Nodular cystic fat necrosis with systemic sclerosis

Nodular cystic fat necrosis is a distinct spectrum characterized clinically by mobile subcutaneous nodules and histologically by encapsulated fat necrosis. We describe herein a case of nodular cystic fat necrosis in a patient with systemic sclerosis in the atrophic stage. Several mobile, firm nodules were surgically removed from the flexural aspect of the forearm and lower leg. Histopathology showed features of nodular cystic fat necrosis with lipomembranous changes and calcification. Of interest, lipomembranous changes were seen also in the biopsied specimen from the extensor aspect of the forearm of scleroderma. We speculate that multiple, chronic, local or systemic events causing a compromise in the blood supply of the subcutaneous tissues may contribute to the induction of lipomembranous changes in the affected skin as well as nodular cystic fat necrosis in this case.     

A Case of Nodular Cystic Fat Necrosis: The End Stage Lesion Showing Calcification and Lipomembranous Changes

Nodular cystic fat necrosis, first described by Przyjemski et al. in 1978, is a distinct, benign subcutaneous lesion characterized histologically by encapsulated fat necrosis. We report a case of nodular cystic fat necrosis in a 22-year-old man who had had two mobile, rice-sized, deep-seated papules on his right shin for ten years after trauma. Histologically, the excised mass showed encapsulated fat necrosis, calcification, and lipomembranous changes. The encapsulation of necrotic tissue may prevent further extension of adiponecrosis. The subcutaneous fat is prone to trauma or ischemia. The observation of lipomembranous changes in nodular cystic fat necrosis seems to support the concept that lipomembranous change is a nonspecific pattern of the fat necrosis due to multiple local or systemic events causing a compromise in the blood supply of the subcutaneous tissue.     

以脂膜炎为皮肤表现的皮肌炎九例研究

目的 探讨以脂膜炎为皮肤表现的皮肌炎的临床、病理特点.方法 收集2012年10月至2016年7月复旦大学附属中山医院皮肤科诊治的9例以脂膜炎为皮肤表现的皮肌炎患者的资料,分析其临床与病理特征.结果 9例中女6例,男3例,年龄28～73岁.9例患者脂膜炎皮损均表现为触痛性斑块或结节,分别发生在臀、股、腰、背、腹、上肢和腮部,先于、晚于或与皮肌炎特征性皮损和肌肉损害同时发生,其中1例脂膜炎先于皮肌炎特征性皮损30年发生.皮损组织病理示基底细胞液化变性,真皮血管周围及脂肪小叶和间隔内以淋巴细胞、浆细胞为主的炎性浸润,可有脂肪细胞坏死、钙化和膜囊性改变,血管壁纤维素样坏死和管腔闭塞.脂膜炎先于皮肌炎特征性皮损发生的2例患者曾被数次误诊为狼疮性脂膜炎和深部硬斑病.多数患者对联用糖皮质激素和免疫抑制剂的治疗反应良好,但伴脂肪膜性坏死者,对治疗反应差.结论 皮肌炎的脂膜炎表现具有一定组织学特征,与皮肌炎典型皮损的发生可不同步,在皮肌炎典型损害前出现易被误诊,持续跟踪随访对确诊具有重要意义.     

Traumatic panniculitis

Traumatic panniculitis refers to changes in the subcutaneous fat related to physical or chemical agents. The clinical picture of traumatic panniculitis is nonspecific. Cutaneous lesions are indurated, warm, red, subcutaneous plaques or nodules not necessary related to the intensity of the injury. The histologic picture includes fat microcysts surrounded by histiocytes, collections of foam cells, and inflammatory cells. Late lesions may show fibrosis, lipomembranous changes, or dystrophic calcic deposits. Traumatic panniculitis is usually a self-limiting disorder and requires only symptomatic treatment.     

Lipomembranous changes and calcification associated with systemic lupus erythematosus

Lipomembranous changes are histological findings without specific clinical correlations, and are thought to be related to vascular impairment in fatty tissue. We describe a case of systemic lupus erythematosus (SLE) in which subcutaneous induration of the lower leg showed lipomembranous changes and calcification. This patient showed thrombophilia with thrombosis of the femoral and iliac veins, and cerebellar and myocardial infarction. A biopsy specimen from the subcutaneous tissue surrounding the multiple leg ulcers revealed degeneration of fat cells, lipomembranous changes in the fatty tissues, and calcification. Another biopsy specimen from the outer edges of the ulcers showed microthrombi and fibrinoid changes in the vessel walls, and mononuclear cell infiltrates in the dermis. Lipomembranous changes have been seen in connective tissue disorders such as lupus profundus, morphoea, systemic sclerosis, and panniculitis associated with dermatomyositis, but rarely associated with SLE. We speculate that ischaemic changes due to the vascular disturbance of the lower legs may have induced lipomembranous changes and calcification in the subcutaneous tissues in the present case.     

Nodular Cystic Fat Necrosis with Calcification in a Patient with Juvenile Dermatomyositis

Nodular cystic fat necrosis is a rare, benign form of encapsulated fat necrosis with distinct histology, characterized by cystic fat necrosis with lipomembranous changes and, in later stages, calcification. We report the case of a 7‐year‐old child with juvenile dermatomyositis who presented with three asymptomatic, firm, mobile nodules on the arms and neck. Histology was consistent with nodular cystic fat necrosis with prominent calcification. This is an unusual presentation of this entity because it has never been previously reported in association with juvenile dermatomyositis.     

Creatine kinase values during isotretinoin treatment

A case of subcutaneous fat necrosis (SCFN) in a newborn who had suffered from asphyxia secondary to meconium aspiration is described. During the third week of life, bluish-red subcutaneous nodules were noticed. A biopsy of the lesions showed subcutaneous necrosis with fat cells containing needle-like crystals with reactive inflammatory infiltrates. Peripheral transient thrombocytopenia was observed prior to the appearance of subcutaneous fat necrosis. The good prognosis is stressed.     

Lipodermatosclerosis: a clinicopathologic study of 17 cases and differential diagnosis from erythema nodosum

Background: The clinical manifestations of lipodermatosclerosis (LDS) may mimic cellulitis and various panniculitides. Methods: To better characterize the histopathologic changes of LDS, we reviewed the clinicopathologic findings of 26 cases with a pathologic diagnosis consistent with LDS. A final diagnosis of LDS was made in 17 cases based on the clinicopathological correlation. As some cases manifested erythema nodosum (EN)-like lesions, 14 specimens of EN were reviewed to identify features for differential diagnosis. Results: Microscopically, the acute LDS lesions were characterized by patchy hemorrhage, ischemic fat necrosis with lipophages or hyalinization in the fat lobules. As the disease progressed to the subacute and chronic stages, lipomembranous or membranocystic fat necrosis, septal fibrosis and background venous stasis in the dermis became more pronounced. In contrast, EN typically displayed minimal venous stasis and membranocystic fat necrosis. Conclusions: LDS may manifest as EN-like lesions. Therefore LDS should be included in the differential diagnosis of EN. Clinicopathologic correlation is essential for diagnosis. Differentiating the acute LDS from the early EN is more difficult. A constellation of the findings of septal/lobular panniculitis, hemorrhage in the subcutaneous tissue, and lipophages and/or ischemic fat necrosis in the fat lobules favors the diagnosis of acute LDS.     

An Unusual Case of Cutaneous Pancreatic Fat Necrosis

BACKGROUND: Cutaneous pancreatic fat necrosis is a pathognomonic sign for pancreatic disease and usually presents as subcutaneous nodules in the pretibial region. OBJECTIVE: A case of cutaneous pancreatic fat necrosis is presented in which the clinical presentation of diffuse erythema was unusual. This disease is discussed and its possible etiologies are reviewed. METHODS: A MEDLINE search for cases of cutaneous pancreatic fat necrosis presenting as diffuse erythema without nodules was conducted. RESULTS: Diffuse erythema is an unusual presentation of cutaneous pancreatic fat necrosis. CONCLUSION: This may be the first case of cutaneous pancreatic fat necrosis presenting as diffuse erythema.     

先天性色素痣伴增生性结节10例临床病理分析

【摘要】 目的 探讨先天性色素痣伴增生性结节的临床特点及组织病理特征。方法 收集第四军医大学西京皮肤医院2015—2019年经临床和病理确诊的10例先天性色素痣伴增生性结节患者的临床及病理资料，并进行回顾性分析。结果 10例患者年龄2～45岁（平均15岁），9例增生性结节发生于婴儿，1例发生于成人。皮损位于四肢4例，头面部3例，躯干2例，躯干及四肢同时受累1例。临床表现为黑色斑片或斑块中出现1个或多个结节，6例增生性结节为多发，4例为单发，单个结节直径0.2～1.5 cm，仅1例出现溃疡。组织病理检查显示增生性结节内黑素细胞均存在成熟现象，核分裂象少，细胞无明显异型性，无坏死现象，免疫组化检查显示痣细胞弥漫表达Melan-A，不表达或仅部分表达HMB45，Ki67增殖指数 < 5%。结论 先天性色素痣伴增生性结节可发生于四肢、头面部及躯干；临床表现为原先天性色素痣皮损上的单发或多发结节；病理上增生性结节内黑素细胞可见成熟现象，免疫组化HMB45及Ki67染色有助于诊断，其预后有待长期随访。     

Neutrophilic lobular (pustular) panniculitis associated with rheumatoid arthritis: a case report and review of the literature.

Rheumatoid nodules, which affect the subcutis around joints, are the most frequent specific cutaneous lesions of rheumatoid arthritis (RA). Panniculitis is a rarely reported and nonspecific complication of RA. We report a 42-year-old woman with seropositive RA who presented with a 2-month history of lower leg panniculitis. Biopsy of a leg nodule showed a lobular neutrophilic infiltrate with lipophages and central basophilic necrosis. In addition, focal changes of lipomembranous fat necrosis indicative of ischemic damage were identified at the margins of the lobular infiltrate. Neutrophilic lobular panniculitis is commonly detected in panniculitis secondary to bacterial infections, pancreatitis, and factitial causes. However, this pattern of panniculitis has also been reported in some cases of erythema nodosum-like lesions found in Beh?et disease or bowel bypass syndrome and in rare cases of seropositive RA. These reported histologic findings fall into the spectrum of neutrophilic vascular reactions described by Jorizzo and Daniels for RA-associated dermatoses. In view of these findings. RA and related neutrophilic dermatoses (e.g., Beh?et disease) should be included in the differential diagnosis of neutrophilic lobular panniculitis.     

Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature

We present a remarkable case of subcutaneous panniculitic T-cell lymphoma (SPTL) that eluded diagnosis for 14 years and illustrates the importance of continued follow-up with repeat biopsy when SPTL is suspected. This case is unusual in that multiple biopsies demonstrated either a nonspecific panniculitis or lipomembranous panniculitis with calcified lipomembranes. A clinicopathologic review of 72 cases of SPTL from the English language literature is also presented, and approaches to diagnosis and treatment are reviewed.     

Nodular cystic fat necrosis in a patient with erythema nodosum

Nodular cystic fat necrosis shows a distinctive spectrum of clinical and histological features. Most of the lesions arc mobile subcutaneous nodules in regions vulnerable to trauma, such as the elbows, knees, and shins. There have been no reports of an association with other conditions. The histology is characterized by encapsulated fat necrosis in which multiple, non-viable adipocytes are surrounded by condensed fibrous tissue. We report a case of early stage nodular cystic fat necrosis associated with erythema nodosum.     

Granuloma with lymphocytic hyperplasia following vaccination: 10 cases. Presence of aluminium in the biopsies

BACKGROUND: Few cases of cutaneous lymphocytic hyperplasia secondary to vaccination have been published, although such lesions are not rare.PATIENTS AND METHODS: We report a series of 10 cases registered between 1993 and 2003.RESULTS: Mean age was 25. The clinical aspect was solitary or multiple subcutaneous nodules, located on the arm, developing after a delay of 1 to 18 months after vaccination. Histologic examination showed a lymphocytic infiltration of the subcutaneous fat, with diffuse and/or follicular pattern, without nuclear atypia, the morphological and immunohistochemical analysis of which revealed the benign nature. In all cases, there was fibrosis and granuloma composed of lymphocytes, plasma cells, eosinophils and macrophages with basophilic cytoplasm. Morin stain showed intralesional aluminium in the 6 investigated cases. Evolution was always benign, with no relapse following exeresis.DISCUSSION: Cutaneous lymphocytic hyperplasia secondary to vaccination has to be suspected in a young patient with subcutaneous nodules appearing at a vaccination site. Evidence of aluminium in the lesions supports the diagnosis and the hypothesis that aluminium in the vaccine excipient might have a role in the onset of such lesions.     

Membranous lipodystrophy-like changes in two cases of traumatic lipogranuloma caused by safety belts

A few cases of subcutaneous nodules with the histological findings of membranous lipodystrophy-like changes have been reported in the literature. However, reports of membranous lipodystrophy-like changes caused by trauma are exceedingly rare. We report two cases of traumatic lipogranuloma that had linear and multiple lesions showing membranous lipodystrophy-like changes and were induced by safety belt trauma after a car accident. These patients showed peculiar changes in their subcutaneous fat and had linear patterned, multiple, nodular lesions with membranous lipodystrophy-like changes caused by trauma.     

Subcutaneous trichoblastoma

We have recently observed three examples of solitary trichoblastomas (TB) with unusual histopathologic features characterized mainly by numerous aggregations of basaloid cells limited to the subcutis. The three trichoblastomas with unusual features were identified from a large series of 38 solitary TB cases collected over a period of 20 years. Clinically, all three neoplasms presented in men (49, 52, and 62 years old) as solitary, 1- to 1.5-cm skin-colored nodules situated on the scalp, face, and lower arm, respectively. Histopathologically, they showed numerous, smooth-bordered aggregations of basaloid cells limited to the subcutis and surrounded by a sclerotic and partly hyalinized stroma. Multiple sections revealed no connections of basaloid aggregations to the overlying epidermis or pre-existing follicular structures. All three cases displayed rather unusual morphologic growth patterns, including areas of variously sized, nodular aggregations of basaloid cells and extensive foci of elongated, thin columns and branching cords of basaloid cells. A striking feature in the stromal component in two cases was the presence of large, prominent areas of hyalinization and sclerosis. Characteristically, all three neoplasms showed numerous foci with rudimentary follicular germs and papillae. Cytomorphologically, the basaloid cells exhibited dark staining nuclei with large prominent nucleoli and scanty, pale or eosinophilic cytoplasm. Variable number of mitotic figures (2-4 mitoses per high-power field) and single necrotic cells were noted. In one case, small, foci of necrosis en masse were observed. Follow-up data after total excision in all three cases (80, 69, and 6 months) revealed no local recurrences. In light of our observations, we suggest that subcutaneous TB represents a rare variant of solitary TB. Besides the exclusive subcutaneous location, this neoplasm also displays a constellation of particular histopathologic features, namely, rather complex epithelial growth patterns and stroma with prominent foci of sclerosis and hyalinization.     

Fibrous hamartoma of infancy manifesting as multiple nodules with hypertrichosis

Fibrous hamartoma of infancy is a rare benign tumor, which usually occurs within the first year of life. Most cases present as a solitary, painless, skin-colored subcutaneous nodule; multiple lesions are rarely reported. There have also been a few cases which have showed overlying skin changes, including hypertrichosis and alterations in pigmentation. We report a rare case of fibrous hamartoma of infancy in a 6-month-old female who presented as multiple nodules with overlying hypertrichosis on the left buttock. To the best of our knowledge, there have been only two reported cases in the dermatological published work that showed these unusual findings simultaneously.     

表现为红斑和皮下结节的结节病一例

报告1例同时表现为红斑和皮下结节的结节病。患者女,55岁。左眉暗红斑,颞部多发皮下结节2个月余。皮肤科检查:左侧眉弓直径约1 cm暗红斑,颞部多发皮下结节,直径0.5~1.5 cm,质硬,边缘不规则。皮损组织病理示:真皮或皮下脂肪层大量的上皮样细胞肉芽肿及多核巨细胞浸润,大部分呈裸结节。诊断:结节病。     

Lupus erythematosus panniculitis with morphea-like lesions

A 22-year-old female with morphea-like lesions, deep subcutaneous nodules and lipoatrophic areas of the skin on lateral aspects of the upper arms, on the breasts and on the buttocks is described. In 1990 a biopsy specimen obtained from a subcutaneous nodule showed hyaline necrosis of fat tissue; there were no epidermal changes. Direct immunofluorescene revealed granular deposits of IgM at the dermo-epidermal junction of the skin overlying the subcutaneous nodule. In a biopsy specimen taken at the onset of the disease in 1988, hyaline sclerosis of the deep dermis, follicular hyperkeratosis and vacuolar degeneration in the epidermis were described. The was weak positivity for antinuclear antibodies. The diagnosis of lupus erythematosus panniculitis (LEP) was made. Administration of Chloroquine resulted in complete clearing of nodules in 3 months. The reported case demonstrates the difficulties in establishing the diagnosis of LEP in patients who present with subcutaneous disease, morphea-like lesions and who do not have other clinical or laboratory evidence of lupus erythematosus. The differential diagnosis of LEP and deep morphea is discussed.     

Unusual Presentation of Subcutaneous Phaeohyphomycosis by Alternaria alternate

The Alternaria species are dematiaceous fungi. Human infection due to dematiaceous fungi is uncommon. Most reported cases of alternariosis have occurred in patients with immunodeficiency. The majority of cases were solid-organ transplantation recipients. Cutaneous alternariosis lesions are usually asymptomatic solitary nodules, plaques of ulcers or subcutaneous cysts. Here we report a case of a 77-year-old female who presented with hemorrhagic skin necrosis in right arm that had developed from hemorrhagic bullae. Her prior medical history included iatrogenic Cushing''s syndrome, hypertension, interstitial lung disease and congestive heart failure. Following administration of itraconazole, her lesions improved.