Pancreatic panniculitis with neuroendocrine pancreatic carcinoma

Pancreatic panniculitis is a rare complication occurring in 0.3-3% of patients with pancreatic disease. Clinical features include erythematous painful subcutaneous nodules usually on the lower leg and foot. A 72-year-old man was diagnosed in 2002 with a neuroendocrine carcinoma of the head of the pancreas. In 2003 following surgery and radiation therapy, he developed liver metastases and painful nodules on his legs. Lipase was found to be markedly elevated; amylase and alpha1-antitrypsin were in the normal range. Histopathologic examination of a nodule showed subcutaneous fat necrosis with ghost cells surrounded by an acute inflammatory infiltrate. The pathogenesis of pancreatic panniculitis is unclear. The pancreatic enzyme lipase may induce lipolysis and fat necrosis with secondary tissue inflammation.     

Pankreopathische Pannikulitis bei neuroendokrinem Karzinom des Pankreas

Pancreatic panniculitis is a rare complication occurring in 0.3–3% of patients with pancreatic disease. Clinical features include erythematous painful subcutaneous nodules usually on the lower leg and foot. A 72-year-old man was diagnosed in 2002 with a neuroendocrine carcinoma of the head of the pancreas. In 2003 following surgery and radiation therapy, he developed liver metastases and painful nodules on his legs. Lipase was found to be markedly elevated; amylase and α1-antitrypsin were in the normal range. Histopathologic examination of a nodule showed subcutaneous fat necrosis with ghost cells surrounded by an acute inflammatory infiltrate. The pathogenesis of pancreatic panniculitis is unclear. The pancreatic enzyme lipase may induce lipolysis and fat necrosis with secondary tissue inflammation.     

Pancreatic Panniculitis Associated with Acinar Cell Carcinoma of the Pancreas: A Case Report

Pancreatic panniculitis is a rare type of disorder associated with pancreatic diseases. We describe here a case of 54-year-old man who was admitted to the Department of Dermatology with the diagnosis of erythema nodosum. The patient presented with a 9-month history of painful erythematous nodules on the extremities, joint pain and swelling, and weight loss. A highly elevated level of pancreatic lipase was found on the laboratory examinations. The biopsy specimens from the skin lesions showed subcutaneous fat necrosis. Abdominal computed tomography (CT) revealed a large mass with central necrosis in the body and tail of the pancreas. Distal pancreatectomy, splenectomy and partial transverse colectomy were successfully performed on day 17 of the hospitalization. The histopathologic findings supported the diagnosis of acinar cell carcinoma of the pancreas (ACCP). Postoperatively, the level of serum lipase returned to normal, and the skin lesions and joint manifestations gradually regressed. However, the swelling did not significantly resolve in the left knee. In view of the non-specific clinical presentation of this disease, clinicians should be alert and have a high index of suspicion for pancreatic panniculitis.     

Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis

Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology. It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. We report the case of a 42-year-old woman with a history of progressive systemic sclerosis who presented with painful subcutaneous nodules on her abdomen along with chronic atrophic papules on her upper and lower limbs. Biopsy results of both types of lesions revealed vascular thrombi without surrounding inflammation. We briefly review the literature on MAP and its association with various connective tissue diseases. To our knowledge, there have been no previous reports of a patient with the clinical and histologic presentations described here. Although the histologic appearance of the subcutaneous nodules was very similar to that of the atrophic papules, the clinical characteristics of the 2 types of lesions were strikingly different. It is fair to theorize that Degos lesions do not start as atrophic porcelain-white papules but rather evolve from a primary lesion. We hypothesize that these lesions start as painful red nodules and may represent part of the disease spectrum in the evolution of MAP.     

Mixed nonseminomatous germ cell tumor presenting as a subcutaneous tissue mass

Extragonadal germ cell tumors most commonly arise in the midline of the retroperitoneum or the mediastinum. Primary tumors involving the skin are very rare. Only one case of malignant primary germ cell tumor located in the skin has been reported. We present the case of a 44-year-old white man with a primary subcutaneous mixed nonseminomatous germ cell tumor. This man had a long-lasting subcutaneous lump of the breast, which became painful. Surgery revealed 3 juxtaposed nodules. Microscopic examination showed a mixed germ cell tumor with a 90% immature teratoma component and a 10% embryonal carcinoma component. Testicular ultrasound and computed tomography of the chest, abdomen, pelvis, and brain were normal. Serum human chorionic gonadotrophin, beta-human chorionic gonadotrophin, alpha-fetoprotein, and lactate dehydrogenase were within normal ranges. A further surgical excision was performed. The patient is presently alive with no evidence of disease after a follow-up of 7 years. Review of the literature indicates that primary cutaneous extragonadal germ cell tumors usually occur as cutaneous or subcutaneous solitary nodules or as ulcerated lesions. They mainly consist of mature teratomas in children. Only 2 cases have been reported in adults.     

Farber Disease in a Newborn

Abstract:   Farber disease is a rare lysosomal storage disease characterized by a clinical triad including painful joint deformity, subcutaneous nodules and hoarseness, due to progressive granulomatous inflammation. We report the case of an early presentation on a female infant who manifested typical signs 1 week after birth.     

Subcutaneous nodules in Whipple''s disease

BACKGROUND: Cutaneous findings other than hyperpigmentation are rare in Whipple's disease. CASE REPORT: We present the case of a 59-year-old man previously diagnosed with Whipple's disease by duodenal biopsy, who developed red-brown, painful, subcutaneous nodules on the buttocks, thighs, arms and legs. Biopsy of these nodules showed a septal panniculitis and foamy macrophages containing PAS-positive, diastase resistant intracytoplasmic material, characteristic of Whipple's disease and similar to that observed in the duodenal biopsy. Ultrastructurally, this material in the histiocytes corresponded to degenerated bacilli. CONCLUSIONS: This is the fourth documented case of subcutaneous involvement by Whipple's disease. One should consider the possibility of Whipple's disease in any patient who presents with symptoms compatible with that condition who demonstrates septal panniculitis with a large amount of foamy histiocytes.     

皮下型Sweet综合征

报告1例皮下型Sweet综合征.患者女,58岁.眼睑、四肢红色结节伴发热1周.皮肤科检查:左上眼睑一直径2 cm的红色结节,边缘隆起似假水疱;前臂伸侧、小腿伸侧及腕、肘、膝、踝部可见十余处直径1～3 cm的红色结节,有压痛,无破渍,无渗出.皮损组织病理检查:表皮、真皮大致正常,脂肪小叶大量中性粒细胞浸润,可见白细胞碎裂.诊断:皮下型Sweet综合征.     

Silicone breast implant rupture presenting as bilateral leg nodules

Gross migration of silicone gel from ruptured breast implants is a rare event. It is associated with extravasation of gel into the breast parenchyma, and to distant locations such as the abdominal wall and inguinal areas. This silicone deposits present as subcutaneous nodules and cause a local reaction known as siliconoma. We evaluated a 56-year-old woman who presented with a 2-year history of painful, firm and ill-defined subcutaneous nodules on the medial aspect of the shins and ankles. Her medical history was notable for bilateral breast augmentation with silicone implants 30 years before presentation. Although there were no signs or symptoms on breast examination, ultrasonography and magnetic resonance imaging confirmed that both implants had ruptured. Histological examination of a punch biopsy from a nodule on the shin found lobular granulomatous panniculitis. An excisional biopsy of the lesion was analysed by scanning electron microscopy and was found to contain silicone. This is a rare case of gross migration of silicone to the shins, originating from ruptured breast implants. To our knowledge, there is no previous report of silicone migration to such a distant location. We discuss the common presentation of silicone migration and highlight the importance of awareness among dermatologists and plastic surgeons about this unusual occurrence.     

Nodular subcutaneous fat necrosis. A manifestation of silent pancreatitis.

A 57-year-old man with asymptomatic pancreatitis developed tender subcutaneous nodules with fat necrosis. These nodules were the sole clinical manifestation of the underlying disease. This case demonstrates that silent pancreatitis should be considered in the differential diagnosis of painful leg nodules.     

Erythematous nodules of the hand: a rare site of metastatic breast carcinoma

Acral cutaneous metastasis is a rare phenomenon. A 38-year-old woman presented with painful lesions in the left hand since 1 month. On examination, multiple painful, erythematous, lobulated plaques and nodules were present over the palmar aspect of the finger tips and thenar eminence. Biopsy of the nodule revealed lobules of small pleomorphic epithelial cells arranged in singles, small nests, cords and small abortive luminal pattern in an abundant mucoid matrix, suggestive of metastatic mucinous carcinoma. Past history revealed that the patient had undergone bilateral mastectomy for carcinoma breast. Diagnosis of cutaneous metastases is based on clinical history and histopathological examination. Identification of the primary tumor is very important for prompt treatment and prognosis. The rarity of pure acral cutaneous metastasis and its possible pathogenetic mechanisms are discussed.     

Primary "cutaneous" T-cell anaplastic large cell lymphoma, CD30+, neutrophil-rich variant with subcutaneous panniculitic lesions, in a post-renal transplant patient: report of unusual case and literature review

Posttransplantation lymphoproliferative disorders (PTLD) presenting clinically in the skin are rare and usually of B-cell phenotype. Only 7 cases of cutaneous T-cell PTLD have been previously reported, mostly mycosis fungoides type, with no known cases of "cutaneous" presentation by CD30 (Ki-1) anaplastic large cell lymphoma (ALCL).The case reported is a 59-year-old male who developed multiple skin nodules on the right leg, 6 years following renal transplantation. Initial biopsy showed ALCL involving the dermis with a background rich in neutrophils. The neoplastic cells were of T-cell phenotype, strongly CD30 with typical staining, and BCL-2 positive, but P53 negative. No EBV was detected by IHC, ISH, or DNA analysis. One year later, he developed painful subcutaneous nodules with surrounding erythema, resembling deep pustules or panniculitis, which on biopsy showed preferential involvement of the subcutaneous fat and prominent component of neutrophils. Twenty-two months following diagnosis, he died of cardiac failure with terminal myocardial infarct. There was however no clinical evidence of systemic spread of the lymphoma.This report adds to the clinical and morphologic spectrum of these rare "cutaneous" lymphomas of T-cell lineage arising in the posttransplantation setting, and suggests that EBV does not play a role in their pathogenesis.     

皮下型急性发热性嗜中性皮肤病1例

报告1例皮下型急性发热性嗜中性皮肤病。患者男,47岁。颈部、躯干、四肢起疼痛性红斑、结节伴发热1周。组织病理检查示真皮、皮下脂肪中有中性粒细胞、淋巴细胞浸润,可见核碎裂像。     

多发性小汗腺血管瘤样错构瘤一例

报道1例多发性小汗腺血管瘤样错构瘤.小汗腺血管瘤样错构瘤是一种少见的皮肤肿瘤.病理表现为增多的小汗腺结构和大量血管成分.本例患儿8岁,左前臂多发性皮下结节伴疼痛4年,近1年局部出现多汗、多毛.经组织病理检查结合临床表现,诊断为小汗腺血管瘤样错构瘤.浅层x线照射后效果不明显,后进行分次手术切除,疗效较佳.提示治疗小汗腺血管错构瘤选择适当的手术方案是可行的.     

Childhood erythema nodosum associated with kerion celsi: a case report and review of literature

Erythema nodosum (EN) in association with kerion celsi is a rare condition in children, with only 11 cases having been reported in the English literature. We describe a new case in a 7-year-old boy in whom the disorder had begun 2 months before. He had many inflamed, boggy, suppurative nodules over the left occipitoparietal area of the scalp and, 2 weeks later, developed multiple painful, erythematous subcutaneous nodules of the shins, thighs, and upper limbs. EN was confirmed by histologic examination. Our review of the literature of all cases of EN during kerion showed that it usually occurs at or slightly after the height of infection or after drug introduction. Trichophyton mentagrophytes was involved in the majority of cases, and improvement of EN usually occurs with griseofulvin.     

Signet ring cells in carcinomatous lymphangitis due to gastric adenocarcinoma

Cutaneous metastases are rare. They usually present as nodules or tumors. Diagnosis is based on histopathological examination and prognosis is unfavorable. This report describes the case of a female patient, 72 years old, with surgically treated gastric antrum adenocarcinoma. Pathology showed poorly differentiated adenocarcinoma with signet ring cells. It evolved with bone involvement, lymph node enlargement in the inguinal region, and skin infiltration in the lower limbs, abdomen, and root of the upper limbs. Skin biopsy demonstrated signet ring carcinoma embolizing the dermal and hypodermic vessels and invasion of adipose tissue, confirming carcinomatous lymphangitis. Carcinomatous lymphangitis is the cutaneous and subcutaneous lymphatic invasion by tumor cells. Cutaneous metastasis is relatively uncommon and presents mainly as cutaneous or subcutaneous nodules, and more rarely as inflammatory lesions. The present case reports carcinomatous lymphangitis associated with gastric cancer.     

Subcutaneous fat necrosis associated with pancreatic islet cell carcinoma.

A 75-year-old man with generalized subcutaneous fat necrosis was found to have an islet cell carcinoma of the pancreas. The histologic diagnosis of islet cell carcinoma was confirmed by electron microscopy, which showed characteristic intracytoplasmic granules. This is the second report of the association of islet cell carcinoma of the pancreas with generalized subcutaneous fat necrosis.     

Metastatic laryngeal large cell neuroendocrine carcinoma: A rare case of presentation and extreme tumor burden

Large cell neuroendocrine carcinoma (LCNEC) of the larynx is an aggressive form of neuroendocrine carcinoma that affects smokers at an average age of 60 years. LCNEC is characterized by large cells with round to ovoid nuclei distributed in a trabecular or nested growth pattern. Previously, laryngeal LCNEC and atypical carcinoid tumors were considered identical; however, laryngeal LCNEC has been shown to have higher mitotic rates and worse prognosis, which has led to laryngeal LCNEC's being separated from atypical carcinoid and classified as a poorly differentiated neuroendocrine carcinoma in the most recent World Health Organization classification. We present a case of a 56‐year‐old female who presented with painful subcutaneous skin lesions that were diagnosed as metastatic carcinoma at an outside facility. Subsequent workup revealed a primary epiglottic lesion. Over the next 4 years, she continued to develop over 100 similar subcutaneous nodules. Additional workup confirmed neuroendocrine differentiation, thus clarifying the diagnosis of metastatic LCNEC. Review of literature has revealed only 1 reported case of LCNEC with skin metastasis. This is the first reported case in which skin metastasis was the initial presenting symptom; moreover, our case is unique with regard to the heavy metastatic burden to the skin.     

Aluminium hydroxide granulomas: light and electron microscopic studies and X-ray microanalysis

Three children are described in whom painful subcutaneous nodules developed 1–2 years after the injection of adsorbed diphtheria, tetanus and pertussis vaccine. The diagnosis of aluminium hydroxide granuloma was established by ultrastructural examination with X-ray microanalysis.     

A case of rheumatoid nodulosis successfully treated with surgery

We report the case of a 76 year-old male with multiple subcutaneous nodules, but without joint symptoms or deformities, who was diagnosed histopathologically with rheumatoid nodulosis after resection of the nodules. Rheumatoid nodulosis is a disease characterized by multiple subcutaneous nodules that are histopathologically similar to rheumatoid nodules, a high titer of rheumatoid factor, and radiologically detectable cystic bone lesions, but with none or few of the systemic manifestations orjoint activity of rheumatoid arthritis (RA). It is considered to be a benign variant of RA. This rare disease must be considered when a case of multiple subcutaneous nodules is encountered, even in aged males withoutjoint symptoms or deformities.