Atypical fibroxanthoma: a series of 56 tumors and an unexplained uneven distribution of cases in southeast Germany.

BACKGROUND: Atypical fibroxanthoma is a rare mesenchymal tumor of the head and neck region. METHODS: We analyzed the files of 3 large dermatology hospitals from the years 2001 to 2013 in southeast Germany. RESULTS: We identified 53 patients (56 tumors) with a male predominance. The mean age was 78.0 years ± 8.3 years. Mohs surgery was performed in all cases resulting in complete remission in 45 patients. Five patients had a relapse within 2 years, and 4 developed metastases. None of the tumors with a safety margin of ≥2 cm relapsed. The majority of cases were found in the Dresden region. CONCLUSION: Atypical fibroxanthoma demonstrates an uneven geographic distribution in southeast Germany that demands further investigations. Mohs surgery with ≥2 cm safety margins is the treatment of choice. A regular follow-up is recommended.

Debulking surgery for elephantiasis nostras with large ectatic podoplanin-negative lymphatic vessels in patients with lipo-lymphedema.

OBJECTIVE: Elephantiasis nostras is a rare complication in advanced lipo-lymphedema. While lipedema can be treated by liposuction and lymphedema by decongestive lymphatic therapy, elephantiasis nostras may need debulking surgery. METHODS: We present 2 cases of advanced lipo-lymphedema complicated by elephantiasis nostras. After tumescent microcannular laser-assisted liposuction both patients underwent a debulking surgery with a modification of Auchincloss-Kim's technique. Histologic examination of the tissue specimen was performed. RESULTS: The surgical treatment was well tolerated and primary healing was uneventful. After primary wound healing and ambulation of the patients, a delayed ulceration with lymphorrhea developed. It was treated by surgical necrectomy and vacuum-assisted closure leading to complete healing. Mobility of the leg was much improved. Histologic examination revealed massive ectatic lymphatic vessels nonreactive for podoplanin. CONCLUSIONS: Debulking surgery can be an adjuvant technique for elephantiasis nostras in advanced lipo-lymphedema. Although delayed postoperative wound healing problems were observed, necrectomy and vacuum-assisted closure achieved a complete healing. Histologic data suggest that the ectatic lymphatic vessels in these patients resemble finding in podoplanin knockout mice. The findings would explain the limitations of decongestive lymphatic therapy and tumescent liposuction in such patients and their predisposition to relapsing erysipelas.

A 10-year analysis of cutaneous mesenchymal tumors (sarcomas and related entities) in a skin cancer center.

BACKGROUND: Mesenchymal neoplasms (sarcomas) of skin are rare. Patients with sarcomas were analyzed over the last decade. METHODS: Over a 10-year period, we conducted a retrospective analysis of patients diagnosed and treated in an urban academic teaching hospital in Saxony, Germany. Clinical and pathologic files were used. RESULTS: We identified 65 adult patients with 67 primary cutaneous sarcomas. The mean age was 73.1 (± 15.5) years with a male predominance (78.5%). None of the sarcomas was detected by a skin cancer screening program. The diagnosis was atypical fibroxanthoma (n = 41 patients with 43 tumors), cutaneous angiosarcoma (eight), dermatofibrosarcoma protuberans (two), nodular epithelioid cell sarcoma (one), Kaposi sarcoma (three), leiomyosarcoma (five), malignant fibrous histiocytoma (two), fibromyxoid sarcoma (one), and cutaneous angiomyxoma (two). The preferred tumor localization was the head and neck area (44 patients). Follow-up was 0.5-5.5 years (mean 18 ± 12 months). We observed metastatic spread of atypical fibroxanthoma in 12.5%, demonstrating that this type of sarcoma can run an aggressive course. Mohs surgery is still the cornerstone of treatment, although new options in palliative or adjuvant treatment are available. CONCLUSIONS: Mesenchymal neoplasms (sarcomas) are an important group of cutaneous malignancies. Awareness needs to be improved.

Nevus comedonicus: an updated review.

The intention of this review on nevus comedonicus (NC) is to update on clinical features, pathogenesis, and therapy. NC is a rare epidermal nevus type. It is part of the nevus comedonicus syndrome, a neurocutaneous disorder with ocular, skeletal, and central nervous symptoms. Recently, acne-related signaling pathways and somatic mutations of tyrosine kinase receptors have been identified and may play a role in NC pathogenesis. On preexistent NC secondary skin tumors can develop, which are often benign. Treatment options of NC include topical therapy, laser, and surgery.

A 10-year analysis of primary cutaneous malignant melanoma with sentinel lymph node biopsy and long-term follow-up.

BACKGROUND: Sentinel lymph node biopsy (SLNB) is an important tool for accurate staging of patients with melanoma. There is an ongoing debate whether the procedure provides therapeutic benefits or not. OBJECTIVE: We wanted to analyze 10-year data from an academic teaching hospital. PATIENTS AND METHODS: During 1999-2009, 977 patients with early cutaneous melanoma have been treated. Of these, 419 patients had tumors ≥ 1 mm thickness. Patients with head and neck tumors were excluded, leaving 364 patients (202 men and 162 women). SLNB was not performed in 163 patients but was performed in 201 patients. For correction of bias, tumors >4 mm thickness were excluded from further statistical analysis. RESULTS: The detection rate of SLN was 94.4%. False negative SLN were observed in 8.9%. Adverse effects occurred in 5.5%. The rate of positive SLNB was 16.4% and lymph node involvement 20%. Patients undergoing SLNB had a lower relapse rate (10.6% vs. 33.3%; P < 0.001). The most important finding is an almost 50% lower total death rate and melanoma-related death rate in the SLNB subgroup (P < 0.001 for both). CONCLUSIONS: Sentinel lymph node biopsy is an accurate instrument for melanoma staging to detect occult regional lymph node involvement. Data suggest a positive effect on relapse-free survival. The observation of improved long-term survival needs validation in prospective multicenter trials. The limitations of this study were that it was a single center retrospective analysis.

Management of severe anogenital acne inversa (hidradenitis suppurativa).

BACKGROUND: Severe anogenital acne inversa (AI) is a debilitating chronic inflammatory disease with a major negative effect on quality of life. OBJECTIVES: To evaluate the role of surgery in the treatment of severe anogenital AI. METHODS: We analyzed the records of patients with anogenital AI from 2000 to 2010. Assessment was done using the Hidradenitis Suppurativa Lesion, Area, and Severity Index (HS-LASI), pain visual analogue scale, physician global assessment (PhGA), and patient global assessment (PaGA). Comorbidities and adverse events were analyzed. RESULTS: Sixty-seven patients with a Hurley score of 3 were identified. Mean follow-up was 56.9 ± 41.3 months. A number of comorbidities were observed. After surgery, mean pain scores decreased from 6.3 ± 1.5 to 0.8 ± 0.7, PhGA improved from 6.8 ± 1.2 to 0.9 ± 0.6, PaGA improved from 7.3 ± 1.2 to 1.1 ± 0.5, and HS-LASI decreased from 41.8 ± 21.3 to 2.4 ± 2.8. Adverse reactions were seen in 10.4%. The total relapse rate was 6% (5 patients) with only one case with healing by secondary intention (2% of 49 patients). CONCLUSIONS: Surgery is the cornerstone of treatment for advanced AI. Healing by secondary intention results in significant reduction of complaints and achieves satisfying body contouring.

Giant keratoacanthoma-like cutaneous horn of the upper leg: A case report.

Giant cutaneous horns are suspicious of malignancy, in particular squamous cell carcinoma. We report on a 67-year-old man that developed a giant horn on his upper leg that resembled a keratoacanthoma. The lesion was surgically removed. Histologic investigation revealed a giant verruca vulgaris. No risk factors such as immunosuppression were evident. Giant verruca vulgaris is a very rare cause of giant cutaneous horns. Complete excision is recommended in order not to overlook a malignant skin tumor in such cases.

Pityriasis rubra pilaris--a retrospective single center analysis over eight years.

BACKGROUND: Pityriasis rubra pilaris (PRP) is a rare papulosquamous dermatosis. We evaluated evaluate co-morbidities, complications, and outcome of treatment regimens. PATIENTS AND METHODS: This is a retrospective study at an academic teaching hospital. We analyzed all patients with the definite diagnosis of PRP seen since 2001. Epidemiologic data, co-morbidities, response to and course during treatment were investigated. RESULTS: We identified 10 PRP-patients (6 men, 4 women), mean age 56.4 years, with type I (n = 9) and type IV (n = 1). Three patients had internal co-morbidities (atrial fibrillation with cardiac insufficiency, dilated cardiomyopathy, diabetes mellitus). Two patients needed psychiatric treatment because of depression. PRP caused ectropium (2 x), diffuse effluvium (1 x), and stenosis of the outer ear canal (1 x). We did not observe a spontaneous remission. Among 9 patients with PRP type I, five were treated with acitretin (two of them as Re-PUVA), and two with methotrexate (in one patient combined with fumaric acids). Systemic corticosteroids were not effective. One patient was treated with infliximab i.v., 5 mg/kg body weight. Starting with the first application, inflammatory activity decreased and erythema got paler. The treatment was well tolerated. CONCLUSIONS: PRP type I is a severe, chronic inflammatory dermatosis responding hesitantly to classic systemic therapies. Tumor necrosis factor-alpha antagonists are an effective treatment option for difficult cases.

Fibromyxoid sarcoma of the leg.

A 48-year-old female with an atypical plaque-like lesion of the lower leg is presented in this article. Histologic investigation revealed a rare low-grade fibromyxoid sarcoma (pT1a cN0 cM0; stage Ia) of suprafascial localization. Staging of the patient did not reveal metastatic spread. The tumor was surgically removed with wide safety margins. The defect was closed using a mesh graft transplant and vacuum-assisted closure. Healing was complete. Regular follow-up for at least 5 years is recommended. Besides the rareness of this tumor, this case is also remarkable because of the localization on the lower leg and the suprafascial soft tissue.

Panniculitis ossificans of the lower leg.

The authors present a rare case of panniculitis ossificans (PO) in a 30-year-old woman. Differential diagnoses and treatment are discussed. PO is an important differential diagnosis to sarcomas.

Myxoid neurothekeoma--a painful case in a less common location.

Myxoid neurothekeoma is a rare benign asymptomatic tumor of the skin. Most tumors are located on the head, shoulders and arms. We present a 19-year-old female patient with a relapsing tumor of this type in a less common localization, i.e., lower trunk, and with painful sensations under mechanical pressure. Wide excision surgery led to complete remission.

Dystrophic calcification in chronic leg ulcers--a clinicopathologic study.

BACKGROUND: Dystrophic calcification (DC) is a risk factor for conservative treatment failure in chronic leg ulcers of various pathologies. PATIENTS AND METHODS: We performed a retrospective noncontrolled trial of 212 patients with 362 chronic leg ulcers who underwent ulcer shave excision with subsequent skin grafting. The ulcers existed for at least 3 months, and no healing was achieved with good ulcer care. Tissue was subjected to histopathology (hematoxylin-eosin and van Kossa stains). RESULTS: DC was evident in 39 patients (18%). Metaplastic subcutaneous bone formation was observed in 15 patients (7%). Clinical symptoms associated with DC were resistance to good ulcer care, pain, and ineffective effects of compression therapy (in venous ulcers). Ulcers were treated with deep dermatome shaving of the ulcer bed and surgical removal of DC. In the same setting, defects were closed using mesh graft transplantation. The procedure achieved a complete take rate in 80% and a significant decrease of pain in 95% of cases. When comparing the take rates in patients with and without DC, DC had a negative effect on outcome (take rate: 91% without DC vs 80% with DC, p<.05). CONCLUSIONS: DC is resistant to conservative treatment. The first-line treatment is deep ulcer dermatome shaving and complete removal of calcifications whenever possible.

Granulomatous tattoo reaction and erythema nodosum in a young woman: common cause or coincidence?

Tattooing has become quite popular in Western countries. With the increasing prevalence, there is also an increased risk of adverse effects. We describe a 17-year-old female patient with a black and red-colored tattoo, who developed immediately after red tattooing general malaise with fever, nausea, and vomiting. A bullous reaction was temporarily seen within the red part of her tattoo. The reaction later shifted to a subacute dermatitis with bacterial superinfection. Two months later, she felt ill again. She developed painful tender nodules on the anterior aspect of both lower legs identified as erythema nodosum without sarcoidosis. Is this is a unique case of adverse reaction to tattoo pigments with a type I and a type IV reaction, or is this a coincidence? The treatment was initiated with systemic and topical corticosteroids and topical antibiotics combined with compression bandages for the legs. After 3 weeks of treatment, the erythema nodosum completely resolved and did not reappear during a 1-year follow-up. The treatment of the local reactions, however, was unsatisfactory without complete response. There is an indispensable need for regulation of tattoo pigments and tattooing to improve consumer safety.

Deep ulcer shaving combined with split-skin transplantation in distal calciphylaxis.

Calciphylaxis is a cause of painful deep ulcers. There is controversy about best wound management in this disease. A retrospective study of inpatients during the 3 years was made. Seven calciphylaxis patients were identified. All patients suffered from various associated pathologies including diabetes mellitus type II and chronic renal insufficiency. Ulcers were treated by aggressive and deep shaving combined with autologous split-skin grafting in the same session. A 30% to 90% take rate of the grafts eventually with a complete ulcer healing in 6 of 7 patients was achieved. No patient developed a deep cutaneous infection or sepsis. All patients are still alive except one. The single death was related to cardiovascular complications. In distal calciphylaxis, aggressive ulcer surgery with defect closure offers a marked improvement in quality of life and prevents early deep skin infections and sepsis as major causes of mortality.

Tumor necrosis factor-alpha inhibitor-induced psoriasis or psoriasiform exanthemata: first 120 cases from the literature including a series of six new patients.

Tumor necrosis factor-alpha (TNFalpha) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients from the literature including six new patients (three women and three men) who developed pustular lesions during treatment with TNFalpha inhibitors. We identified 72 women and 36 men (several papers did not specify the gender of patients) with an age range of 13-78 years (mean 42.3 years). The primary diagnoses were rheumatoid arthritis (n = 61), ankylosing spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFalpha treatment (n = 73), followed by palmoplantar pustular psoriasis (n = 37) and psoriasis of the nail (n = 6), sometimes combined in the same patient. Palmoplantar pustulosis and psoriasiform exanthema was the diagnosis in ten patients each. A positive personal history of psoriasis was recorded in 25 patients. A positive family history was noted in eight patients. No data about personal (n = 7) or family history (n = 46) were available in a number of patients. Newly induced psoriasis was diagnosed in 74 patients whereas an exacerbation or aggravation of a pre-existing psoriasis was noted in another 25 patients. All three TNFalpha inhibitors available on the market were involved: infliximab (63 patients), etanercept (37 patients), and adalimumab (26 patients). Several patients were treated with more than a single TFNalpha inhibitor. The timing of cutaneous adverse effects (psoriasis and psoriasiform rash) varied considerably among patients, ranging from after a single application to a delayed response of up to 63 months after initiation of treatment. The mean time to appearance of the cutaneous adverse effect for all TNFalpha inhibitors was 9.5 months. Cessation of the responsible TNFalpha inhibitor was carried out in 47 patients either alone or in association with adjuvant anti-psoriatic therapy (mostly topical). This resulted in complete remission in 21 patients, partial remission in 20 patients, and stable disease in another three patients; in the other three patients, the outcome was not reported. TNFalpha inhibition was continued in 47 patients but anti-psoriatic adjuvant therapy was introduced. The outcome in this group was complete remission in 22 patients, partial remission in 25 patients, and stable disease in 2 patients. The response rate (complete remission plus partial remission) was 93.2% and 95.9%, respectively, in each group. In six patients, switching from one TNFalpha inhibitor to another one immediately after cutaneous adverse effects occurred resulted in an improvement in five patients. In nine patients, a second TNFalpha inhibitor was initiated after a break in TNFalpha inhibition. The response to a second or third drug in these patients was mixed. The underlying pathomechanisms of induction of psoriasis or psoriasiform exanthemata by TNFalpha inhibitors remain elusive but there is reason to assume that induction of such adverse events has more than one pathophysiology.

Isolated plexiform schwannoma of the hand.

BACKGROUND: The plexiform schwannoma, a peripheral nerve sheath tumor, is a very rare entity. But dermatologists should be familiar with since they may be the first who make diagnosis possible by taking a deep biopsy. MAIN OBSERVATION: A 24-year-old male presented with multiple asymptomatic subcutaneous nodules of the palmar side of his right hand. Histologic investigations revealed a plexiform schwannoma with numerous Antoni-A areas. There was no evidence of neurofibromatosis type 1 or 2. CONCLUSIONS: Plexiform schwannoma of the hand is a rare nerve sheath tumor. In individual (symptomatic) cases hand surgery is an option that needs a critical indication. In every case histologic investigations are mandatory to confirm the diagnosis and not to overlook the malignant variant of this disease.

Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma.

Disabling pansclerotic morphea of childhood (DPMC) is a rare and severe variant of scleroderma. This report presents 3 cases that presented to the authors and studies 25 patients from the literature (English language only) for the presence of chronic nonhealing ulcers of skin and skin cancer. The authors identified a total of 30 patients (9 male and 21 female) aged between 1 and 37 years at time of presentation. All cases were less than 14 years old when the disease started. The majority of patients had an aggressive course with deep sclerotic lesions leading to joint contractures and immobility. Five patients suffered from chronic nonhealing leg ulcers (17%), but ulcers were present on other parts of the body (upper limbs, trunk, head) as well (n = 6). Four patients died because of complications of the disease such as sepsis or gangrene. Two patients developed a squamous cell carcinoma at the age of 16 years and 19 years, respectively (6.7%). The available treatment of DMPC-associated ulcers is unsatisfying. Only temporary improvements have been seen in a minority of patients. We report on marked improvement of chronic leg ulcers by a combination of sildenafil 3 x 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix.

Painful ANA-positive scleroderma-like disease with acral ulcerations: a case of chronic gangrenous ergotism.

Chronic ergotism is a rare cause of limb ischemia. In this case report, the authors present a 62-year-old woman with history of long-term use of ergotamine alkaloids for the treatment of menstrual pain, who developed a severe painful disease initially misdiagnosed as systemic sclerosis (scleroderma) for 3 decades. She presented with a combination of acral gangrene, foot ulcer, renal obstruction, mild pulmonary fibrosis, and reduced esophageal motility. Right-sided renal obstruction was evident. The condition was extremely painful and had led to muscular contractions and immobility, drug abuse, and anemia. After establishing the diagnosis of chronic gangrenous ergotism, changing drug therapy, mobilization, and treatment of chronic wounds, she showed a remarkable recovery. Eventually the foot ulcer was closed successfully using a mesh graft transplantation, and the patient was able to walk alone. Chronic ergotism is rare but has to be taken into account when presented with painful chronic digital and foot ulcers.

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.