Satoshi Kore-Eda

Basal cell carcinoma cells resemble follicular matrix cells rather than follicular bulge cells: immunohistochemical and ultrastructural comparative studies.

To detail the histogenetic relationship between basal cell carcinoma (BCC) and hair follicles, we immunohistochemically compared BCC cells to follicular matrix cells and follicular bulge cells using a panel of monoclonal antibodies against melanocytes, cytokeratins, subepidermal extracellular matrix components, and bullous pemphigoid (BP) sera, as well as using electron microscopy. Cytokeratin expression patterns were not consistent with the variety in types of cytokeratins and in cases of BCC. The distribution of some extracellular matrix components was not only linear along the interfaces of BCC tumor nests and stroma, and follicular matrix and follicular papilla; granular deposits were also seen in the stroma and follicular papilla, whereas they were only linearly distributed along the follicular bulge. The BP antigens and integrin alpha 6, which were absent in BCC and follicular matrix, were expressed in the follicular bulge area. Electron microscopically, hemidesmosomes were poorly organized in these three tissues, but the lamina densa was incomplete in BCC and follicular matrix, whereas the lamina densa in the follicular bulge area was continuous. These morphologic similarities between BCC and follicular matrix cells, and coexistence of melanocytes in the BCC tumor nest strongly suggest the differentiation of BCC toward the follicular matrix cells.

Perturbations of both nonregulatory and regulatory FOXP3+ T cells in patients with malignant melanoma

Background  ‘FOXP3+ regulatory T cells’ (Tregs) are reported to be increased in tumour‐bearing hosts including patients with melanoma, leading to tumour immune suppression. However, this idea is challenged by recent evidence that the ‘FOXP3+ Treg’ fraction in fact contains activated ‘nonregulatory’ T cells. Also, FOXP3+ T cells are reported to have functionally and kinetically distinct subsets.

Focal dermal hypoplasia (Goltz syndrome) associated with multiple giant papillomas

We report a case of focal dermal hypoplasia (FDH) with multiple giant papillomas on nonperimucosal areas. The patient had had cribriform hyperpigmented and depigmented plaques on the trunk and extremities since birth. There were also hypoplastic skin lesions on the right arm, left elbow and right thigh. The multiple giant papillomas began to appear. when she was 22 years old, on the trunk and extremities. Cryotherapy was effective in controlling them.

Adult-onset Multiple Eccrine Angiomatous Hamartoma in Enlarging Hairy Plaques

Sir, Eccrine angiomatous hamartoma (EAH) is a rare benign hamartoma of eccrine glands and blood vessels. It most commonly develops before adulthood as a single, slowgrowing lesion on the extremities. We describe here a case of adult-onset EAH with generalized multiple hairy reddish-brown indurated plaques with a rapidly progressive course. As the lesions showed histopathological findings such as an increase in mature eccrine glands and blood vessels, the diagnosis was determined to be EAH. This case was observed carefully because the lesion had characteristics not only of a hamartoma but also of a tumour.

Malignant melanoma derived from cerebriform intradermal naevus.

We report a case of malignant melanoma (MM) derived from cerebriform intradermal naevus (CIN) in a 66‐year‐old Japanese man. The patient had cutis verticis gyrata (CVG) on the posterior area of the scalp at birth. He noticed a dome‐shaped nodule at the centre of the CVG at 66 years of age. Histopathological examination found a nodule of MM arising within an extensive area of intradermal naevus. There was no metastasis to lymph nodes or other organs. To our knowledge, only two cases of CIN in which MM had later developed have been reported. We estimated that the incidence of melanoma from CIN including our case is 4.5% (3 of 67 reported cases), which seems to be comparable to the frequency of malignant alteration of giant pigmented naevi. This suggests that pathological examination is recommended for CVG, and once pathological diagnosis of CIN is confirmed, long clinical follow‐ups are necessary for detecting development of MM.

A Case of Autoimmune Bullous Dermatosis with Features of Pemphigus Vulgaris and Bullous Pemphigoid

Pleomorphic blisters, including tense bullae and annularly arranged vesicles around the erythema as well as erosive eruptions in the oral cavity, appeared on a 61-year-old woman 5 years after surgery for cholangiocellular carcinoma. A biopsy specimen from the oral cavity showed intraepidermal blisters, and those from skin lesions showed subepidermal blisters with infiltrates of eosino-phils and neutrophils. The early-stage vesicles showed infiltrates along the epidermal-dermal junction, where electron microscopy disclosed disruption of the lamina densa, basal cells remaining on the dermis, and acan-tholytic keratinocytes among the infiltrates, but there was no cleavage of the epidermal-dermal junction at the lamina lucida. Direct immunofluorescence studies showed immune deposition at the intercellular space (ICS) and along the basement membrane zone (BMZ). Indirect immunofluorescence studies confirmed coexistence of IgG class anti-ICS and anti-BMZ antibodies. Although this case showed immunohistochemical features of bullous pemphigoid, the presence of suprabasal cleavage in the oral mucosa, acantholytic cells in the blister cavity, the deposition of IgG at the ICS of the perilesional epidermis, and circulating anti-ICS antibodies strongly suggested that this case was primarily pemphigus. The strong inflammation along the epidermal-dermal junction due to unknown factors may have modified the clinical appearance and the histopathology.

Syringocystadenocarcinoma Papilliferum in the Perianal Area

Syringocystadenocarcinoma papilliferum (SCACP) is a very rare cutaneous adnexal neoplasm. SCACP presents histologic variability, and it is difficult to establish the diagnosis from a punch biopsy. SCACP has an overall configuration similar to that of syringocystadenoma papilliferum (SCAP). When we diagnose SCACP, the histologic features of SCAP can be contributing and immunohistochemical staining is useful. Our case shows the histologic variability of SCACP and the pitfalls of a punch biopsy for the diagnosis of SCACP.

Large cell transformation mimicking regional lymphomatoid papulosis in a patient with mycosis fungoides.

A 57‐year‐old Japanese man with tumor‐stage mycosis fungoides suddenly presented multiple small papules on the right chest. Histopathology of a biopsy specimen from the papules revealed medium‐to‐large pleomorphic lymphoid cells throughout the entire dermis but not in the epidermis, and the large cells expressed CD30 antigen. These newly‐developed papules underwent spontaneous remission in the following 3 months. We reviewed the reported cases of mycosis fungoides, which showed CD30‐positive large cell transformation and those of CD30‐positive lymphoproliferative disorders associated with mycosis fungoides.

Mosaic expression of uncein and 180-kDa bullous pemphigoid antigen in generalized atrophic benign epidermolysis bullosa

Immunofluorescence microscopy of epidermodermal junction components in serial cryosections from the perilesional skin of a patient with generalized atrophic benign epidermolysis bullosa (GABEB) showed broken line‐like staining of both BPAG2 (180‐kDa bullous pemphigoid antigen) and uncein (antigen of 19‐DEJ‐l monoclonal antibody), whereas integrin α6 and laminin 5 were continuously expressed along the basement membrane zone. Immunoelectron microscopy revealed a mosaic distribution of the BPAG2/uncein positive and negative cells. BPAG2, a candidate protein of GABEB, probably has a close connection with uncein, an anchoring filament component.

Reactivation of herpes simplex virus and cytomegalovirus in a case of thymoma-associated graft-versus-host disease-like erythroderma.

a 64-year-old woman with a 4-year history of pruritic erythema was admitted to our hospital because of aggravation of the erythema that did not respond to 20 mg/ day oral prednisolone. The patient had had malignant thymoma and myasthenia gravis for 12 years, together with recurrent oral herpes infection for several years. In spite of thymectomy, chemotherapy, and radiation therapy, malignant thymoma was disseminated to the thoracic cavity and no more treatment options could be utilized on admission. The patient presented with erythema that was scaly, mildly keratotic, and distributed over the face, body trunk, and extremities (Fig. 1a). The patient reported severe pruritus. Biopsy specimen of the erythema revealed superficial perivascular dermatitis with parakeratosis, hypogranulosis, dyskeratosis with satellite cell necrosis, mild acanthosis, and vacuolar change in the epidermis, and moderate infiltration of lymphocytes in the upper dermis (Fig. 1b). Direct immunofluorescence was negative (data not shown). Immunohistochemistry revealed that there were more CD8+ T cells than CD4+ T cells infiltrated in the epidermis and dermis (Fig. 1c, d). Differential diagnoses included psoriasis, lichen planus, pityriasis rosea, lichenoid drug eruption, pityriasis lichenoides chronica, and thymoma-associated GVHD-like erythroderma. psoriasis was ruled out by the presence of dyskeratosis, and parakeratosis is not usually observed in lichen planus. The clinical course was too long for pityriasis rosea, and lichenoid drug eruption could be ruled out because of the absence of eosinophils in the dermis. Furthermore, pityriasis lichenoides chronica is not usually pruritic and the eruptions are more oval than those of our case. Based on the characteristic distribution and clinical features of the erythema and pathological findings, a diagnosis of thymoma-associated GVHD-like erythroderma was made. potent topical steroid (clobetasol propionate 0.05%), systemic steroid (prednisolone 2 mg/kg/day), and cyclosporine (5 mg/kg/day) were prescribed for 2 weeks, but did not control the erythema (Fig. s1; available from http://www.medicaljournals.se/acta/content/?d oi=10.2340/00015555-1577). Intriguingly, after the initiation of oral acyclovir (15 mg/kg/day) for exacerbated oral herpes infection, the erythema began to subside. acyclovir was then switched to valacyclovir hydrochloride (15 mg/kg/day). Incidentally, we detected a high level of CMV antigenemia of up to 1,564 cells/slide, although it was only 11 cells/slide 6 months previously. The patient did not report any symptoms of retinitis, pneumonia, or colitis, thus we administered ganciclovir (5 mg/kg/day) and discontinued valacyclovir. The erythema recurred when prednisolone was tapered to 0.75 mg/kg/day; therefore, prednisolone was increased up to 1 mg/kg/day, which was not successful. Next, broadband ultraviolet Reactivation of Herpes Simplex Virus and Cytomegalovirus in a Case of Thymoma-associated Graft-versus-host Disease-like Erythroderma