Ayumi Takahashi

Antiphospholipid Antibody Syndrome Complicated by Grave's Disease

The report describes a woman with primary antiphospholipid antibody syndrome complicated with Graves disease. Developing symptoms included a small cutaneous nodule on her finger and subsequently ecchymotic purpura on the cheeks, ears, buttocks and lower legs. Histological examinations showed thrombosed vessels in the dermis without or with hemorrhage, respectively. Laboratory investigation revealed positive lupus anticoagulant and immunogenic hyperthyroidism due to Graves disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Graves disease and a possible link of antiphospholipid antibody syndrome with Graves disease was suggested both by the etiology of the disease as well as the disease activity.

Cutaneous papules and nodules in the diagnosis of the antiphospholipid syndrome

Of 11 patients with primary or secondary antiphospholipid syndrome (APS), four exhibited papules or nodules on the finger, sole or leg as the initial cutaneous manifestation. Histological examination demonstrated thrombosed vessels or vessels containing organized thrombi in the dermis or in the subcutaneous fat tissue. Cutaneous papules and nodules should be recognized as skin manifestations of APS. Screening tests for antiphospholipid antibodies and lupus anticoagulant are required in patients with cutaneous papules or nodules of unknown aetiology. In cases of positive antiphospholipid antibodies and/or lupus anticoagulant, histological examination is critical in the establishment of the diagnosis of APS.

Prognostic significance of L-type amino acid transporter 1 (LAT1) expression in cutaneous melanoma.

Amino acid transporters play a crucial role in the development and invasiveness of cancer cells. However, it remains unclear whether or not the expression of L-type amino acid transporter 1 (LAT1) has prognostic significance in patients with cutaneous melanoma. A total of 128 patients with cutaneous melanoma were evaluated. Tumor sections were stained by immunohistochemistry for LAT1, CD98, Ki-67, and microvessel density determined by CD34 and p53. We also analyzed 30 specimens of patients with melanocytic nevi as negative controls. LAT1 and CD98 were highly expressed in 58% (75/128) and 75% (97/128), respectively. The rates of positivity for LAT1 in the melanocytic nevi were 0% (0/30). The expression of LAT1 was associated significantly with tumor thickness, T factor, CD98 expression, cell proliferation (Ki-67), and microvessel density (CD34). By Spearman’s rank test, LAT1 expression was correlated with CD98, Ki-67, and CD34. By univariate analysis, tumor thickness, ulceration, disease staging, LAT1, and CD34 showed a significant relationship with overall survival and disease-free survival. Furthermore, a multivariate analysis confirmed that LAT1 was an independent prognostic factor for predicting a poor prognosis. This study had a small sample size. LAT1 can serve as a significant prognostic factor to predict a poor outcome and it may therefore play an important role in the aggressiveness of cutaneous melanoma.

Use of a reverse-flow plantar marginal septum cutaneous island flap for repair of a forefoot defect

Soft-tissue reconstruction of the foot frequently requires flap coverage to preserve exposed tissues such as bones, joints, tendons, and weight bearing areas. Recently, reconstruction of skin defects in the heel has been greatly improved by use of the medial plantar sensory flap. However, forefoot coverage remains a challenge, since the alternatives for flap coverage are very limited. A case report is presented of a 52-year-old man with a chronic forefoot ulcer, successfully covered with a plantar marginal septum cutaneous island flap. This recently reported technique, which utilizes a reversed-flow flap based on the superficial branch of the medial division of the medial plantar artery, is relatively easy and disadvantages to the donor site are minimal.

Bowen's disease involving the urethra.

Bowens disease developing on mucous or mucocutaneous regions is clinically called erythroplasia of Queyrat. We report herein a 56‐year‐old male with Bowens disease extending from the penis shaft to the glans penis, and urethral meatus. Physical examination revealed bright red velvety plaques on the prepuce and glans penis and an irregularly pigmented scaly lesion on the dorsum of his penis shaft. Histopathological findings of both lesions were compatible with those of Bowens disease, supporting the concept that erythroplasia of Queyrat and Bowens disease should be regarded as one clinicopathologic entity. A partial penectomy was finally performed, because tumor cells were pathologically observed in the mucous epithelium of the urethra. Although several therapeutic modalities exist for Bowens disease on the external genitalia, treatment options are limited when Bowens disease extends to the urethral meatus. We discussed the recent therapeutic modalities in genital Bowens disease.

A case of fibro‐osseous pseudotumour of the digits

Sir, Topical 5-aminolaevulinic acid (5-ALA) photodynamic therapy (PDT) has become an effective treatment option in dermatology. We report a 70-year-old man who presented with extensive erythematous patches on his abdomen and infiltrated plaques on his arms and neck. These were preceded by extensive figurate and annular erythema on his trunk and limbs for 10 years. He had a history of congestive cardiac failure, atrial fibrillation and chronic obstructive airway disease. Mycosis fungoides (MF) was confirmed histopathologically and he cleared with a course of oral 8-methoxypsoralen (8-MOP) plus ultraviolet A (PUVA). During the next year his MF progressed in extent and he also developed a solitary tumour (1 ́5 1 ́5 cm) on his left posterior thigh. The extensive patch and plaque MF cleared with a further course of oral 8-MOP PUVA, but the nodule remained and became ulcerated and painful over the next 3 months. Biopsy of this tumour showed an ulcerated epidermis with replacement of the subcutaneous tissue by a dense infiltrate of malignant CD31 T cells (Fig. 1a). There was no lymphadenopathy or organomegaly. Blood investigations were normal. Computed tomographic scans of the thorax and abdomen were negative. The tumour was treated with 5-ALA PDT: 5-ALA 20% in a cream base (Aladerm, Crawford Pharmaceuticals, Milton Keynes, U.K.) was applied, under polythene occlusion, to the tumour. Four hours later, excess 5-ALA was wiped off and fluorescence was graded as satisfactory using Woods light. He received 20 J cm at 20 mW cm using a Waldmann PDT lamp (MSR 1200; 580±740 nm). Irradiance was measured using a calibrated handheld meter (International Light 1400A and Selo33/F/W/QND52 detector with spectral shaping and neutral density filters calibrated by D.Taylor, Gloucester, U.K.). He graded pain during treatment as 8/10 but experienced no other adverse effects. The lesion cleared after five consecutive treatments over 12 weeks. Repeat biopsy showed a lymphohistocytic infiltrate but complete clearing of the original infiltrate of malignant T cells (Fig. 1b). During his course of PDT his generalized patch and plaque MF relapsed. The tumour site has remained clear on clinical examination 1 years later. He has required two courses of PUVA therapy for generalized patch and plaque MF during this period. Topical 5-ALA PDT has been proven to be effective for superficial cutaneous cancers, but there are few reports of its use in treating MF. Shanler et al. treated patch/ plaque-stage cutaneous T-cell lymphoma and showed that protoporphyrin IX accumulated within lymphocytic infiltrates; early therapeutic results were promising. Boehncke et al. using an argon laser at 630 nm showed inhibition of proliferation of malignant transformed T cells in vitro and in vivo. Wolf et al. have demonstrated the efficacy of PDT (20% 5-ALA) using a broad-spectrum source (40 J cm at 44 mW cm) in two patients with MF who cleared after four and five PDT treatments, respectively. However, Amman and Hunziker reported a poor response for an infiltrated plaque of MF to just one PDT treatment using an identical regimen. This suggests that multiple treatments are required to obtain a complete histological response. We have demonstrated the benefits of low light dose, low dose rate topical 5-ALA PDT for nodular MF, but a formal study is needed to confirm our findings.

A Case of Polypoid Dermatofibroma

Dermatofibroma is a common benign cutaneous tumor that usually appears as a slowly growing firm nodule. Polypoid nodular dermatofibroma is a variant type that is rarely encountered. We reported a case of polypoid dermatofibroma with a review of the previously reported cases. Polypoid dermatofibroma tends to arise on the leg, especially below the knee. Its size is often larger than that of common dermatofibroma. It is speculated that both the underlying firm tissue and long‐term development may lead the tumor to form a polypoid appearance.

Langerhans cell histiocytosis masquerading as hidradenitis suppurativa

Dear Editor, A 42-year-old Japanese man presented with a 22-year history of painful nodules and ulcers with recurrent draining sinuses on the axillary and perianal regions. He developed a pneumothorax at the age of 25 years, which was diagnosed as eosinophilic granuloma. His axillary and perianal lesions had been treated with surgical drainage of the abscesses in other hospitals. Because the lesions had enlarged, he visited our hospital. A physical examination revealed brownish indurated plaques with reddish-purplish papules, ulcers and fistulas, and active purulent drainage on the bilateral axillaries (Fig. 1a). The perianal region showed multiple ulcers and fistulas with scars due to repeated surgical drainage over the past 20 years (Fig. 1b). At that time, we clinically diagnosed these ulcers and fistulas as hidradenitis suppurativa. Bacterial culture revealed a small number of Staphylococcus aureus and Prevotella disiens. We performed debridement and split-thickness skin grafting on the axillary lesions. A pneumothorax developed on postoperative day 2, which was treated with a partial pneumonectomy. Histological examination revealed sinuses lined with epidermis in the dermis (Fig. 1d) and a dense infiltration of histiocytic mononuclear cells and multinucleated giant cells from the dermis to subcutaneous tissue (Fig. 1e). The histiocytic cell populations expressed CD1a (Fig. 1f) and S-100 proteins (Fig. 1g). The hypermetabolic lesions of F-fluorodeoxyglucose positron emission tomography/computed tomography were confirmed in the soft tissue from the right neck to the back, thyroid (Fig. 1h), perianal region and adductor magnus muscle. The histological findings of the resected lung tissue and needle biopsies from the thyroid and soft tissue of the neck were compatible with those of Langerhans cell histiocytosis (LCH). The results of a plain-film bone survey and bone marrow puncture were normal. Finally, we established the diagnosis of adult-onset LCH with multisystem involvement. He was treated

Cutaneous squamous cell carcinoma, thyroid cancer and Langerhans cell histiocytosis in a patient with X-linked recessive Mendelian susceptibility to mycobacterial diseases with a nuclear factor-κB essential modifier mutation

Nuclear factor (NF)‐κB essential modifier (NEMO), also known as IκB kinase subunit‐γ (IKKγ), is a pivotal molecule in the NF‐κB signaling pathway. Mutations of NEMO cause incontinentia pigmenti and X‐linked ectodermal dysplasia with immunodeficiency. Mendelian susceptibility to mycobacterial diseases (MSMD), which confers an almost selective predisposition to mycobacterial infection, is also caused by NEMO mutations. We herein report the first case of a patient with X‐linked recessive (XR) MSMD who developed cutaneous squamous cell carcinoma, thyroid cancer and Langerhans cell histiocytosis. The relationship between NEMO mutation and oncogenesis is discussed.

Detection of Human Papillomavirus Type 67 in Subungual Bowen's Disease Presenting as Longitudinal Melanonychia.

© 2015 The Authors. doi: 10.2340/00015555-2054 Journal Compilation