Epidermoid Cysts - A Wide Spectrum of Clinical Presentation and Successful Treatment by Surgery: A Retrospective 10-Year Analysis and Literature Review.

Epidermoid cysts are common benign lesions of hair-bearing, and less often glabrous skin. They can also occur in oral mucosa and internal organs. In case of cutaneous lesions, an epidermal punctum is a clinical diagnostic hallmark. The clinical presentation is variable leading to some differential diagnoses. Diagnosis of epidermoid cysts needs histopathological confirmation - not only of the potential of malignant transformation. The treatment of choice is surgery. We report a retrospective analysis of 2159 epidermoid cysts treated surgically. Most of the cases can be performed under local anaesthesia. The complication rate of 2.2% is low. To avoid relapses, the cyst wall has to be removed completely. Rare genetic disorders with multiple cysts are Gardner and Lowe syndrome.

Dermatofibrosarcoma Protuberans: Retrospective Single Center Analysis Over 16 Years.

Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control. We observed no recurrence and no metastatic spread. We discuss prognostic factors and emerging treatments.

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.

Cutaneous Langerhans cell histiocytosis : The spectrum of a rare cutaneous neoplasia.

Langerhans cell histiocytosis (LCH) is a rare disorder that is now recognized as a true malignancy of dendritic Langerhans cells. Various somatic mutations have been discovered for BRAF and MAP kinases. The clinical variability is remarkable. A very rare variant is cutaneous LCH without internal organ manifestations. We present a case series of cutaneous LCH illustrating the spectrum of clinical symptoms, treatment, and disease course. Cutaneous LCH needs a careful investigation of possible internal involvement and a long follow-up since second malignancies may develop over time. Overtreatment should be avoided, since too-aggressive treatment will not improve the outcome.

Scalping Surgery - Dermatologic Indications beyond Curative Primary Skin Cancer Surgery.

Skin tumours are among the most frequent tumour types of mankind. In the case of large tumours, field cancerization, or satellitosis scalping surgery is a possible option. The procedure can also be used in a palliative setting with tumour debulking. Less common indications are multiple benign tumours of the scalp and chronic inflammatory scalp dermatoses not responding to medical treatment. We present a case series and discuss surgical modalities beyond curative surgery of primary skin cancer.

Pyogenic Granuloma - A Common Benign Vascular Tumor with Variable Clinical Presentation: New Findings and Treatment Options.

Pyogenic granuloma is a common benign vascular tumour occurring in all ages. Both skin and mucous membranes can be affected. Of pathogenetic importance are trauma, BRAF mutations and probably herpes virus type 1, Orf virus and/or human papilloma virus type 2. The tumour consists of capillary proliferations, venules and fibromyxoid stroma. The development of a lesion occurs in three stages and bleeding is a common symptom. The tumour can mimic various other vascular lesions, solid tumours, and soft tissue infections. In recent years, targeted tumour therapies have become the most common cause of drug-induced pyogenic granulomas. The backbone of treatment is surgical procedures including laser therapy. New developments in medical drug therapy include topical and systemic beta-adrenergic receptor antagonists timolol and propranolol. Drug therapy is an alternative for young children, ocular and periungual pyogenic granuloma.

Rapid Evolving Unilateral Indurated Oozing Facial Plaques in a Patient with Head-and-Neck Cancer: Peripheral T-Cell Lymphoma Not Otherwise Specified (NOS).

BACKGROUND: The sudden development of facial plaques and nodules may be an alarming clinical sign for underlying malignancies. Nevertheless, a broad range of inflammatory and infectious diseases must be considered as well in the differential diagnosis. CASE REPORT: We report on a 53-year-old male patient with a left-sided cheek infiltration with oozing but no lymphadenopathy. He had a medical history of head-and-neck cancer. The primary differential diagnosis was herpes zoster with secondary impetiginization or pyoderma facial. About eight weeks later, the patient presented with progressive formation of nodules and plaques on the face and isotretinoin was stopped. Skin biopsy suggested mycosis fungoid and an oral treatment with bexarotene was started. After limited response for another eight weeks, he returned later with massive facial swelling, nodules and impetiginization. Another skin biopsy was performed to exclude diagnostic error or investigate possible disease progression. Microscopic evaluation and multiplex-polymerase chain reaction confirmed the diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTL-NOS), stage Ia (T1 N0 M0). Imaging techniques excluded metastatic spread. By interdisciplinary tumour board, R-CHOP (rituximab, cyclophosphamide, hydroxyl-doxorubicin, vincristine, and prednisolone) was recommended and initiated by hemato-oncologists. CONCLUSIONS: PLT-NOS confirmed in the present patient has a poor prognosis with a 5-year survival rate of less than 20%.

Sentinel lymph node biopsy in early melanoma-comparison of two techniques for sentinel removal.

BACKGROUND: Sentinel lymph node biopsy is an established technique for melanoma staging. OBJECTIVE: There are no studies available comparing different techniques for the removal of sentinel lymph nodes related to safety and postsurgical complication rate. METHODS: This is a retrospective single-center trial. We analyzed the postsurgical complications in 201 consecutive melanoma patients with ligature of lymphatic vessels by sutures (group A) and in 91 consecutive patients with occlusion of lymphatic vessels by bipolar tweezers (group B). We paid particular attention to complications related to disturbed lymphatic function, such as lymph edema, lymphatic fistula, and seroma. RESULTS: The complication rate was 5.5 % (group A) and 9.6 % (group B) which is in the range of other published trials (p = 0.89). There was no increase of complications related to lymphatic vessels in group B, although the rate of patients with more than two sentinel lymph nodes removed was 5­times higher than in group A. CONCLUSIONS: Removal of sentinel lymph nodes with the use of bipolar tweezers does not increase the risk of postsurgical complications, and in particular it is not associated with a higher rate of complications related to lymphatic vessel dysfunction.

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.

Pegylated liposomal-encapsulated doxorubicin in cutaneous composite lymphoma: A case report.

BACKGROUND: Cutaneous composite lymphomas are very rare. Their treatment depends upon the different contributing lymphoma entities. Peripheral T-cell lymphoma, not otherwise specified, (PTCL-NOS) represents an aggressive lymphoma subtype. Follicular cutaneous B-cell lymphoma (FCBCL) runs an indolent course. Treatment with pegylated liposomal encapsulated doxorubicin (PLE-DOXO) has yet not been reported in this entity. CASE PRESENTATION: A 73-year-old male patient presented with 3 rapidly growing, painful nodules on his left leg. He was diagnosed as composite cutaneous lymphoma consisting of PTCL-NOS and FCBCL. All lesions had been surgically removed. Staging was unremarkable. After 4 months a relapse occurred with involvement of inguinal lymph nodes and systemic treatment with PEL-DOXO 20 mg/ m every 3 weeks was initiated. After 6 cycles PLE-DOXO, which were well tolerated without grade 3 or 4 toxicities, a mixed response was obtained with complete remission of cutaneous lesions.Lymph nodes were treated by radiotherapy. A second relapse occurred after 8 months and various polychemotherapy regimens were applied without remission. The overall survival was 28 months. CONCLUSION: PEL-DOXO is a possible initial systemic treatment in case of PCTL-NOS. Whether polychemotherapy offers an advantage for survival remains questionable but further investigations are needed.

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.

Chronic Expanding Organized Hematoma of the Lower Leg: A Rare Cause for Nonhealing Leg Ulcers.

Chronic expanding hematoma is a rare entity on the leg. A 55-year-old women presented with 2 small nonhealing leg ulcers. On examination we observed a painless bulky tumor-like mass that developed slowly after deep soft tissue infection almost 2 years ago. Vascular computed tomography suggested an organized hematoma. Important differential diagnoses include sarcoma and lymphoma. Treatment of choice is surgery. Histology confirmed the diagnosis of an organized hematoma. Chronic expanding hematoma is a rare cause of nonhealing leg ulcers.

Basal cell carcinoma of the outer nose: overview on surgical techniques and analysis of 312 patients.

BACKGROUND: Basal cell carcinoma of the nose is common, with a potential of local recurrence and high-risk features. MATERIALS AND METHODS: We provide a review on anatomy of the nose, tumour surgery and defect closure on the nose. We analysed our own patients with nasal BCC of a 24 months period. RESULTS: We identified 321 patients with nasal BCC. There was a predominance of female patients of 1.2 to 1. The mean age was 74.8 years. Slow Mohs technique was employed for all tumours until 3D tumour-free margins were achieved. That resulted on average in 1.8 ± 0.7 Mohs stages. The most common histologic types were solitary (n = 182), morpheic (79), and micronodular (20), Perineural infiltration was evident in 56 tumours. Primary closure after mobilisation of soft tissue was possible in 105 BCCs. Advancement flaps were used in 91 tumours, rotation flaps in 47, transposition flaps in 34 tumours, and combined procedures in 6 cases. In 36 patients full-thickness skin grafting was performed. In two patients healing by second intention was preferred. Partial flap loss was seen in four patients (1.4%). All of them had significant underlying pathologies. None of the tumours treated showed a relapse during the observation time. However, this is a limitation of the present study since follow-up was on average only 10 months. CONCLUSIONS: BCCs of the nose are common. Only 3D-controlled micrographic surgery (Mohs or slow Mohs) guarantee a high rate of complete tumour removal and a very low risk of recurrence.

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.