Morsicatio labiorum is a form of tissue alteration caused by self-induced injury, mostly occurring on the lips, and is considered to be a rarely encountered mucocutaneous disorder. Clinically, it is a macerated grey-white patch and plaque of the mucosa caused by external stimuli (self-induced injury) such as habitual biting, chewing, or sucking of the lip. It is often confused with other dermatological disorders involving the oral mucosa, which can lead to a misdiagnosis. We herein report three cases of morsicatio labiorum; two cases were misdiagnosed as exfoliative cheilitis at the time of the first visit.

Dowling-Degos disease (DDD) is an autosomal dominant genodermatosis and this disease is a genetically determined disturbance of epidermal proliferation. It is characterized by acquired, slowly progressive pigmented lesions that primarily involve the great skin folds and flexural areas such as the axilla, neck, limb flexures, the inframammary area and the inguinal folds. The vulva is an unusual location for DDD. A 41-year-old woman presented with a 10-year history of multiple, small, reticulated and brownish macules distributed symmetrically on the bilateral external genital regions. We found no other similarly pigmented skin lesions on her body, including the flexural areas. There was no known family history of similar eruptions or pigmentary changes. The histologic examination showed irregular rete ridge elongation with a filiform or antler-like pattern and basilar hyperpigmentation on the tips. Fontana-Masson staining showed increased pigmentation of the rete ridges and the S100 protein staining did not reveal an increased number of melanocytes in the epidermis. From these findings, we diagnosed this lesion as DDD.

Myopericytoma is a benign neoplasm consisting of cells that appear to have a distinct differentiation towards presumed perivascular myoid cells. Amongst myopericytoma, an intravascular variant appears to have been reported only rarely. A 67-year-old man presented with a 15-year history of a painful, slow growing 3 × 3.5 cm sized mass in the subcutis of his right lateral thigh. Histopathological studies showed a subcutaneous mass entirely within the lumen of a vein. The tumor was composed of spindle-shaped myoid-appearing cells in a concentric arrangement, intimately associated with thin-walled vascular channels. Tumor cells were diffusely positive for smooth muscle actin, focally positive for CD34, and negative for desmin and CD31. From these findings, we diagnosed this lesion as intravascular myopericytoma. Unlike previous reports, our case showed a relatively large painful subcutaneous mass, although this tumor has an intravascular nature.

BackgroundOur aim was to investigate the clinical pattern of hemophagocytic lymphohistiocytosis following Kawasaki disease (HLH-KD), to enable differentiation of HLH from recurrent or refractory KD and facilitate early diagnosis.MethodsWe performed a nationwide retrospective survey and reviewed the clinical characteristics of patients with HLH-KD, including the interval between KD and HLH, clinical and laboratory findings, treatment responses, and outcomes, and compared them with historical data for both diseases.ResultsTwelve patients with HLH-KD, including 5 previously reported cases, were recruited. The median age was 6.5 years (range, 9 months-14.7 years). Eight patients were male and 4 were female. The median interval between the first episode of KD and the second visit with recurrent fever was 12 days (3-22 days). Of the 12 children, 2 were initially treated with intravenous IgG (IVIG) for recurrent KD when they presented at the hospital with recurrent fever. Eventually, 10 children received chemotherapy under an HLH protocol and 2 received supportive treatment. Two patients died of combined infections during chemotherapy, 1 was lost to follow up, and 9 remain alive. The overall survival rate at 4 years was 81.1% with a median follow up of 45.1 months.ConclusionA diagnosis of HLH-KD should be considered when symptoms similar to recurrent KD develop within 1 month of the first episode of KD. Our findings will help physicians differentiate between HLH and the recurrent form of KD.

Diphenylcyclopropenone immunotherapy can be used effectively for both children/adolescents and adults. The time required for complete cure is shorter in children/adolescents than in adults.