Eosinophilic Fasciitis - Report of Three Cases and Review of the Literature.

BACKGROUND: Eosinophilic fasciitis is a rare fibrosing disorder of muscle fascia with rapid onset of erythema, induration, oedema and tenderness affecting extremities bilaterally. CASE REPORT: We report three cases of eosinophilic fasciitis in 3 females aged 64, 65 and 73 years, in two of them in association with morphea. They fulfilled the proposed diagnostic criteria. Associated malignancies could be excluded in all of them. They were treated by systemic corticosteroids. In the two females with associated morphea higher prednisolone dosages and a combination with methotrexate was necessary. CONCLUSIONS: Eosinophilic fasciitis is a differential diagnosis of systemic scleroderma. Response to treatment is often delayed. Systemic corticosteroids are the first line therapy. Patients with associated morphea need combined drug therapy, in our patients with methotrexate. There is no close correlation between laboratory signs of inflammation and clinical response to treatment.

Wells Syndrome - An Odyssey.

BACKGROUND: Wells syndrome is a rare idiopathic dermatosis of the eosinophilic spectrum. Diagnostic criteria include cutaneous eruptions of variable morphology with eosinophilic infiltrates, peripheral blood eosinophilia, a relapsing, remitting course, and exclusion of systemic disease. Diagnosis is often delayed. CASE PRESENTATION: We present a 28-year older man with recently developed pruritic and sometimes painful erythema. His medical history was positive for coughing in the evening that started in November 2012. Later, a pansinusitis developed. Early diagnosis improves the outcome. CONCLUSION: Standardized treatment has yet to be developed. In our case, systemic corticosteroids were of limited value only.

Advanced Ulcerated Squamous Cell Carcinoma of the Hand with Locoregional Axillary Lymph Node Metastasis - Case Report and Literature Review.

BACKGROUND: Cutaneous squamous cell carcinoma (SCC) of the hand is the most common soft-tissue malignancy in this particular region. A literature survey suggested a higher rate of metastases in advanced SCC of the hand compared to head-and-neck cutaneous SCC. CASE REPORT: An 84-year-old man presented with an ulcerated firm tumour on the dorsum of his right hand. A diagnostic biopsy confirmed the diagnosis SCC. Imaging suggested an involvement of the tendons of digits 3 and 4. A diagnostic ultrasound suggested a loco-regional axillary lymph node metastasis. After discussion in the interdisciplinary tumour board, amputation of the affected digits followed by lymph node excision was recommended. CONCLUSIONS: Advanced SCC of the hand requires interdisciplinary management. Amputation is part of the surgical spectrum in advanced cases.

Treatment of lipedema by low-volume micro-cannular liposuction in tumescent anesthesia: Results in 111 patients.

Lipedema is a painful disease of subcutaneous adipose tissue leading to bilateral increase of leg and/or arm volume, but sparing hands and feet. Although conservative treatment with complex decongestive therapy has been considered as the fundamental treatment, micro-cannular liposuction in tumescent anesthesia has become a surgical option. We report on 111 patients mostly with advanced lipedema treated by this technique in our center between 2007 and 2018. The median age of the patients was 44 years. Eighty percent of patients had at least one comorbidity. There was an association of longstanding and advanced disease to obesity and diseases of the metabolic syndrome-spectrum. The median total amount of lipoaspirate was 4,700 ml, with a range of 950-14,250 ml. The median reduction of limb circumference was 6 cm. The median pain level before treatment was 7.8 and 2.2 at the end of the treatment. An improvement of mobility could be achieved in all patients. Bruising was also reduced. Serious adverse events were observed in 1.2% of procedures, the infection rate was 0% and the bleeding rate was 0.3%. Liposuction is an effective treatment for painful lipedema. The procedure should be performed in specialized centers.

Acute edema induced by toluidine blue extravasation-first report.

Methemoglobinemia is characterized by an increased level of methemoglobin (MetHb) in the peripheral blood. MetHb levels increase after tumescent anesthesia and need to be monitored. If a patient becomes symptomatic and/or the MetHb levels increase >10%, intravenous injection of an antidote is recommended. Toluidine blue is twice as effective as methylene blue in this respect.A 27-year-old woman with advanced lipedema underwent her third liposuction under tumescent anesthesia. After surgery, her MetHb levels increased and needed injection of toluidine blue. She developed an acute and painful edema after extravasation of some toluidine blue due to a bursting vein. This is the first report in the recent medical literature. Clinical presentation, course, and treatment are described.

The Role of Complex Treatment in Mixed Leg Ulcers - A Case Report of Vascular, Surgical and Physical Therapy.

BACKGROUND: Leg ulcers are a burden to patients, their families and society. The second most common cause of chronic leg ulcers is the mixed arterio-venous type. An 80-year-old female patient presented to our department due to painful enlarging chronic leg ulcer of mixed arteriovenous origin on her left lower leg. She suffered from peripheral arterial occlusive disease stage I and chronic venous insufficiency Widmer grade IIIa, and a number of comorbidities. AIM: The aim of our ulcer treatment was a complete and stable wound closure that was hampered by arterial occlusion, exposed tendon, and renal insiffuciency. CASE REPORT: To improve the prognosis for ulcer surgery, we performed percutaneous transluminal angioplasty, transcutaneous CO2 and deep ulcer shaving. The wound was closed by sandwich transplantation using elastin-collagen dermal template and meshed split skin graft. She had a 100% graft take with rapid reduction of severe wound pain. CONCLUSION: Complex approaches are necessary, to gain optimum results in leg ulcer therapy in mixed leg ulcers. Therapeutic nihilism should be abandonend.

Segmental Erythema Multiforme-Like Drug Eruption by Aromatase Inhibitor Anastrozole - First Case Report and another Example of an Immunocompromised District.

Anastrozole is a non-selective aromatase inhibitor for adjuvant breast cancer therapy in postmenopausal women. Cutaneous adverse events have been reported. We observed a 64-year-old female patient with a medical history of locally advanced breast cancer of her right breast that was treated with radiotherapy and adjuvant drug therapy with anastrozole. She developed a segmental bullous eruption limited to the cancer-affected breast. Cessation of the aromatase inhibitor and systemic therapy with prednisolone cleared the lesions completely. This is the first report of a segmental erythema multiforme like drug eruption by anastrozole and another example of the concept of the immunocompromised district of skin.

Ulcerating Lichen Planopilaris - Successful Treatment by Surgery.

Lichen planus is a T-cell mediated autoimmune disorder affecting the skin and mucous membranes. Ulcerating lichen planus is uncommon mostly on oral and genital mucosa but not skin. Lichen planopilaris, however, is a subtype of lichen planus affection hair follicles and leading to permanent scarring alopecia. We report a case of lichen planopilaris of the scalp with multiple alopecic patches ulceration - a hitherto unreported clinical feature. The patient was treated surgically, and the defect could be closed by combined tissue advancement and extension.

Unilateral Palmar Callus and Irritant Hand Eczema - Underreported Signs of Dependency on Crutches.

Leg amputees who can't use prostheses and patients with arthritis are often dependent on crutches. Their chronic use can exert significant friction forces. The palmar skin will respond by forming a hyperkeratotic callus. We report for the first time unilateral palmar callus formation caused by friction from using crutches. Another possible adverse effect is the triggering of irritant contact dermatitis by the handholes of crutches. We report two cases with hand dermatitis due to the chronic dependence on crutches and discuss treatment options.

Cutaneous Microembolism of Fingers and Toes.

A macro vascular embolism is a well-known emergency. In contrast, cutaneous microembolism is a lesser known symptom. However, cutaneous microembolism of fingers and toes is a red flag symptom for vascular emergencies. The underlying cause may involve infectious, immunological, metabolic and physical disorders, coagulation disorders and malignancies. Early recognition can help to live safe.

Acrocyanosis - A Symptom with Many Facettes.

Acrocyanosis is an uncommon complaint belonging to the acro-syndromes. It typically presents with coolness and bluish discolourations of hands, feet, ears, nose, lips and nipple. The most frequently affected parts of the body are the hands. This review discusses physical factors, vascular disorders, infectious diseases, haematological disorders, solid tumours genetic disorders, drugs, eating disorders, and spinal disease presenting as or leading to acrocyanosis.

Palmar hyperhidrosis treated by noninvasive ultrasound stellate ganglion block.

Focal palmar hyperhidrosis is a common and often debilitating eccrine sweat gland disorder with negative impact on quality of life and self-esteem. For treatment of recalcitrant cases, a stellate ganglion block is a nonsurgical alternative. Although this method has only a temporary effect, surgical risks can be avoided. The usual way to perform the block is by ultrasound-guided injection of local anesthetics. Here we describe the use of therapeutic ultrasound at 0.8 MHz for stellate ganglion block. Ultrasound was applied for 1 min unilaterally every other day for 6 days. The efficacy was monitored by video capillaroscopy and Minor's iodine starch test. Treatment was well tolerated and no adverse effects were noted. Sweating was stopped and capillary blood flow increased. The effect lasted for several weeks.

Madelung's Disease - Case Series and Treatment by Tumescent Liposuction or Lipectomy.

Madelung disease is a disfiguring disorder belonging to the heterogeneous group of lipomatosis. The aetiology is not well understood, but alcohol consumption has been regarded as of importance. The reported incidence is about 1 in 25,000 inhabitants. We reviewed our files of the last ten years and identified eight adult patients with an equal gender distribution. Their age was between 60 and 85 years of life. Comorbidities are frequent. Clinical presentation may vary. Surgical treatment is reported and discussed. Both cold steel surgery and tumescent liposuction have their place in treatment.

Hereditary Lymphedema of the Leg - A Case Report.

Primary of hereditary lymphedema is a rare but progressive disease. It is yet not curable. We present a 48-year-old male patient with hereditary lymphedema of his left leg, that was realised by minor trauma (able twist) when he was seven years old. He had never been treated for lymphedema but experienced multiple erysipelas during his life. After diagnostic procedures to exclude other causes of leg swelling, the diagnosis of hereditary lymphedema of the leg, stage III was confirmed. We initialized complex decongestive therapy. During two weeks of intensive treatment, the circumference of the left leg could be reduced by 10 cm. This case illustrates the "natural course" hereditary lymphedema. But it raises the hope that even after decades of ignorance, the patients benefits from complex decongestive treatment. Therapeutic nihilism is unnecessary and poses lymphedema patients to risks of infection and secondary malignancies like Stewart-Trewes syndrome.

Anogenital giant seborrheic keratosis.

Seborrheic keratosis (SK) are very common benign epidermal tumors. Giant seborrheic keratosis (GSK) is a rare variant with clinical characteristics, which leads very often to misdiagnosis. A genital site of SK is very unusual clinical manifestation and although the cause is still unknown, current literature data point to a possible pathogenetic role of chronic friction and HPV infection. The rare genital localization makes Buschke-Löwenstein tumor and verrucous carcinoma important differential diagnoses. GSK may also show some clinical features of a melanoacanthoma, which makes cutaneous melanoma as another possible differential diagnosis. The clinical diagnosis of genital GSK is often a very difficult one, because the typical clinical features of GSK disappear and the most common dermoscopic features of GSK are usually not seen in the genital region lesions. The diagnosis of GSK of the anogenital area should be made only and always after the exact histological verification and variety of differential diagnosis should be carefully considered. The treatment of GSK is primary surgically. We present a rare case of GSK with concomitant HPV infection in the anogenital region of 72-year-old patient. Surgical approach was performed with excellent outcome.

Comorbidities, treatment, and outcome in severe anogenital inverse acne (hidradenitis suppurativa): a 15-year single center report.

BACKGROUND: Acne inversa (hidradentitis suppurativa; AI) is a severe chronic relapsing inflammatory skin disease of unknown cause. Anogenital AI has the strongest negative impact on quality of life and causes great disability. OBJECTIVES: We analyzed patients, comorbities, outcome of extensive surgery, and possible adverse effects. PATIENTS AND METHODS: We included patients from January 2000 to March 2015 with anogenital AI Hurley grade III. They were further characterized by modified Sartorius scale, gender and age, comorbidities, pretreatments, and type of surgery (open or closed), and complications. Pain was measured by visual analogue scale (VAS). Treatment was performed by wide excision surgery. RESULTS: A total of 117 patients were identified; mean age was 40.6 ± 12.6 years. The mean modified Sartorius score was 30.4 ± 8.4. Risk factors of obesity and active smoking were seen in 31.6% and 14.5%, respectively. Colostomy was needed in a single patient only. Pain was reduced significantly. Intra- and postoperative complications were seen in 20.5% (24/117). CONCLUSIONS: Anogenital AI is a severe and potentially life threatening disease. Severe anogenital AI can be effectively treated by aggressive surgery. Outcome depends also on comorbidities.

Rare association of cystic squamous cell carcinoma and small lymphocytic B cell lymphoma: successful surgical approach.

Non-melanoma skin cancer is the most common type of cutaneous neoplasm worldwide. While basal cell carcinoma is the most common tumor, squamous cell carcinoma (SCC) causes higher morbidity and has a risk of metastatic spread, depending on immune status, tumor size, and desmoplastic growth. We reported the case of a 77 year old male patient with retroauricular tumor, which started growing 3 years ago and was excised, buth relapsed three times. The initial diagnosis was infundibular cyst. Delayed Mohs surgery was performed, as was an additional open lymph node biopsy of the patient's right groin, on the occasion of an indolent swelling of the same which developed within 3 months. The first histopathological report confirmed the diagnosis of a cystic squamous cell carcinoma. The histopathologic evaluation of the groin tumor revealed a small lymphocytic B cell lymphoma (BCL). The patient fulfilled the following criteria for high-risk SCC: tumor size ≥2 cm (or 1 cm on the head and 6 mm on the genitals, hands, and feet), tumor thickness ≥4 mm, recurrent tumor, rapid growth. Therefore, lymph node metastasis had to be considered. High-risk SCC has a propensity to metastasize. In cases of primary tumor, Mohs surgery is the most effective treatment, particularly in relapsing tumors. The combination of cystic SCC with a small-sized BCL is very rare. The differential diagnosis and treatment may be challenging. In high-risk SCC, lymph node enlargement warrants histologic evaluation. However, not all suspicious lymph node lesions corroborate as metastatic.

Complicated Skin and Skin Structure Infection After Erysipelas: Urgent Need for Antibiosis and Surgery.

Erysipelas are common soft tissue infections responding to first-line antibiosis. Because of factors of related to responsible bacteria and host, complications can occur that need extensive surgery in addition to intensified drug therapy. We report on a 65-year-old woman with leg ulcer who developed an absceding and necrotizing panniculitis of the affected leg complicating erysipelas. Escherichia coli and Pseudomonas aeruginosa were identified. Debridement and surgical removal of inflamed subcutaneous adipose tissue was decisive to interrupt the process. Wound bed preparation was realized by vacuum-assisted closure. Final wound closure was done by split-skin mesh graft. Complicated skin and skin structure infections need a combined approach of intensified antibiosis and surgery to save life.

Juxta-articular adiposis dolorosa (Dercum's disease type IV): report of four cases and treatment by dermolipectomy.

Juxta-articular adiposis dolorosa is a rare subtype of Dercum's disease. It manifests mainly on the medial parts of the knees. Pain and impaired mobility are common symptoms. We report on four females (aged between 52 and 83 years) who suffered from juxta-articular adiposis dolorosa for more than 10 years. These patients were successfully treated by dermolipectomy resulting in dramatically improved pain and mobility. Adverse effects and complications were minor with a lymph fistula in a single patient which was treated by surgery.