as a solitary dome-shaped, deep red to violaceous nodule or indurated plaque and tends to have a shiny surface, often with overlying telangiectasia. The overlying skin is usually intact. Histologically, anastomosing cords and strands of neoplastic cells are seen in the dermis and subcutis, but not in the epidermis. The individual cells are monomorphous with round vesicular nuclei and scanty, ill-defined cytoplasm. Ultrastructurally the tumor cells are characterized by the presence of paranuclear filament whorls and membrane-bound neurosecretory granules approximately 100 ~ 150 nm across. Immu- nohistochemistry is an important tool in the diagnosis of MCC. The tumor cells have epithelial and neural properties because coexpression of cytokeratin filaments and neurofilaments can be observed. Immunohistochemical study results are positive for neuroendocrine markers (chromogranin, synapto- physin, neurospecific enolase and neurofilaments), low molecular weight cytokeratins and membrane epithelial antigen. Especially, perinuclear dot-like staining for cytokeratin 20 is a characteristic finding.
A 73-year-old man had a 2-month history of a rapidly growing painless mass on the left upper arm.

INTRODUCTION
Merkel cell carcinoma (MCC), also referred as neuroendocrine or trabecular carcinoma, is an uncommon malignancy originally described by Toker in 1972. Although the exact origin of the Merkel cell is unknown, it probably arises from neuro¬endocrine cells between the basal epidermis and then grows vertically into the dermis and sub¬cutaneous tissue. It usually arises in the head and neck area and extremities of elderly people as a nodule or plaque lesion. Cases of MCC have a poor outcome characterized by locoregional and distant relapse. It has been reported that 34% of affected patients die because of the tumor. Recent evidences suggest that surgery and adjuvant locoregional radiotherapy may produce a better disease-free survival compared with surgery alone. Read the rest of this entry »
Ultrastructural analysis shows tumor cells closely apposed to each other by continuous basal laminae and rudimentary cell junctions. Cells show abundant cytoplasm containing numerous mitochondria and aggregates of glycogen.