br Discussion As a previous report suggested invasive EMPD i

Discussion As a previous report suggested, invasive EMPD is highly metastatic to lymph nodes (47%), and when metastatic lesions expand beyond the inguinal lymph node and have metastasized systemically, it is difficult to cure. For treatment, although radiotherapy is useful for local control of noninvasive EMPD, it is difficult to irradiate at the appropriate therapeutic dose of radiation by conventional radiotherapy for lymph nodes adhering to vital organs. Based on the above findings, and because CyberKnife is useful to treat various inoperable, metastatic cancers with minimal side effects, we selected CyberKnife for the local control of metastatic EMPD. Like other apocrine-origin cancer such as breast cancer, Paget Go 6983 cost express RANKL and MMP7 in the lesional skin of EMPD. To induce the immunological effects on other cells, such as TAMs around the tumor cells, membrane-bound RANKL should be cleaved to its soluble (s)RANKL by MMP7, and sRANKL then stimulates TAMs to produce CCL17, which promotes the recruitment of Tregs to develop EMPD. Notably, in the lesional skin of EMPD, significant numbers of Tregs were detected adjacent to TAMs. These reports suggested that the depletion or modulation of TAMs could improve the tumor microenvironment, and could be an adjuvant immunotherapy for the patients with invasive EMPD. Indeed, BPs could enhance the antitumor immune response and suppress the progression of tumor growth in various cancer systems by the depletion of TAMs. In other reports, BPs modulate the chemokine profiles from TAMs to generate an antitumor immune response. These reports suggested that BPs could be useful for cancer that contains activated TAMs.
Dermatoporosis is a relatively new term that refers to chronic skin fragility in the aged population. This syndrome is characterized by chronic cutaneous weakness clinically represented by senile purpura, stellate pseudoscars, skin atrophy, and wounds following minimal trauma. It is known that skin aging and long term solar exposure contribute to its etiopathogenesis. In addition, chronic use of corticosteroids is regarded as a trigger factor for secondary dermatoporosis.
Bullous pemphigoid (BP) usually affects the elderly, but BP in infancy has also been reported. Here, we report our observation in a case of childhood BP in Taiwan. A 15-month-old boy presented with recurrent blisters on his face, trunk, and extremities 1 month later after receiving measles, mumps, rubella, and varicella vaccination (A–1D). A few vesicles were also seen on his tongue and oral mucosa, and the palms and soles were also involved (E). Direct and indirect Nikolsky signs were negative. Laboratory examinations showed an elevated immunoglobulin G (IgG) level (1290 mg/dL), low Complement component 3 (C3) (76.6 mg/dL), and negative findings for antinuclear antibody. Indirect immunofluorescence testing revealed positive antibasement membrane zone antibody (1:40) and a negative result for anti-intercellular substance antibody. Histopathologic examination of a blister on the left hand revealed a subepidermal bulla containing neutrophils, eosinophils, and lymphocytes, with papillary edema and congested dermal vessels surrounded by mononuclear cells (A–2B). Direct immunofluorescence (DIF) study showed strong linear deposits of IgG and C3 along the dermoepidermal junction and weaker IgA and complement component 1, q subcomponent (C1q) deposits, which supported a diagnosis of BP (C–2D). The patient was admitted to our hospital and the lesions on the trunk and extremities responded well to systemic steroid treatment. However, the oral blisters persisted after 3 weeks of intravenous corticosteroid (prednisolone 1–2.5 mg/kg/d) treatment. A dose of intravenous immunoglobulin (IVIG) with 1.8 g/kg was administered for the refractory oral lesions, after which the oral mucosa improved. BP was well-controlled by low-dose systemic steroid use. BP is an autoimmune bullous disease that is typically seen in the elderly and is very rare in infants and children. The first peak occurs more frequently from the 1 year of life (53.2%) and the second peak was reported at the age of 8 years (8.8%). Childhood BP is more frequent in girls. As in adults, lesions usually begin as urticarial pruritic papules and plaques followed by tense bullae on an erythematous background. In infancy, BP usually presents with blistering of the palms, soles, and face; mucosal involvement is uncommon. However, mucosal involvement is more frequent among older children, particularly genital involvement in girls.