IgA vasculitis is a type of small vessel vasculitis that is characterized by IgA- immune deposits, often involves the skin,gastrointestinal tract, and glomerulus, and frequently causes arthritis and nephritis. In adults and especially in elderly people,IgA vasculitis is more likely to manifest large purpura, ulcerative skin lesions, and chronic renal insufficiency than in children. We describe an 81-year-old female with IgA vasculitis who presented with multiple ulcers on both legs. A direct immunofluorescence analysis revealed the deposition of IgA, IgM and C3 on the vascular wall. Treatment with oral prednisolone was started but it took approximately 3 months for the ulcers to epithelize and the process of recovery from the renal disorder was also protracted. Although thrombus formation in the dermal blood vessels was seen histologically, antiphosphatidylserine/prothrombin autoantibodies were not detected. The protracted healing of the ulcers was thought to be caused by more than one factor such as a secondary infection along with tissue fragility and phlebostasis due to aging.

An 83-year-old woman had complained of pain of her genital region for one month. She was suspected of having a malignant tumor at a nearby clinic and was admitted to our hospital for further examination. At her first visit, verrucous vegetation was seen on the entire genital region. After a limited time, erosions at the axilla and lips and thickening of the tongue also appeared. Histologically, acanthosis and a microabscess containing numerous eosinophils were observed at the lesional epidermis. Direct immunofluorescence analysis revealed the deposition of IgG at keratinocyte surfaces. Her serum levels of anti-desmoglein (Dsg) 1 and anti-Dsg3 antibodies were elevated. Based on clinical, histological, and laboratory findings, this case was diagnosed as Hallopeau type pemphigus vegetans. Although she developed a widespread erosion that was not well controlled with oral corticosteroids only, the erosion was controlled by combining corticosteroids with high-dose intravenous immunoglobulin therapy.

A 79-year-old female who had suffered from asymptomatic, annular, hyperkeratotic, brownish macules on the extremities and trunk for 7 years spontaneously developed pruritic erythematous changes of the pre-existing lesions as well as newly formed erythematous papules. A histopathological examination showed typical cornoid lamella and a perivascular infiltration of lymphocytes and eosinophils in the upper dermis. Laboratory investigations revealed eosinophilia. After an inflammatory period lasting for 16 months, the pruritic erythematous changes and the eosinophilia gradually improved, leaving annular, brownish, mildly hyperkeratotic macules. Our case was unusual for its transient inflammatory condition of disseminated superficial porokeratosis (DSP), which was consistent with the previously reported “Inflammatory stage of DSP”.

A 68-year-old man presented to our hospital with a gradually enlarging subcutaneous mass in the posterior neck. At initial examination, there was a soft subcutaneous tumor with a diameter of 15 cm in the nuchal region, and multiple soft subcutaneous tumors with unclear borders symmetrically distributed on the trunk and limbs. Microscopic examinations of the tumor revealed mature adipocyte proliferation. Based on the microscopic findings and a history of alcoholic liver cirrhosis / hyperuricemia, the patient was diagnosed with benign symmetric lipomatosis. Benign symmetric lipomatosis is a disease in which adipose tissue proliferates bilaterally and symmetrically, mainly in the neck, trunk, and proximal extremities, and is known to be associated with alcoholic liver injury, impaired glucose tolerance, hyperlipidemia, hyperuricemia. In this study,we reviewed 125 Japanese cases, including our present case. The tumors arise predominantly in 50s and 60s men, with men overwhelmingly represented at 85%. The tumors predominantly distributed on the upper body sites, such as the neck, back and upper arms. Although there are many recurrences, surgical excision may be performed to relieve pressure on other organs or for cosmetic reasons. The pathogenesis of this disease has not been elucidated and there is no established treatment.

A 49-year-old Japanese woman visited our clinic for the evaluation of a slowly progressing, well-circumscribed subcutaneous tumor on her back with a 3-year history. The tumor was excised under local anesthesia. A histopathological examination revealed that the cyst consisted of keratinous material covered by a squamous epithelium with a granular layer in the dermis. Moreover, focal ossification was observed beside the cyst wall. Epidermal cysts are common skin tumors composed of a cyst wall formed from epidermal cells and keratinous material. However, it is rare for epidermal cysts to be accompanied by ossification.

A 93-year-old man had had multiple brown spots with keratinization on his face, trunk, and extremities for 30 years and was diagnosed with porokeratosis. Some erosions appeared on his brown spots 3 years ago. He was referred to our hospital because of nodular lesions that arose on the erosions. There were multiple brown keratotic plaques on his skin throughout the body with some erosions on the abdomen and two red nodules on the left forearm and the right leg. A histological examination of the brown plaques revealed the formation of cornoid lamellae. At the sites of the erosions and red nodules, the proliferation of atypical keratinocytes in the epidermis and their invasion into the upper dermis were observed. We diagnosed this patient as disseminated superficial porokeratosis with multiple squamous cell carcinoma and Bowen's disease. Some cases of porokeratosis show autosomal-dominant inheritance ; however, most cases are sporadic. When we examine a patient with porokeratosis, it is necessary to follow them up regularly and carefully observe them for malignant skin lesions throughout the whole body.

An 88-year-old woman presented with a red nodular lesion on her left cheek. Histologic examination revealed the proliferation of small round tumor cells in an alveolar configuration. Immunohistochemically, the tumor cells were positive for CK20, chromogranin A, and synaptophysin. Thus, we diagnosed the tumor as Merkel cell carcinoma. In other 2 cases, a 77- year-old man presented with a red nodular lesion on his right chin and an 83-year-old man presented with a red nodular lesion on his anterior chest. The lesions presented similar pathological and immunohistochemical findings and were both diagnosed as Merkel cell carcinoma. On dermoscopic examination, all 3 cases presented polymorphous vessels including linear irregular vessels, milky red areas, and nonshiny white areas. The dermoscopic image of Merkel cell carcinoma is variegated and there is no disease-specific finding. However, we can frequently observe the following demoscopic findings in Merkel cell carcinoma : 1) various vascular structures, 2) stractural abnomalities, and 3) white areas that refrect tissue fibrosis. Dermoscopy should be an useful non-invasive diagnostic method in Merkel cell carcinoma.

A 75-year-old woman presented with a red hemorrhagic tumor on her right heel in 2013. The tumor was surgically excised in 2014. Then she was admitted to our hospital with the diagnosis of malignant melanoma. After a wide excision of the primary tumor, pelvic-inguinal lymph node dissection, and DAVFeron (dacarbazine, nimustine, vincristine, and interferon-β) therapy, skin, liver, lungs, and pelvic bone mestases were appeared in 2015. Nivolumab administration was started to treat the metastases, but its effect was poor in limiting tumor progression and vemurafenib administration was started. The metastases in the skin, lungs, liver, and pelvis rapidly regressed, but some bone metastases remained. At three months after the administration, disorientation, loss of appetite, and fluctuations appeared. Computed tomography and magnetic resonance imaging (MRI) of the head showed no abnormal findings. At four months after administration, loss of consciousness appeared and cancer meningitis and brain metastasis were diagnosed by contrast-enhanced MRIand cerebrospinal fluid cell examination. She died two weeks after admission. Because vemurafenib hardly reaches the brain, it has little effect on central nervous system metastases. In addition, the brain metastasis was not detected by non-contrast-enhanced MRI in this case because of the non-pigmented type of malignant melanoma. Therefore, the use of contrast-enhanced MRI was necessary for detecting the brain metastasis.