Sarcoidosis with subcutaneous lesions

A 30-year-old man presented for evaluation of multiple, hypopigmented macules and erythematous papules on his upper and lower lips and upper extremities. He subsequently developed firm, non-tender, subcutaneous, mobile nodules on the left forearm, left calf, and right thigh. Clinical and histopathologic features were consistent with cutaneous and subcutaneous sarcoidosis. Systemic evaluation showed no evidence of extracutaneous involvement. Subcutaneous sarcoidosis is a rare form of nodular cutaneous sarcoidosis. Typical skin findings are painless, firm, mobile nodules without overlying epidermal involvement and with a predilection for the trunk and extremities. Histopathologic alterations include epilthelioid cell tubercles in the subcutaneous fat. As this is a diagnosis of exclusion, other etiologies of granulomatous inflammation must be ruled out with tests and special stains. The mainstay of treatment is with oral glucocorticoids.     

瘢痕性结节病国内首报

患者女,54岁。５年前出现全身乏力,双膝关节肿痛,体质量下降。就诊半年前于左眉外侧陈旧瘢痕处出现一硬结。皮疹先后累及躯干和四肢,特别是手部原有陈旧瘢痕处均出现红斑,结节。体检：双腋下淋巴结肿大。左眉外侧一斑块,位于原有瘢痕处。右上臂伸侧可及一皮下肿块,左手瘢痕处和双足底可见多个暗红色结节。左侧前臂及躯干少许散在的红色小丘疹。血清血管紧张素转化酶82 kU/L。CT示两肺散在多发模糊小结节灶,纵隔及腋下多发肿大淋巴结影,双侧肺门淋巴结未肿大。组织病理符合结节病。予甲泼尼龙口服后皮损明显好转。本例皮损同时具有瘢痕、结节、斑块、皮下肿块和丘疹,较为少见。对原有的陈旧性瘢痕出现活动,表现为红肿、结节,是诊断结节病的重要线索。     

Ossifying plexiform tumor

We report a rare case of ossifying plexiform tumor in a 64‐year‐old female. The patient had a 2‐year history of gradual hardening of the right thumb pad and pain that radiated up the forearm. Physical examination showed a tender, mobile 2‐cm subcutaneous nodule distending the tip of the right thumb. The biopsy specimen showed a well‐delineated tumor with multiple lobules of epithelioid and spindled cells arranged in a plexiform pattern separated by fibrous bands and having foci of bone formation. The neoplastic cells had scant‐to‐moderate amphophilic cytoplasm with mild nuclear pleomorphism in a myxocollagenous background. No necrosis, mitoses or cytological atypicia were seen. The osteocytes present in the bone islands were bland, with occasional rimming osteoblasts. X‐ray showed stippled calcification in the soft tissue of the distal thumb without involvement of the phalanx. The patient is tumor free for 1 year after complete local excision. Only three cases of ossifying plexiform tumor have been reported. All previous cases and the current case presented as subcutaneous nodules on hand digits of females, measuring 1–2 cm in greatest dimension. Ossifying plexiform tumor appears to be a benign neoplasm with no reports of progression or metastasis.     

Etanercept-induced cutaneous and pulmonary sarcoid-like granulomas resolving with adalimumab

A 59-year-old female with rheumatoid arthritis on etanercept therapy presented with a 7-cm-large subcutaneous forearm mass. Multiple smaller nodules subsequently developed on the upper and lower extremities. Except for a new cough, the patient was systemically well. Biopsy of the mass showed sarcoidal type granulomatous inflammation with nodular aggregations of non-necrotizing epithelioid histiocytes in the subcutis. A chest computed tomography (CT) scan showed mediastinal adenopathy consistent with pulmonary sarcoidosis. Etanercept was discontinued, and the patient was started on adalimumab for rheumatoid arthritis control. The cutaneous nodules fully resolved in 6 months with no additional treatment. A 4-month follow-up CT scan showed significant regression of mediastinal adenopathy. The patient has since been maintained on adalimumab therapy for 2 years with no recurrence of sarcoid-like manifestations. Biologic response modifiers targeting tumor necrosis factor alpha (TNFα) are effective treatments of chronic inflammatory conditions such as rheumatoid arthritis and psoriasis. TNFα represents a major cytokine in granuloma formation, and TNFα inhibitors are sometimes efficacious in the treatment of sarcoidosis. Paradoxically, there is a small volume of literature implicating TNFα inhibitors in the development of sarcoid-like disease. We present this case to promote the recognition of TNFα inhibitor-induced sarcoidosis and to illustrate the wide clinicopathologic differential of sarcoidal type granulomas.     

Epithelioid sarcoma of the right hand

A 35-year-old man presented with swelling, indurations and nodules on the thumb, wrist and fingers of the right hand. History revealed that the findings were slowly progressive and had been present for at least eight years. Histopathologic analysis of a nodule showed a diffuse infiltrate with atypical spindle-shaped cells and expression of cytokeratin, epithelial membrane antigen (EMA) and CD34; the diagnosis of epithelioid sarcoma (ES) was made. Because of diffuse extension of the tumor, forearm amputation was performed along with axillary dissection and local radiotherapy because of axillary lymph node metastases. ES is a rare subtype of soft tissue sarcoma with a harmless appearance and indolent course over years. ES represents a diagnostic challenge, with consequent delay in diagnosis and adequate treatment. The most important measure in the treatment of ES is early surgical excision with adjuvant radiotherapy if local metastases are present.     

卡波西样血管内皮细胞瘤1例

报告1例卡波西样血管内皮细胞瘤。患儿男,13个月。右下腹红斑,硬结伴疼痛9个月,皮诊进行性扩大。皮肤科检查见右下腹大片浸润壮斑块。组织病理检查示真皮及皮下组织内长梭形细胞及椭圆形细胞聚集成团块,伴管腔形成,免疫组化示CD31及CD34阳性,诊断为卡波西样血管内皮细胞瘤。     

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。     

Subcutaneous sarcoidosis as the first manifestation of systemic disease

There are dermatological symptoms in up to 25% of patients with sarcoidosis, and the appearance of specific subcutaneous nodules as a manifestation of this entity is rare. They may even predate other manifestations of sarcoidosis. We present the case of a 38-year-old woman with asymptomatic subcutaneous nodules in the limbs, which corresponded to deep sarcoid granulomas in the histological study. She did not present with any extracutaneous indications. The imaging tests performed revealed right paratracheal adenopathies. This led to the diagnosis of sarcoidosis, in both its subcutaneous and pulmonary forms (stage I). Subcutaneous sarcoidosis is probably an underdiagnosed entity, as fewer than 40 cases are reflected in literature. Its value lies in the fact that it may be the first manifestation of extracutaneous or systemic sarcoidosis, which means that this form of sarcoidosis must be considered in the differential diagnosis of subcutaneous nodular lesions; close follow-up of these patients is also necessary.     

皮下型结节病一例并文献复习

患者,女,51岁。左膝肿块半年,右膝皮下结节1月余。双膝部皮损组织病理：真皮深部至皮下可见大的由组织细胞、上皮样细胞形成的结节,境界清楚,无干酪样坏死。网状纤维染色：（+）,结节周围有网状纤维增生。诊断：结节病（皮下型）。予羟氯喹联合复方甘草酸苷治疗1个月后,结节明显消退。     

Unusual clinical features of cutaneous tuberculosis in an immune compromised patient

We report a case of an unusual form of cutaneous tuberculosis in an 82-year-old woman. She visited our hospital because of an intractable ulcer on the fifth finger of her right hand. While examining the ulcer surrounding half of her right fifth finger and covered with necrotic tissue, we also perceived a nodule with crust on the forearm, multiple subcutaneous nodules on the right forearm and upper arm, and a hen's-egg-sized agglomerative nodule on the axilla. All the lesions were located on her right arm. Skin biopsy specimens showed granulomatous tissue with necrosis in the lesions. Mycobacterium tuberculosis was identified by culture of a biopsied specimen, so the diagnosis was confirmed. Further examination revealed that she also had pulmonary tuberculosis. Cutaneous tuberculoses are classified morphologically with reference to host immune status, but no satisfactory classification exists. The present case can't be classified into any of the types which have been proposed so far. She is elderly and suffers from liver cirrhosis, hepatocellular carcinoma and myelodysplastic syndrome. The resulting acquired immunosuppression may have caused a unique form of cutaneous tuberculosis.     

Unusual cutaneous dissemination of a tropical fish tank granuloma

A patient with an unusual disseminated tropical fish tank granuloma is presented. The infection manifested first as red, subcutaneous nodules of the right hand and forearm. During subsequent days and weeks, nodules appeared on the left arm, on the ventral and dorsal thorax, on one leg, and the face. The diagnosis was based on, in addition to the history and the clinical aspects, a positive reaction to intracutaneous testing with a specific Mycobacterium marinum antigen and on the histologic examination of biopsy specimens. The larger nodules were treated with excision. Smaller nodules disappeared spontaneously after the larger ones had been surgically removed.     

Subcutaneous sarcoidosis--clinicopathological study of 10 cases

BACKGROUND: Subcutaneous sarcoidosis is a specific cutaneous lesion of sarcoidosis that is rarely reported. OBJECTIVE: Our purpose was to analyse the clinicopathological features of 10 patients with subcutaneous sarcoidosis and its relationship with the systemic features of the disease. PATIENTS AND METHODS: The patients with systemic sarcoidosis, diagnosed from 1974 to 2002 at a university hospital in Barcelona, Spain, who developed subcutaneous involvement, were included in the study. The diagnosis of systemic sarcoidosis was made according to conventional criteria. All the patients were monitored prospectively at the sarcoidosis clinic of the hospital. Skin biopsies were performed when granulomatous cutaneous involvement was suspected clinically. RESULTS: Granulomatous cutaneous involvement was demonstrated in 85 of 480 patients with systemic sarcoidosis. In 10 of these 85 patients subcutaneous sarcoidosis was diagnosed (11.8%). The lesions were most frequently located in the extremities, involving the forearms in nine patients. Indurated linear bands from the elbow to the hand were observed in five patients. In all of our patients the subcutaneous nodules appeared at the beginning of the disease. In six patients, the nodules remitted spontaneously in less than 2 years. In two cases foreign particles were detected under polarized light. CONCLUSIONS: Subcutaneous sarcoidosis is a quite uniform clinicopathological entity usually appearing at the beginning of the disease. It usually heralds forms of sarcoidosis with nonsevere systemic involvement and is not associated with chronic fibrotic disease.     

Sweet's syndrome associated with cellulitis - a challenging diagnosis

Sweet''s syndrome is a neutrophilic dermatosis with worldwide distribution that has been associated with inflammatory autoimmune diseases, infections, malignancies, drugs, and pregnancy. The disease is idiopathic in up to 50% of patients. A 64-year-old woman, diagnosed with right limb cellulitis (4 days of evolution), was seen at our department, due to persistent cellulitis and progressive appearance of painful nodules and plaques in both shins and the right forearm (2 days of evolution). Taken together, clinical, laboratory and pathological data suggested the diagnosis of Sweet''s syndrome, probably secondary to cellulitis of the right inferior limb. We suggest that cellulitis may be associated with Sweet''s syndrome, a rare association in the literature.     

Cutaneous manifestations in a patient with a long-term history of untreated ACTH-dependent Cushing's syndrome

Cushing's syndrome is accompanied by many different skin symptoms. A case of a 43-year-old female patient with unrecognized adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome is reported. Besides numerous skin lesions, the patient presented with a rapidly progressive leg ulcer due to venous thrombosis. The initial skin symptoms appeared on her lower leg one month prior to admission. The patient was treated with oral prednisone, however, rapid progression of the skin lesion was observed. On admission the patient presented with a large, very painful ulceration, partially covered with a crust, on the right lower leg with several satellite smaller ulcerations. The other leg showed no changes. Moreover, the patient had pronounced hirsutism and skin darkening on the face, and Muehrcke's lines were observed on all her nails. Microscopic findings of skin biopsy were uncharacteristic, with some signs of angiopathy and vasculitis. Based on the elevated serum levels of ACTH and cortisol, and the result of dexamethasone suppression test, ACTH-dependent Cushing syndrome was diagnosed. Sonography of leg veins revealed a recanalized thrombus of the right popliteal vein with reflux. The patient was transferred to surgical department where both suprarenal glands were removed. Upon suprarenal gland removal, significant improvement of the patient's general condition and rapid healing of the leg ulcer were observed. Briefly, this patient is presented to point out that a leg ulcer caused by venous insufficency may mimic fulminant purpura or some other type of vasculitis in patients with Cushing syndrome.     

Löfgren's syndrome presenting with erythema nodosum-like eruption

L?fgren's syndrome is an acute form of sarcoidosis that mostly presents with erythema nodosum, periarticular ankle inflammation, arthritis, conjunctivitis, and unilateral or bilateral hilar or right paratracheal lymphadenopathy. Erythema nodosum is the most common cutaneous lesion in acute sarcoidosis, but it is histologically nonspecific, as it does not reveal sarcoidal granulomas upon microscopic examination. We present a case of a 57-year-old woman with acute onset of tender erythematous nodules on the calves, which were clinically erythema nodosum-like lesions. Histologic analysis revealed sarcoidal granulomas located in the dermis and subcutaneous tissue. Upon further work-up, the patient was found to have both pulmonary and ocular sarcoidosis. Based on this case, we recommend that all patients with erythema nodosum-like lesions undergo biopsy and further work-up for sarcoidosis is recommended in case of biopsy proven sarcoidal granulomas.     

A case of Werner's syndrome associated with osteosarcoma.

We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.     

A case of tuberous sclerosis with subcutaneous nodules

Tuberous sclerosis (TS) is a well-known clinical disease, characterized by epilepsy, mental retardation, adenoma sebaceum of Pringle, and various congenital anomalies. There have been a few reports of subcutaneous nodules as a cutaneous manifestation of TS. A 9-year-old Japanese boy who was diagnosed as having typical TS had one subcutaneous nodule on the breast and three on the back. Histologic examination of the breast nodule showed proliferation of homogeneous collagenous bundles encapsulated by thin connective tissue.     

Annular lesions of cutaneous sarcoidosis with granulomatous vasculitis

Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62‐year‐old female was diagnosed with sarcoidosis by chest‐abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non‐caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis.     

Bilateral eyelid swelling as the presenting sign of subcutaneous sarcoidosis

An 18-year-old Black female presented with a 2-year history of bilateral upper eyelid swelling and the recent onset of multiple subcutaneous nodules on the arms. She had previously undergone evaluation and treatment for presumed angioedema. Biopsies of the eyelid and an arm nodule demonstrated non-necrotizing granulomatous inflammation with special stains negative for acid-fast bacilli and fungi, and the patient was diagnosed with subcutaneous sarcoidosis. The isolated finding of bilateral eyelid swelling 2 years prior to the onset of additional cutaneous findings led to a significant delay in diagnosis, highlighting the importance of considering sarcoidosis in the differential diagnosis for bilateral eyelid swelling.     

皮下血管内大B细胞淋巴瘤

报告1例皮下血管内大B细胞淋巴瘤.患者男,73岁.因双侧大腿皮下结节,于2007年2月初就诊.皮损组织病理检查见血管腔内有大量肿瘤细胞聚集,免疫组化染色结果示肿瘤细胞CD20阳性,诊断为皮下血管内大B细胞淋巴瘤(IVLBCL).予环磷酰胺、长春新碱、泼尼松(COP方案)化疗后皮损缩小.IVLBCL恶性程度较高,临床表现无特异性,预后较差,皮损组织病理检查有助于诊断.