Mycobacterial spindle cell pseudotumor of the skin

BACKGROUND: Mycobacterial spindle cell pseudotumor is a rare tumor-like lesion characterized by the proliferation of spindle cells engorged with mycobacterial microorganisms. To our knowledge, only a few cases of cutaneous lesions have been described in the literature. CASE REPORT: A 58-year-old Japanese woman presented with a nodule in her left forearm. She had systemic lupus erythematosus and interstitial pneumonia for 17 years, treated with prednisolone and azathioprine. She was also receiving insulin treatment for diabetes mellitus. RESULTS: Histologically, the lesion was located in the deep dermis with extension into the subcutaneous fat tissue and was composed mainly of spindle cells focally showing a vaguely storiform pattern. In limited focal areas, foam cells, epithelioid histiocytes, and multinucleated giant cells were seen sparsely. Ziehl-Neelsen staining showed numerous acid-fast bacilli within the spindle cells and epithelioid histiocytes. The acid-fast bacilli were determined by culture and polymerase chain reaction to be Mycobacterium intracellulare. CONCLUSIONS: We emphasize that mycobacterial spindle cell pseudotumor should be included in the differential diagnoses of a spindle cell lesion in the skin, especially in immunosuppressed patients.     

A Case of Cutaneous Mycobacterium Chelonae Abscessus Infection in a Renal Transplant Patient

Abstract Background: Mycobacterium chelonae is an atypical "fast-growing Mycobacteria" that is a rare cause of human infection. There have been several reports of cutaneous infection among immunosuppressed patients, as well as in immunocompetent individuals following trauma. Most cases to date seem to have occurred among renal transplant recipients, raising the possibility that there is something inherent to the renal transplant patient that increases their susceptibility more than other immunocompromised patients. Objective: The differential diagnosis of subcutaneous nodules distributed in a sporotrichoid pattern is extensive, particularly in an immunocompromised host. Although several cases of cutaneous M. chelonae abscessus infection have been reported among both immunosuppressed and immunocompetent patients, the clinical presentation has varied, and few cases have reported the appearance of lesions in a sporotrichoid pattern. We present a case of a renal transplant patient with a reported history of trauma to the lower extremities, who presents with subcutaneous nodules distributed in a sporotrichoid pattern. The patient is found to have M. chelonae abscessus infection, fails several treatment regimens, and presents with a recurrence. The literature of M. chelonae infection is reviewed, and the various treatment options are discussed. Methods: An initial skin biopsy was stained with Hematoxylin and Eosin and revealed deep dermal abscesses with acid-fast bacilli in clusters. The culture became positive for Mycobacterium chelonae abscesses in four days and was found to be sensitive to multiple antibiotics. The patient underwent surgical excision of 14 nodules, which revealed findings consistent with the skin biopsy, and was subsequently treated with the appropriate antibiotics. Results: Despite treatment with a full course of an organism-sensitive antibiotic regimen, the patient returned with persistent and recurrent nodules six weeks later. The patient was then treated as an inpatient with a seven-week course of intravenous antibiotics and was discharged home on a combined intravenous and oral regimen. Conclusion: Although M. chelonae abscessus is an extremely rare cause of infection among humans, there seems to be a predominance of cases reported among renal transplant patients. The explanation for this is not entirely clear; however, the organism must be considered as a cause of infection in any renal transplant recipient who presents with subcutaneous nodules. Eradication of the organism presents a tremendous challenge to the clinician, and, as presented here, even with appropriate antibiotics, there is a high rate of recurrence.     

Nontuberculous mycobacteria infection after mesotherapy: preliminary report of 15 cases

BACKGROUND: Mesotherapy is an increasingly used technique which is currently causing several mycobacterial infections owing to contaminated substances being injected, and also to poor aseptic measures being held by nonprofessional practitioners. PATIENTS AND METHODS: We collected 15 cases of nontuberculous mycobacteria (NTM) infection after mesotherapy in a 6-month period. RESULTS: All patients were female with ages ranging from 19 to 52 years; the main substances injected were procaine and lecithin, and the time between mesotherapy and the appearance of the lesions varied between 1 and 12 weeks. Clinical lesions were mostly nodules and abscesses, which were localized in the abdomen and buttocks in the majority of cases. The main patient complaint was local pain but some presented with systemic symptoms such as fever and malaise. Biopsies reported granulomatous chronic inflammation in the majority of cases. Skin cultures were positive for NTM and Mycobacterium chelonae. DISCUSSION AND CONCLUSIONS: Mesotherapy not performed with quality controlled substances can be a predisposing factor for NTM infection.     

Multiple squamous cell carcinomas in the setting of psoriasis treated with etanercept: a report of four cases and review of the literature

Background  Psoriasis is a common, chronic, hyperproliferative disease of the skin characterized by overexpression of type 1 cytokines, including tumor necrosis factor α. There is concern that antitumor necrosis factor agents such as etanercept may increase the incidence of cutaneous malignancies; however, the data are conflicting. Our objective was to further understand the characteristics and association of squamous cell carcinoma (SCC) development in patients with psoriasis who used etanercept. Methods  Four patients with psoriasis were identified as having SCCs in the setting of etanercept. The histories of these patients were reviewed retrospectively. Results  All four patients had lifelong psoriasis. The mean time of SCC onset was 11 months after etanercept therapy was begun (range, 1–17 months), and the number of SCCs in each patient ranged from five to more than 50. Conclusions  Currently, reports are conflicting about the effect of etanercept on SCC development. We present the first case series of patients in whom SCC developed in the setting of etanercept therapy. More research is needed to better characterize the effects of etanercept on the development and behavior of SCC in patients with psoriasis.     

Characteristic appearance of spindle cell hemangiomatosis,often misdiagnosed as venous malformation: A retrospective study of 11 cases

Spindle cell hemangiomatosis is a benign multifocal vascular proliferation that mostly occurs in the distal extremities. It is a relatively rare disease and causes difficulties in clinical diagnosis and differential diagnosis of venous malformation. We retrospectively assessed the medical history, and clinical features, imaging features, pathological features and follow up of 11 patients diagnosed pathologically with spindle cell hemangiomatosis after surgery. There are two types of clinical appearances in spindle cell hemangiomatosis in the distal extremities: bleb-like nodules and varix-like nodules. Bleb-like nodules are mostly superficially located in the palm or interphalangeal joints with obvious hemorrhage in lesions and mainly composed of cavernous spaces rather than spindle cells. Varix-like nodules are located in the back of the hands or arms with normal skin color and mainly composed of solid areas of accumulated spindle cells. Surgery is the standard therapy for spindle cell hemangiomatosis, while sclerotherapy is invalid. Despite the tendency to develop new lesions, there are no residual lesions at the surgical site during follow up. Bleb-like nodules in the palm and interphalangeal joints are a typical clinical appearance in spindle cell hemangiomatosis which can help make clinical and a differential diagnosis of venous malformation. Surgery is the standard therapy for spindle cell hemangiomatosis with no residual lesions remaining at the surgical sites.     

Sweet's syndrome associated with non-tuberculous mycobacterial infection: a report of five cases

We report the rare association of Sweet's syndrome with non-tuberculous mycobacteria in five patients (three women, two men, aged 25–41 years). Clinical and histological evidence supported the diagnosis of Sweet's syndrome in all patients. The skin lesions responded well to systemic corticosteroid but recurred in two cases. All of our patients had chronic disseminated non-tuberculous mycobacterial infection. They initially presented with lymphadenopathy and developed involvement in other organs later. All of them were treated as having tuberculous lymphadenitis based on pathological findings before definite diagnosis was made by culture. The organisms isolated were Mycobacterium chelonae in three cases, M. scrofulaceum in one case and M. avium intracellulare complex in one case. All the patients gradually improved with treatment but one had multiple recurrences. The search for an infectious agent, especially non-tuberculous mycobacteria, should be performed in cases of Sweet's syndrome that appear in association with chronic granulomatous lymphadenitis which is recalcitrant to antituberculous drugs.     

The fish tank strikes again: Metachronous nontuberculous mycobacterial skin infection in an immunosuppressed host

An 82‐year‐old woman on long‐term prednisolone for chronic obstructive airways disease presented with a 2‐month history of nodules on her left forearm. This occurred 10 years after nodules on her right forearm caused by a culture‐proven Mycobacterium marinum infection. Histopathological examination, polymerase chain reaction and culture of biopsy specimens were positive for M. chelonae. To our knowledge this is the first case of metachronous nontuberculous mycobacterial skin infection reported, and it highlights the diagnostic and therapeutic challenges of such infections.     

Malignant fibrous histiocytoma of the skin with marked inflammatory infiltrate: a sarcoma mimicking malignant lymphoma

Three cases of malignant fibrous histiocytoma of the skin with a marked inflammatory infiltrate in the stroma are reported. The inflammatory infiltrate, composed mainly of T-lymphocytes, obscured the nature of the neoplasms, and immunohistochemical studies were required to establish the diagnosis. Two tumors arose in the sun-damaged skin of the face, and one tumor arose in the chest wall. One patient developed a local recurrence with histopathologic findings similar to those observed in the original lesion, including the inflammatory infiltrate. Possible differential diagnoses include large cell lymphoma, inflammatory pseudotumor, inflammatory leiomyosarcoma, and spindle cell squamous cell carcinoma. The presumed rarity of inflammatory changes in malignant fibrous histiocytoma of the skin is supported by the absence of reported cases.     

Spindle cell variant of epithelioid sarcoma: an easily misdiagnosed tumour

Epithelioid sarcoma is a histologically distinct soft tissue sarcoma of high grade malignancy. We report a case of epithelioid sarcoma in a young man who presented with multiple nodules over the left forearm, with bony invasion and pulmonary metastases. The histological features of the dermal tumour were those of a malignant spindle cell tumour with positive cytokeratin and vimentin staining and differed from the classical epithelioid sarcoma in its absence of typical necrobiotic nodular epithelioid pattern. It was the clinical presentation and the histology of the subcutaneous nodules that led to the final diagnosis of epithelioid sarcoma. This case illustrates a predominance of spindle cell pattern in the dermal tumour of epithelioid sarcoma, which has previously been reported as fibroma-like variant of epithelioid sarcoma.     

Diagnostic odyssey of a cutaneous mycobacteriosis rare in central Europe

Cutaneous infection with Mycobacterium chelonae is an uncommon disease, although this atypical mycobacterium is an acid-fast bacillus ubiquitous in the environment. It is often misdiagnosed and treated as a fungal or common bacterial infection. We report a case of disseminated atypical mycobacterial skin infection of a 72-year-old woman who was treated with different topical and systemic antimycotic and antibiotic drugs over a period of 5 months without remarkable improvement. Eventually, repeated tissue cultures on special medium and performance of PCR led to the diagnosis of M. chelonae infection. The patient was treated successfully with oral clarithromycin within 8 weeks. In case of abscessing cutaneous infection, M. chelonae should be considered in the differential diagnosis of prolonged disease when common antibiotics are not effective after 2-4 weeks of treatment.     

梭形细胞血管内皮瘤

报告1例梭形细胞血管内皮瘤.患者男,16岁.右跖部结节10余年,相继波及左足和双小腿下段,无自觉症状.皮损组织病理检查可见真皮中下层由海绵状血管腔隙和梭形细胞组成的团块.依据临床表现和组织病理学改变,诊断为梭形细胞血管内皮瘤.     

Spindle B‐cell lymphoma: a rare variant of follicle center lymphoma

Primary cutaneous follicle center lymphoma (PCFCL) is a common subtype of primary cutaneous B‐cell lymphoma (PCBCL). The prognosis of this subtype is favorable and recurrence is observed in up to 50% of patients. The dissemination to lymph nodes or internal organs is rare. In this study, two cases of rare variant of PCFCL are reported. Both cases presented with multiple erythematous nodules, plaques and some annular configurations. Histopathological examination revealed dermal lymphocytic infiltrates consisting of admixed centrocytes and centroblasts. Interestingly, spindle‐shaped cells with elongated nuclei, dispersed chromatin and scant cytoplasm were also detected. Immunohistochemical analysis revealed that all cells including the spindle cells were positive for CD20 and negative for CD3, CD43, CD10, CD34, CD68 and CD138. They were also negative for desmin and S‐100. They consistently expressed nuclear bcl‐6, but did not express bcl‐2. The histopathological and immunohistochemical examination suggest a rare case of primary cutaneous spindle cell B‐cell lymphoma (PCSBCL). Although few data is published about this rare subtype, PCSBCL is recently considered as a rare subtype of PCFCL. The prognosis and the nature of this peculiar subtype are not yet cleared. This indicates a great need for more investigations.     

Mycobacterium chelonae cutaneous infection in a patient with mixed connective tissue disease

Around 50 mycobacteria species cause human disease. Immunosuppressive states predispose to non-tuberculous mycobaterium infection, such as Mycobacterium chelonae: AFB, non-tuberculous, fast growth of low virulence and uncommon as a human pathogen. It may compromise the skin and soft tissues, lungs, lymph nodes and there is also a disseminated presentation. The diagnosis involves AFB identification and culture on Agar and Lowenstein-Jensen medium base. A 41-year-old female with MCTD (LES predominance) is reported, presenting painless nodules in the right forearm. She denied local trauma. Immunosuppressed with prednisone and cyclophosphamide for 24 months. Lesion biopsy has demonstrated positive bacilloscopy (Ziehl-Neelsen stain) and M.chelonae in culture (Lowenstein-Jensen medium base), therefore clarithromycin treatment has been started (best therapy choice in the literature).     

No evidence for increased risk of cutaneous squamous cell carcinoma in patients with rheumatoid arthritis receiving etanercept for up to 5 years

OBJECTIVE: To determine the incidence of cutaneous squamous cell carcinoma (SCC) in patients with rheumatoid arthritis receiving etanercept, a tumor necrosis factor antagonist, for up to 5 years. DESIGN: An etanercept clinical trials' database and an etanercept postmarketing surveillance database were retrospectively analyzed for the incidence of SCC. SETTING: Patients enrolled in clinical trials of etanercept were from private and institutional practices. The postmarketing database comprised reports from postmarketing trials and solicited and spontaneous reports.Patients A total of 1442 patients with rheumatoid arthritis with 4257 patient-years of etanercept exposure (median exposure, 3.7 years) are included in the clinical trials' database. More than 125,000 patients with more than 250,000 patient-years of etanercept exposure are included in the etanercept postmarketing database.Interventions Most patients enrolled in clinical trials of etanercept received a dosage of 25 mg of etanercept subcutaneously twice weekly for most of the time they received etanercept therapy. RESULTS: Only 4 cases of SCC were observed in the etanercept clinical trials' database, an incidence that compares favorably with the expected incidences based on general population data from Arizona (13.1) and Minnesota (5.9). Similarly, few cases of SCC (1 per 10,000 patient-years) have been reported during postmarketing surveillance of etanercept therapy. CONCLUSION: In patients with rheumatoid arthritis, etanercept use of up to 5 years does not seem to be associated with an increase in the incidence of cutaneous SCC.     

鱼刺伤肉芽肿四例分析

报道4例鱼刺伤所致肉芽肿,患者均为老年女性,均有鱼类接触史,皮疹特点均为暗红色丘疹、结节,其中一例伴有上肢串珠样皮下结节。组织病理示感染性肉芽肿。虽然微生物感染证据缺乏,但诊断考虑非结核分枝杆菌感染,予利福平、克拉霉素行经验性治疗有效。     

Patients with moderate-to-severe psoriasis recapture clinical response during re-treatment with etanercept

Background  Patients with psoriasis experience remission and gradual reappearance of erythematous and scaly plaques and require individualized treatment over time. A goal of psoriasis treatment is to provide optimal efficacy with a flexible therapeutic regimen that may include treatment pauses.
Objectives  To determine whether patients receiving initial treatment with etanercept who then pause therapy would subsequently recapture response during re-treatment.
Patients and methods  A post-hoc analysis of 226 patients with moderate-to-severe psoriasis from a large multicentre trial was performed. Patients had received etanercept 50 mg twice weekly subcutaneously until a target clinical response had been achieved, then had paused treatment and eventually relapsed. They were then re-treated with etanercept 25 mg twice weekly. The number of patients recapturing a Physician Global Assessment (PGA) of psoriasis rating of ≤ 2 (clear, almost clear or mild) on first re-treatment was assessed. Patient satisfaction during the initial treatment and first re-treatment period was also determined.
Results  A total of 187 (83%) patients recaptured the target clinical response of a PGA of ≤ 2 after re-treatment. The majority of patients [219 of 226 (97%)] reported satisfaction with etanercept re-treatment. No new safety concerns emerged during re-treatment.
Conclusions  In this post-hoc analysis, patients with psoriasis who were re-treated with etanercept 25 mg twice weekly effectively recaptured clinical responses that patients found satisfactory. A flexible treatment option is available to dermatologists and patients for individualized care.     

Multiple spindle cell lipomas and dermatofibrosarcoma protuberans within a single patient: evidence for a common neoplastic process of interstitial dendritic cells?

This case report describes a 48-year-old man with multiple spindle cell lipomas of the neck and a dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation of the chest. The presence of familial and nonfamilial multiple spindle cell lipomas within a single patient is a rare event, with only two reports in the current literature. This case represents the first report of multiple spindle cell lipomas occurring in association with a DFSP. It is of particular interest in that both spindle cell lipoma and DFSP represent, at least in part, neoplastic proliferations of CD34(+) spindled cells. The exact nature and differentiation of these spindled cells remains controversial, but prior studies have suggested that they could represent neoplastic interstitial dendritic cells. The association of DFSP and spindle cell lipoma within this single patient suggests that these two tumors (and their histologic variants) may well be linked, conceptually, as neoplastic proliferations of CD34(+) interstitial dendritic cells.     

Infección cutánea por Mycobacterium chelonae. Revisión de seis casos

—Mycobacterium chelonae (M. chelonae) is a rapid-growth atypical mycobacteria, which belongs to Runyon group IV. Infection often develops either in patients with immunosuppresion or in immunocompetent hosts associated with penetrating trauma or injury.We present six female immunocompetent patients, who presented nodules, plaques or ulcers, with abscess formation and clear fluid drainage that followed a protracted course in spite of cloxaciline therapy. Although one patient referred a previous history of liposuction, the others did not recall an inoculation size. Treatment of M. chelonae infection required oral clarithromicina for at least 3 months, in addition to other drugs. The histopathologic analysis showed an inflammatory infiltrate, and abscess and granuloma formation. M. chelonae was cultured from skin biopsy specimens.Although M. chelonae infection usually occurs in immunologically compromised patients or may follow puncture wound or trauma, our series shows that chronic suppurative lesions in otherwise healthy women with no previous trauma might be due to M. chelonae.     

A range of histologic findings in infantile digital fibromatosis

Abstract:  Infantile digital fibromatosis is a benign proliferation of myofibroblasts with characteristic eosinophilic intracytoplasmic inclusion bodies. Clinically, patients present in the first several years of life with asymptomatic, smooth, flesh-colored nodules located on the dorsal or lateral aspect of the fingers or toes. The lesions have a tendency for spontaneous regression and recur over 60% of the time following excision. Observation is often recommended, while surgery is reserved for cases of rapid growth or functional impairment. We report three patients, ranging in age from 8 months to 8 years, with infantile digital fibromatosis. All three patients presented with nodules on the hands or feet and all shared histologic findings of spindle cells with characteristic inclusion bodies. Pathologic features ranged from a haphazard arrangement of spindle cells with few inclusion bodies and numerous lymphocytes to a well-organized arrangement of spindle cells with abundant inclusion bodies and rare lymphocytes. All three patients underwent excision for diagnosis or treatment of their tumors with two of the patients experiencing a recurrence.     

Scrofuloderma and Sweet's syndrome

BACKGROUND: In recent years, the rare association of Sweet's syndrome with nontuberculous mycobacterial lymphadenitis has been reported. OBJECTIVE: To report the clinical, demographic, and bacteriologic data and association with Sweet's syndrome of 18 patients with scrofuloderma and scrofuloderma-like condition caused by nontuberculous mycobacterial infections seen during the past 7 years (1994-2000). METHODS: In all patients, a biopsy specimen was obtained for histopathologic and microbiologic studies. Patients from whom Mycobacterium tuberculosis and nontuberculous mycobacteria were isolated from the culture of skin biopsy specimens were included. Deep fungal infection was excluded by the lack of a fungal element in histologic section and cultural methods. The patients were treated with antimicrobials or antituberculous drugs according to the causative species. RESULTS: Eighteen cases of scrofuloderma (nine male, nine female; mean age, 36.9 years) were found among 104 patients with cutaneous tuberculosis and nontuberculous mycobacterial cutaneous infections. Sixteen of the 18 cases had lymphadenitis as the underlying focus of scrofuloderma: 15 cases occurred in the cervical group and one case in the inguinal area. One case drained from the soft tissue and one from the paranasal air sinus. Five cases had multiple episodes of Sweet' s syndrome during the course of treatment. Most cases in this group (four of the five) were middle-aged women with cervical lymphadenitis, and the most common species were rapid growers. CONCLUSIONS: Age, sex, and the site of infection may have some influence on the association with Sweet's syndrome in nontuberculous mycobacterial infections.