SummaryEpidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies to type VII collagen, a major component of anchoring fibrils. Most patients with EBA are adult and develop autoantibodies to the non-collagenous 1 (NC-1) domain of type VII collagen.In this report, we describe a 4-year-old Japanese boy presenting pruritic vesicles and tense blisters over his whole body.Immunofluorescence studies revealed linear IgG/C3 deposits along the dermal-epidermal junction of the patient's skin, and circulating IgG autoantibodies mapping to the dermal side of 1M NaCl-split skin. By immunoblotting analysis using dermal extracts as a substrate, the patient's IgG antibodies labeled a 290 kD protein corresponding to type VII collagen.Immunoblotting studies using recombinant proteins demonstrated that the patient's circulating autoantibodies recognized not only the NC-1 but also the non-collagenous 2 (NC-2) domain of type VII procollagen.Review of the previously reported cases and the present case suggested that EBA cases with autoantibodies to regions other than the NC-1 domain are all children younger than 10 years of age with clinical features of an inflammatory phenotype.2
A case of hidradenoma papilliferum with mixed features of syringocystadenoma papilliferum (SCAP) and anogenital mammary-like glands is reported. A single, fresh red-colored nodule developed in the sulcus between the labia majora and minora of a 49-year-old Japanese woman. Histopathologically, the tumor showed epithelial lining with apocrine secretion and slight connective tissues characteristics. Our case was unique because, like SCAP, the tumor was connected to the epidermis and cystic invaginations extended downward into the deep dermis. In addition, beneath the tumor, tubular structures that resembled normal mammary tissue were present in the subcutaneous fatty tissue. In this study, it has been suggested that this tumor might have been developed from these mammary-like glands.
The first case of primary cutaneous squamous cell carcinoma (SCC) to cause zosteriform and epidermotropic metastasis to skin is reported. The patient is a 72-year-old Japanese woman. A cutaneous SCC appeared on the lateral side of her right knee and was removed. After dissection of the right inguinal lymph nodes, which revealed metastases, and irradiation of the right inguinal region, the patient presented with slightly pruritic and painful erythematous papules on the right hip and small brownish papules and vesicles with crusts on the anterior side of the right thigh. The eruptions were in a zosteriform distribution along the right L1 to L3 dermatomes. Histologically neoplastic squamous cell nests were observed in the epidermis, below the epidermal-dermal junction, and within lymphatic vessels in the deeper reticular dermis. We postulate that neoplastic cells with the ability to fuse with adjacent squamous epithelium may have been carried beneath the basal lamina or to the epidermis via dermal lymphatic backflow, resulting in epidermotropic metastasis.
Human T-lymphotropic virus-1 (HTLV-1) is considered to be the cause of adult T-cell leukaemia-lymphoma (ATL). Monoclonal integration of HTLV-1 proviral DNA, as is analysed by Southern blotting, has been demonstrated in ATL patients. Unusual integration patterns of HTLV-1 proviral DNA have occasionally been described, and it is suggested that the patterns have clinical implications for ATL pathophysiology. Multiple, complete and defective types of integration patterns, in that order, are apparently associated with prognoses from good to poor. We report a 73-year-old Japanese woman with lymphoma-type ATL and a bulky cutaneous tumour on the left thigh. Four bands of slightly differing intensity were seen after EcoRI digestion of skin and lymph node samples on Southern blot analysis of HTLV-1 proviral DNA. Analysis for T-cell receptor-beta gene revealed five novel bands after restriction enzyme digestion with HindIII, indicating that the patient has four separate tumour cell clones, each of which carries one copy of the provirus. She was treated with chemotherapy and radiation and remains under reasonable control despite some relapsing cutaneous nodules. The indolent course in this present case could be related to the multiple integration pattern of HTLV-1 proviral DNA detected.
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