The very frequent hamartomatous malformation from connective tissue, with variable epidermal, melanocytic, vascular, and lipogenic components, usually occurs for the first time during puberty, either in single or multiplied form, and especially in overweight persons. There is no gender preference. The preferred site is mechanically highly stressed areas, such as the neck, axilla, submammary, or groin. The lesion presents as a skin-colored, often pedunculated papule with pleats on the surface. Nodules or tumors (skin sack) rarely develop. By twisting the stalk, they often become reddish to blackish, and are painful through infarction (Fig. 1). Table 1 Helpful immunohistochemical markers for soft tissue lesions Differentiation Possible antibody Melanocytic S100 protein MelanA HMB45 Keratotic AE1/AE3 MNF116 KL1 CAM 5.2 Adnexal EMA CEA BerEP4 (EP-CAM) Neuroendocrine Neuron-specific enolase Synaptophysin Chromogranine CK 20 Fibrocytic CD34 Myogenic Actin Desmin Myoglobin Myosin Caldesmon Myogenin MYOD1 Vascular CD31 CD34 ERG Lymphogenic D2-40 (anti-podoplanin) Neural S100 protein Neurofilament protein Glial fibrillar protein Perineural EMA Dendritic CD1a Factor XIIIa CD34 Monocytes/macrophages KP1 (CD68) KiM1p CD163 Lymphocytic Chapter "▶ Cutaneous Lymphomas"