Therapy-resistant skin ulcers on hypoplastic leg associated with neurofibromatosis type 1

We describe a case of therapy-resistant skin ulcers in sporadic neurofibromatosis type 1. A 40-year-old woman had suffered from neurofibromata on her trunk and extremities since 30 years prior. She suffered from hypoplasia of her right leg from a young age and had a 1-year history of therapy-resistant skin ulcers on the leg and inguinal region. Magnetic resonance angiography disclosed a narrowed femoral artery at the level of the upper thigh with poor root in the inner side corresponding to the ulcerated lesions. The vascular changes were thought to be the cause of the skin ulcers.     

Muir-Torre syndrome: a case of this uncommon entity

A 69-year-old Hispanic woman presented for the evaluation of nodules on the head and back. In the past, she had been treated for basal cell carcinoma (BCC) of the face; the referring physician was concerned that the new lesions might also be BCC. The patient had an extensive past medical history. In addition to BCC, she had been treated for breast cancer, colon cancer, and cervical cancer prior to emigrating to the USA. Her colonic malignancy had been localized proximal to the splenic flexure. She also had a history of colonic polyps and distal colonic villous adenoma. She denied ever being treated with radiation. Further details of her medical history and cancer staging were not available. Her family history was significant for a sister with colon cancer and transitional cell carcinoma of the urinary bladder. In addition, she had a great aunt with oral cancer and a great uncle with lung cancer. Neither the patient or her relatives had any history of tobacco use. On physical examination, in addition to scars from a radical mastectomy and midline abdominal laparotomy, four skin lesions were noted: two on the scalp, one on the tragus, and one on the mid-back. The first lesion on the vertex of the scalp was a yellow-brown waxy papule measuring 0.6 x 0.5 cm. This lesion was similar to that on the mid-back, except in size. The lesion on the back measured 1.2 x 1.0 cm. The second lesion on the frontal scalp measured 0.8 x 0.6 cm and was red-brown with a pearly appearance and some central hyperkeratosis. The tragus lesion was similar in appearance to that on the frontal scalp. Shave biopsies of all lesions were obtained. The lesions on the scalp and mid-back revealed lobules of sebaceous cells in the dermis with a minority of surrounding basaloid cells, consistent with a diagnosis of sebaceous adenoma (Fig. 1). Although the lesion on the frontal scalp also showed sebaceous differentiation, there were a greater number of basaloid cells, some with hyperchromatic nuclei and mitotic figures; this was consistent with a diagnosis of sebaceous epithelioma (Fig. 2). The final lesion (tragus) was histologically consistent with a keratotic BCC. No further treatment was required for these benign sebaceous tumors, but their presence defined our patient's condition as Muir-Torre syndrome. Mohs' micrographic surgery was performed on the tragus BCC and the margins were tumor free in one stage. The patient returned 1 year later with a lesion anterior to the left axilla which was biopsied to rule out BCC (Fig. 3). Histologically, this lesion was also consistent with sebaceous epithelioma.     

Basal Cell Carcinoma Following Chronic Otitis Media

Abstract: A 36-year-old white woman presented with a gradually enlarging lesion in the right preauricular area of 15 years duration. The patient had a 25-year history of chronic otitis media of the right ear; in spite of multiple courses of systemic antibiotics, she frequently noted a slight discharge from the affected ear, draining across the right preauricular area, causing a mild pruritic eruption. She recalled having had approximately 30 roentgenographs of mastoids, but denied any history of fluoroscopic examination. The patient denied a history of excessive sun exposure or exposure to arsenic or other carcinogenic factors. There was no family history of skin cancer.     

A Case of Cutaneous Malignant Fibrous Histiocytoma

A 54-year-old Japanese man with cutaneous malignant fibrous histiocytoma on the back is reported. He not only had a past history of thyroid cancer 1 year prior to the onset of the skin tumor, but also had simultaneous bladder cancer. Despite the early, wide resection, the prognosis was rapid and progressive. Histologically, the primary lesion of the skin tumor was difficult to differentiate from dermatofibrosarcoma protuberans; however, the recurrent and the metastatic lesions changed in appearance.     

Alopecia areata associated with regression of cutaneous melanoma

A 41-year-old white female with a past medical history of hypothyroidism and alopecia universalis presented on January 24, 2002 with a recently changing mole. She indicated changes in size and color of the superior aspect of a mole that had been present for more than 8 years. She had approximately 20 lifetime peeling sunburns due to being a lifeguard. No family or previous personal history of skin cancers, including melanoma or atypical nevi, was reported. Her history of alopecia universalis began 12 years previously and has partially resolved with remaining patchy alopecia of the scalp and eyebrows. On diagnosis of alopecia universalis, she was initially treated with oral prednisone for 1 year and topical minoxidil for 3 months. Currently, she is not being treated for this condition. She denied other previous skin conditions. She had a surgical history of tonsillectomy at the age of 7 years. Her current medication includes levothyroxine (0.015 microg) for hypothyroidism diagnosed 12 years previously. She reported no known drug allergies. During the initial physical examination, she presented with phototype II skin with two adjacent pigmented lesions on her left foot within a 1.3 cm square. The first lesion on the left posterior distal heel was an irregular, brown-black, 0.5 x 0.6 cm macule. The second lesion, on the left posterior proximal heel, was an irregular, brown, speckled, 0.3 x 0.4 cm macule (Fig. 1). The patient had ophiasis of the scalp and total alopecia of the bilateral eyebrows. In keeping with the patient's wishes, alopecia lesions were not biopsied and clinical photographs of the alopecia are not included in this article. Two 3 mm punch biopsies were performed within each lesion. The left posterior proximal lesion showed malignant melanoma, with a Breslow depth of 0.4 mm, anatomic level II, marked lymphocytic response and partial regression (Fig. 2). The left posterior distal lesion showed malignant melanoma in situ, arising in a lentiginous compound nevus, with architectural disorder and cytological atypia. These two lesions were concluded to be one lesion with clinical regression. She underwent local excision with 1-cm margins and sentinel lymph node biopsy owing to the presence of regression, which showed no evidence of metastatic melanoma. Lactate dehydrogenase and chest X-ray were within normal limits. The alopecia areas were not biopsied previously or at that time.     

皮肌炎/多发性肌炎患者伴发恶性肿瘤危险因素的研究与Logistic回归分析

目的探讨皮肌炎/多发性肌炎(DM/PM)患者伴发肿瘤的危险因素及两者间的关系。方法对DM/PM伴发恶性肿瘤者的临床表现、实验室辅助检查指标和治疗情况进行单因素和Logistic回归分析。结果恶性肿瘤年龄偏大者多见,好发于DM后1~2年,PM后1~5年。DM伴发恶性肿瘤的危险因素有中度日光性皮炎和/或皮肤瘙痒、中度咽喉部肌群受累(如吞咽困难、声嘶等)、重度颈部肌群受累(如抬头困难)、消瘦等;而PM则为轻度呼吸肌受累(如呼吸困难)。结论对出现中度日光性皮炎和/或皮肤瘙痒、消瘦、中度咽喉部肌群、重度颈部肌群和轻度呼吸肌受累的中老年DM/PM者尤其在发病2~5年内应警惕伴发的恶性肿瘤。     

Successful treatment with bosentan for refractory digital ulcers in a patient with systemic lupus erythematosus

We report a 32-year-old woman with a 12-year history of systemic lupus erythematosus. Physical examination revealed indurated plaques with small ulcers on her extremities and trunk, which were histologically diagnosed as lupus erythematosus profundus. On her arms and knees, multiple small calcified nodules were noted in the dermis and subcutis. An elevated level of anti-phosphatidylserine/prothrombin antibodies was noted. She had been suffering from digital ulcers on the left fourth finger. Despite conservative therapies, new ulcers appeared on other fingers. After the administration of bosentan, no new lesion has developed. Bosentan, recognized as a choice of treatments for digital ulcers in systemic sclerosis, is worth trying in systemic lupus erythematosus patients with refractory digital ulcers.     

Skin infection caused by Mycobacterium avium

A patient with skin infection due to Mycobacterium avium is reported. A 9–year–old female had 10 subcutaneous nodules and two ulcers on the abdomen and legs. She had no medical history of systemic disease, skin disease or immunosuppressive therapy. Cultures of a biopsy specimen and of aspirated seropurulent fluid in nodules showed acid–fast bacteria, identified as M. avium by the DNA–DNA hybridization method. We treated her with a combination of surgery and the antibiotics, cycloserine, isoniazid and clarithromycin.     

Glabellar pomade crust mimicking dyskeratosis follicularis

A 79-year-old female patient presented with a slowly developing crusting, itching verrucous lesion of the forehead of 3 months' duration. She had no personal or family history of skin disease. On examination she presented with a hyperpigmented plaque of the glabellar region that resembled dyskeratosis follicularis Darier. A detailed medical history was taken and a skin biopsy was performed. Epidermal acanthosis and enlargement of follicle ostia with foreign material were found. The patient reported use of an ointment twice daily. She had a single cosmetic treatment where powder of unspecified composition had been used. The diagnosis of pomade crust was confirmed. Follicular material was enucleated mechanically and the area was treated with a metronidazole cream. The lesions completely disappeared.     

Malignant histiocytosis with panniculitis--a case report

We report a case of malignant histiocytosis which began with the skin lesions of panniculitis. A 32-year-old woman presented with recurrent erythematous plaques, subcutaneous nodules, and ulcers on the trunk and the extremities and intermittent fever for 7 months. The cutaneous lesions consisted of erythematous and brownish irregular-shaped patches and tender cutaneous nodules 0.5-1.0 cm in diameter. Central necrosis and shallow ulcers were seen in the lesions. The patient also suffered from general fatigue, arthralgia, and weight loss. She was anemic and thrombocytopenic and had progressive impairment of liver function with coagulation defect. Histopathological study of skin lesions showed lobular panniculitis without vasculitis in the subcutaneous fat tissue. In the panniculitis lesion, moderate mixed cell infiltration consisting of lymphocytes and histiocytes was observed. Bone marrow aspiration revealed an increase in the number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having malignant histiocytosis and treated with cyclophosphamide, vincristine, and prednisolone which she responded well; her fever subsided and the lesions healed with hyperpigmentation. In this patient, benign histiocytes with hemophagocytosis without immature forms were found in the skin lesions. According to our knowledge, this is the first Thai report of malignant histiocytosis with clinical features of panniculitis.     

Reactions to Penicillamine: A Case of Cutis Laxa,Elastosis Perforans Serpiginosa and “Pseudo” Pseudoxanthoma

This patient was a 61‐year‐old white female who received several years of penicillamine therapy for the treatment of cystinuria. She subsequently developed penicillamine induced cutis laxa, elastosis perforans serpiginosa, and pseudoxanthoma elasticum like skin lesions. In addition, she suffered from numerous chronic bilateral lower extremity skin ulcerations. Her past medical history was also significant for end stage renal disease requiring hemodialysis and pulmonary fibrosis. She presented to the University of Miami Wound Care Center in 1/04 for treatment of her chronic ulcerations. On physical examination, the patient had multiple large hyperpigmented plaques with central ulcerations on her lower extremities. Some of the ulcers had overlying crust and others were covered with yellow fibrinous tissue. She also had generalized thickened, lax skin with multiple folds. On her neck, thighs, back and arms were violaceous, atrophic, serpiginous plaques with peripheral crusted erosions. A biopsy taken from the patients left thigh revealed dermal elastosis and the features of pseudo‐pseudoxanthoma. Two additional biopsies taken from the left thigh demonstrated elastosis perforans serpiginosa. This case highlights multiple skin manifestations of penicillamine therapy.     

Ulcerations caused by livedoid vasculopathy associated with a prothrombotic state: Response to warfarin

A 50-year-old woman had a lifelong history of painful ulcerations as a result of livedoid vasculopathy. She was a heterozygous carrier of factor V Leiden and prothrombin gene mutations and was receiving hormone replacement therapy. The ulcers healed after warfarin therapy, which has been reported to be effective in only one previous patient with this condition.     

Mycetoma of the foot

A forty-year-old woman suffering from maduromycosis of the right sole is described with a disease history of 17 years. After a contusion and surgery for fibroma she developed an edema on the skin of the right sole, then ulcers with purulent discharge. She was repeatedly treated on an outpatient basis and in hospital by surgeons, phthisiologist, and dermatologist for osteomyelitis, tuberculosis of the bones, and Kaposi's sarcoma without effect. The diagnosis of maduromycosis of the sole was confirmed by laboratory examinations and histologically by detecting Actinomyces elements. Since conservative therapy has proved ineffective, surgical treatment was recommended.     

Eccrine porocarcinoma arising in a seborrheic keratosis evaluated with dermoscopy and treated with Mohs' technique

A 78-year-old white woman returned for a routine 6-month skin cancer examination. She had a history of actinic keratosis and multiple basal cell carcinomas. She had no personal or family history of dysplastic nevi or melanoma. The patient was asymptomatic and unaware of any new or changing skin lesions. The patient had multiple lentigines, hemangiomas, and actinic and seborrheic keratoses on all sun-exposed areas. There were no less than 10 seborrheic keratoses on the right mid-back, and one was found to have a 1-cm, reddish nodule asymmetrically located within it (Figs 1 and 2). A clear papule on the left preauricular area was found on biopsy to be a basal cell carcinoma. The nodule on the back was still present 1 month later and it was felt that further evaluation was indicated. As melanoma has been reported to develop in seborrheic keratoses, we decided to examine the lesion using digital dermoscopy. With digital dermoscopy, a well-demarcated reddish nodule was asymmetrically located within a brown lesion. It blanched significantly with pressure. Within the nodule, there were dotted and irregular linear vessels (atypical vascular pattern; also known as polymorphous vascular pattern) and regular-appearing brown dots. Surrounding the reddish nodule, there were pale and pigmented, comedo-like openings, fissures, and ridges (brain-like appearance). Some of the follicular openings appeared to be within the wall of the nodule (Figs 3 and 4). Comedo-like openings, fissures, and ridges are primary dermoscopic criteria for the diagnosis of a seborrheic keratosis; however, the vascular pattern seen has not been reported in seborrheic keratosis. Due to the patient's age and the rarity of significant pathology arising in a seborrheic keratosis, a shave biopsy was performed. To our surprise, the specimen was interpreted by an experienced dermatopathologist as a well-differentiated eccrine porocarcinoma. Due to the high local recurrence rate and metastatic potential of this carcinoma, the patient was referred for Mohs' surgery. Both the basal cell carcinoma and the eccrine porocarcinoma were excised in one stage. A metastatic work-up was negative and the patient appears to be doing well.     

变应性肉芽肿病一例

报告一例变应性肉芽肿病。患者,女,33岁,过敏性哮喘6年,伴外周血嗜酸性粒细胞增高。2月来出现双下肢丘疹、结节、溃疡伴瘢痕形成,腓肠肌疼痛2周,皮损病理示血管炎和嗜中性粒细胞和嗜酸性粒细胞浸润,确诊为变应性肉芽肿病。     

乳腺癌皮肤转移1例

患者女,46岁。胸部皮肤破溃伴瘙痒和疼痛2个月,右大腿疼痛1月余。4年前行左乳腺癌根治术,术后定期化疗。皮肤科情况:前胸部可见一3.0cm×7.0cm大小暗红色不规则溃疡,隆起,质硬,无触痛。上覆黑痂、出血和少许脓性分泌物。CEA140.10ng/mL,CA-1251029.00U/mL,CA-153163.10U/mL。右股骨正侧位和胸片示骨、肺转移。诊断:乳腺癌皮肤转移合并骨、肺转移。拒绝治疗,出院。     

A Case of Pyoderma Gangrenosum with Ulcerative Colitis Treated with Mesalazine

Pyoderma gangrenosum (PG) manifests as recurrent deep ulceration of the skin and PG is associated with a variety of disorders. Approximately 30% of the cases of PG develop in patients with inflammatory bowel disease. A 34-year-old woman presented with a one-week history of recurrent ulcers on the right cheek and back. She was diagnosed with ulcerative colitis (UC) 4 years previously and with PG 1 year previously. The clinical course of the skin lesions followed the status of her UC. The patient''s skin lesions and bowel symptoms were not improved with prednisolone. After she was started on mesalazine, we observed rapid resolution of skin lesions and bowel symptoms. Herein, we report a case of recurrent PG with UC, and we discuss the possible association between these two conditions, and the efficacy of mesalazine therapy for the treatment of PG combined with UC.     

Cutaneous chancroid in a visitor from Vanuatu

A 23-year-old woman from Vanuatu presented to an Australian hospital with a 3-week history of a non-healing ulcer on the lower leg. A swab was submitted for a multiplex polymerase chain reaction designed to investigate genital ulcerative conditions. Haemophilus ducreyi was detected and the gene product was subsequently sequenced, confirming the diagnosis of cutaneous chancroid. The lesion responded to intramuscular benzathine penicillin. This report adds further evidence that cutaneous chancroid should be considered in the evaluation of skin ulcers in the south Pacific.     

Squamous cell carcinoma in a patient with Netherton's syndrome

A 29-year-old white woman with a history of Netherton's syndrome presented with two squamous cell carcinomas on the right dorsal hand and the left upper arm. She reported a 2-year history of these lesions, which were originally treated as warts. She denied excessive sun exposure, immunosuppressive therapy, or a previous history of skin cancer. Her past medical history included acute renal failure, multiple urinary tract infections, meningitis, and recurrent otitis media as a child. In addition, she had an ovarian abscess at 4 years of age with resulting salpingo-oophorectomy. She also reported a history of severe myopia, glaucoma, and multiple ocular infections with a resulting corneal scar. In addition to atopic dermatitis, she had a 10-year history of psoriasis. Her medications included topical steroids and emollients for atopic dermatitis and psoriasis, in addition to Timolol ophthalmic drops for glaucoma. Her family history was significant for a 22-year-old sister with Netherton's syndrome (Fig. 1). She denied any history of skin cancer in her sister or other members of her family. On physical examination, she had an exfoliative erythroderma, madarosis, and diffuse patchy alopecia. In the bilateral axilla, she had well-defined pink scaly plaques which were confirmed as psoriasis by biopsy. On the right dorsal hand, she had a 1.5 x 1.0 cm pink verrucous plaque (Fig. 2). On the left upper arm, she had a 1.5 x 0.8 cm pink scaly plaque. Biopsies of both sites confirmed squamous cell carcinomas. Both lesions were completely excised with 4 mm margins.