Erina Homma

Response of intractable skin ulcers in recessive dystrophic epidermolysis bullosa patients to an allogeneic cultured dermal substitute.

Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited skin disorder caused by mutations in the COL7A1 gene, which encodes collagen VII (COL7). Skin ulcers in RDEB patients are sometimes slow to heal. We describe here the therapeutic response of intractable skin ulcers in two patients with generalized RDEB to treatment with an allogeneic cultured dermal substitute (CDS). Skin ulcers in both patients epithelialized by 3-4 weeks after this treatment. Immunohistochemical studies demonstrated that the COL7 expression level remained reduced with respect to the control skin and that it did not differ significantly between graft-treated and untreated areas. Electron microscopy showed aberrant anchoring fibrils beneath the lamina densa of both specimens. In conclusion, CDS is a promising modality for treatment of intractable skin ulcers in patients with RDEB, even though it does not appear to increase COL7 expression.

Sequential local injection of low-dose interferon-beta for maintenance therapy in stage II and III melanoma: a single-institution matched case-control study.

Objective: To determine the beneficial effect of maintenance therapy in stage II and III melanoma by sequential local injection of low-dose interferon-β. Methods: We reviewed 46 patients with stage II and III primary melanoma at our institution from 2004 through 2009. Twenty-one patients were treated with interferon-β maintenance therapy consisting of subcutaneous injection of natural interferon-β at a dose of 3 × 106 IU/day for 10 consecutive days, and 25 patients underwent observation alone. Results: Compared with all patients, overall survival and relapse-free survival were significantly worse in the observation group than in the interferon-β group (p = 0.024 and 0.029, respectively). In stage II, a significant difference in overall survival, but not in relapse-free survival, was seen between the two groups (p = 0.041). When the interferon-β group was stratified by subgroup, there was a statistical difference only between dosage and duration (p = 0.027 and p < 0.001, respectively). Conclusions: This study demonstrates that maintenance therapy by interferon-β is beneficial in the outcome of the disease without substantial toxic effects, especially in patients with stage II melanoma. Extension of the duration of treatment beyond 2 years could further improve the therapeutic efficacy of interferon-β.

Controlling the histological margin for non‐melanoma skin cancer conveniently using a double‐bladed scalpel

In some countries, intraoperative histological evaluation to control the surgical margin for non‐melanoma skin cancer is widely used instead of Mohs micrographic surgery. Nevertheless, this evaluation by frozen section analysis is usually limited to suspicious areas.

MUC5AC expression correlates with invasiveness and progression of extramammary Paget's disease

Patients with in situ extramammary Pagets disease (EMPD) tend to have a good prognosis, although dermal invasion and metastasis are associated with significantly increased morbidity and mortality. Previous studies have addressed mechanisms underlying the EMPD pathogenesis; however, no molecular markers that reflect invasiveness or progression have been established.

Bowen's disease of the nail matrix presenting as melanonychia: detection of human papillomavirus type 56.

© 2009 The Authors. doi: 10.2340/00015555-0723 Journal Compilation

Warty intralymphatic histiocytosis successfully treated with topical tacrolimus.

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Ultrasound B-mode and elastographic findings of angiomatoid fibrous histiocytoma

ezary syndrome T-cell clones display T-helper 2 cytokines and express the accessory factor-1 (interferon-gamma receptor betachain). Blood 1996; 88: 1383–9. 5 Sugaya M, Miyagaki T, Ohmatsu H et al. Association of the numbers of CD163(+) cells in lesional skin and serum levels of soluble CD163 with disease progression of cutaneous T cell lymphoma. J Dermatol Sci 2012; 68: 45–51. Ultrasound B-mode and elastographic findings of angiomatoid fibrous histiocytoma

Squamous Cell Carcinoma in a Chronic Genital Ulcer in Behçet's Disease

© 2010 The Authors. doi: 10.2340/00015555-0902 Journal Compilation

Squamous cell carcinoma of the auricle with rhabdoid features

To the Editor, Squamous cell carcinoma (SCC) is one of the most common malignancies of the external ear. It frequently occurs in elderly people and is associated with a higher metastasis rate, compared to carcinoma found in other locations.1 As prognoses differ significantly among cutaneous SCC variants,2 histopathological patterns of individual cases must be carefully examined. Cutaneous SCC can be classified into several histologic subtypes, differing in prognostic significance.2 A low-risk subtype includes verrucous SCC and those arising in actinic keratosis. An intermediate-risk subtype consists of adenoid SCC and lymphoepithelioma-like carcinoma. Finally, SCC arising from scars, Bowen’s disease with invasion and adenosquamous carcinoma constitute a high-risk subtype. Other rare subtypes, of which a prognosis is not well established, include signet ring cell SCC, follicular SCC, papillary SCC, pigmented SCC, and clear cell SCC. Further studies are required to define the biological behavior and establish a prognostic indication of these rare subtypes of SCC. Cutaneous SCC with a rhabdoid phenotype is histologically characterized by large cytoplasmic eosinophilic hyaline inclusions with peripheral displacement of vesicular nuclei. Immunohistochemically, these tumor cells show diffused reactivity for cytokeratin and vimentin, which are localized to the cytoplasmic inclusions, but usually not for desmin, smooth muscle actin, or other skeletal muscle markers. To the best of our knowledge, only a few cases of cutaneous SCC with such rhabdoid features have been reported in the literature.3– 6 An 88-year-old Japanese man presented with an ulcerated reddish colored tumor on the right ear. The tumor, which initially appeared as a small, crusted nodule 2 years ago, had slowly enlarged in size. Bleeding had been occurring from the surface of the tumor for the past 6 months. The past medical history indicates that the patient had been diagnosed with a cerebral infarction and dementia for 3 years. The patient has been taking an anticoagulant agent without any immunosuppressive drug. Physical examination revealed an erosive, fresh-reddish colored, 37 × 25 × 14−mm sized nodule on the right post-auricular shaded area (Fig. 1A). No evidence of regional lymphadenopathy was reported at the time of presentation. The histological examination of an incision biopsy initially led to the diagnosis of pyogenic granuloma with fibrosis. However, as the small biopsy specimen was taken from a relatively shallow region, the possibility that the nodule represented a case of undifferentiated SCC, could not be completely excluded. Therefore, the lesion was excised widely including cartilage to test for possible malignancy. The surgical specimen showed dense, dermal and subcutaneous neoplastic proliferation (Fig. 1B) of poorly differentiated tumor cells with numerous plasma cells and histiocytes. The tumor cells consisted predominantly of monomorphic polygonal neoplastic cells, arranged in focally solid sheets (Fig. 2A), particularly in the center of the nodule. The cells possessed abundant eosinophilic, cytoplasmic inclusions with peripheral displacement of the nuclei, charactereristic of the rhabdoid phenotype (Fig. 2B). Vascular invasion and lymphatic permeation were not observed. Actinic keratosis in the overlying epidermis was identified only at the border of the nodule as the epidermis was extensively ulcerated. The tumor cells were tested positive for vimentin and cytokeratin AE1/AE3, but negative for S-100, desmin or smooth muscle actin (SMA). Interestingly, the area tested positive for cytokeratin AE1/AE3 was localized only to the center portion of the lesion (Fig. 2C). One year after his surgery, the patient remains alive and has not developed any metastatic or recurrent lesions. Since malignant rhabdoid tumor of the kidney was first described,7 additional, extra-renal cases have been reported. These cases were regarded as a variant of rhabdomyosarcomatous tumor, based on

Dermoscopy of periungual pigmented Bowen's disease: its usefulness in differentiation from malignant melanoma

1 Laubach H-J, Tannous Z, Anderson RR, Manstein D. Skin responses to fractional photothermolysis. Lasers Surg Med 2006; 38: 142–149. 2 Orringer JS, Ritti e L, Baker D, Voorhees JJ, Fisher G.Molecular mechanisms of nonablative fractionated laser resurfacing. Br J Dermatol 2010; 163: 757–768. 3 Taudorf EH, Danielsen PL, Paulsen IF et al. Non-ablative fractional laser provides long-term improvement of mature burn scars-A randomized controlled trial with histological assessment. Lasers Surg Med 2014. doi:10.1002/lsm.22289 [Epub ahead of print]. 4 Tierney E, Mahmoud BH, Srivastava D, Ozog D, Kouba DJ. Treatment of surgical scars with nonablative fractional laser versus pulsed dye laser: a randomized controlled trial. Dermatol Surg 2009; 35: 1172–1180. 5 Hedelund L, Moreau KER, Beyer DM, Nymann P, Haedersdal M. Fractional nonablative 1,540-nm laser resurfacing of atrophic acne scars. A randomized controlled trial with blinded response evaluation. Lasers Med Sci 2010; 25: 749–754. 6 Kim D-W, Hwang N-H, Yoon E-S, Dhong E-S, Park S-H. Outcomes of ablative fractional laser scar treatment. J Plast Surg Hand Surg 2014. doi:10.3109/2000656X.2014.919927 [Epub ahead of print]. 7 Park KY, Oh IY, Seo SJ, Kang KH, Park SJ. Appropriate timing for thyroidectomy scar treatment using a 1,550-nm fractional erbium-glass laser. Dermatol Surg 2013; 39: 1827–1834. 8 Conologue TD, Norwood C. Treatment of surgical scars with the cryogen-cooled 595 nm pulsed dye laser starting on the day of suture removal. Dermatol Surg 2006; 32: 13–20. 9 Capon A, Iarmarcovai G, Mordon S. Laser-assisted skin healing (LASH) in hypertrophic scar revision. J Cosmet Laser Ther 2009; 11: 220–223. 10 Capon A, Iarmarcovai G, Gonnelli D, Degardin N, Magalon G, Mordon S. Scar prevention using Laser-Assisted Skin Healing (LASH) in plastic surgery. Aesthetic Plast Surg 2010; 34: 438–446.