scholarly journals Multiple Reticulohistiocytomas in an 88-year-old Man: a Case Report

2013 ◽  
Vol 5 (2) ◽  
pp. 87-93
Author(s):  
Branislava Gajić ◽  
Tatjana Roš ◽  
Andrijana Arapović ◽  
Nada Vučković ◽  
Aleksandra Fejsa Levakov
Keyword(s):  
Treatment Options ◽  
Skin Lesions ◽  
Systemic Involvement ◽  
Multicentric Reticulohistiocytosis ◽  
Mucosal Involvement ◽  
Multinuclear Cells ◽  
Systemic Symptoms ◽  
One Year ◽  
Foamy Cytoplasm

Abstract Solitary and multiple reticulohistiocytomas, often referred to as diffuse cutaneous reticulohistiocytosis, that are generally restricted to skin, must be differentiated from multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis is a rare histiocytic proliferative disease affecting joints, skin and mucous membranes, while arthropathy usually precedes skin and mucosal involvement. Other organs may also be involved, and 20% of patients have an associated internal malignancy. Multicentric reticulohistiocytosis has been reported to be associated with autoimmune diseases and malignancies. We present an 88-year-old man, with a sudden eruption of asymptomatic, firm, skin to brown colored papules and nodules, 6 to 17 mm in diameter, localized on forehead, arms, legs, and buttocks. There were no symptoms of joint or other organ involvement. Clinical and dermoscopic characteristics pointed to reticulohystiocytomas. Multiple excision biopsies of different tumors were performed and histopathology reports confirmed the diagnosis. In the case presented, reticulohistiocytosis was limited to the skin, exhibiting multiple reticlohistiocytomas with typical clinical, dermoscopic and rather peculiar histopathology presentation. Apart from this, some histologic features were seen in early lesions of multicentric reticulohistiocytosis, such as multinuclear cells dissociating collagen fibers with pale eosinophilic and foamy cytoplasm. Besides surgical excisions, no other treatment options were done. Laboratory and other tests showed no presence of extracutaneous illness, and no autoimmune or paraneoplastic processes. At one year follow up, the remaining tumors were of the same size, but there were no recurrences at excision sites, no signs of disease progression or systemic involvement. Since diffuse cutaneous reticulohistiocytosis without arthropathy as well as isolated reticulohistiocytomas have been described, in some cases of multiple reticulohistiocytomas even without systemic symptoms and signs, multicentric reticulohistiocytosis should be considered with an appropriate follow up. In such cases, skin lesions have the same histological features as lesions in multicentric reticulohistiocytosis, but they are not associated with joint problems or neoplasms.

scholarly journals Case for diagnosis

2013 ◽  
Vol 88 (1) ◽  
pp. 131-133 ◽  
Author(s):  
Paula Maio ◽  
Candida Fernandes ◽  
Ana Afonso ◽  
Fernanda Sachse ◽  
José Cabeçadas ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Skin Lesions ◽  
Cell Tumor ◽  
Lesional Skin ◽  
Systemic Involvement ◽  
Initial Stage ◽  
Dermal Infiltrate ◽  
Multiple Nodules

Blastic plasmacytoid dendritic cell tumor is a rare, highly aggressive systemic neoplasm for which effective therapies have not yet been established. We describe a 73-year-old man with multiple nodules and patches emerging on the trunk and limbs. Lesional skin biopsy revealed a plasmacytoid dendritic cell tumor with dense dermal infiltrate of tumor cells with blastoid features. No apparent systemic involvement was identified in the initial stage. The patient was treated with prednisone daily, with notorious improvement of the skin lesions, although no complete remission was obtained. During the six-month follow-up period, no disease progression was documented, but fatal systemic progression occurred after that period of time.

scholarly journals Sertoli-Leydig Cell Tumor with Concurrent Rhabdomyosarcoma: Three Case Reports and a Review of the Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Trisha Plastini ◽  
Arthur Staddon
Keyword(s):  
Leydig Cell ◽  
Malignant Tumors ◽  
Case Reports ◽  
Treatment Options ◽  
Treatment Strategies ◽  
Salvage Chemotherapy ◽  
Response To Chemotherapy ◽  
One Year ◽  
Lost To Follow Up

Sertoli-Leydig Cell Tumors (SLCTs) make up <1% of all ovarian tumors and are benign or malignant, androgen-secreting tumors. Rhabdomyosarcoma (RMS) is a heterogeneous group of malignant tumors that resemble developing skeletal muscle. There have been case reports of patients with concurrent SLCT and RMS with limited treatment options. We aim to demonstrate treatment strategies used in our patients, which seemed to have prolonged survival when compared to prior case reports of patients not cured by surgical resection. Herein we describe 22 cases of SLCT with RMS elements as discussed in prior case reports and three cases from the authors’ institution. Of the 19 cases from prior case reports, five were lost to follow-up and two had NED after surgical intervention. Eleven patients had recurrence and were deceased within one year. Of those patients not surgically cured, only three patients were documented as living beyond two years, all of whom received chemotherapy. The three patients presented from our institution had clinical evidence of response to chemotherapy that is traditionally used for RMS. In conclusion, chemotherapy with doxorubicin and ifosfamide has activity in patients with SLCT and RMS as does salvage chemotherapy with vincristine, irinotecan, and temozolomide.

Sirolimus Combined with Dexamethasone for the Treatment of a Patient with Interferon Resistant Hydroa Vacciniform-Like Lymphoma

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4992-4992
Author(s):  
Zhigang Liu ◽  
Yongqian Jia ◽  
Yuping Gong ◽  
Yong Guo ◽  
Jiazhuo Liu ◽  
...  
Keyword(s):  
T Cell ◽  
Conflicts Of Interest ◽  
Serum Biochemistry ◽  
Skin Lesions ◽  
Cytotoxic T Cell ◽  
Cell Phenotype ◽  
Ebv Dna ◽  
One Year

Abstract Abstract 4992 Hydroa vacciniform-like lymphoma is an unusual pediatric cutaneous T-cell lymphoma, which rarely involved with adults. Chemotherapy and/or radiotherapy had little or no benefit. Patients may have a response to α-interferon. We report here an adult case with interferon resistant hydroa vacciniform-like lymphoma that has been successfully treated with sirolimus, an mTOR pathway inhibitor. The patient is a thirty-five year old women,she begun with recurrent cutanous rash and small vesicule around her mouth and nose 3 years ago. One year before, the symptoms were exacerbated with obvious vesiculopapular eruption, edematous, blisters, ulcers, scarring and crusts in her face. Scattered vesiculopapular could be seen in her upper chest and extremities accompanied with fever and submandibular lymphadenopathy. Facial skin biopsy showed angiocentric infiltrates from the epidermis to the subjacent dermis. The infiltrate cells showed a cytotoxic T-cell phenotype, with positive TCR gene rearrangement and EBER expression in situ hybridization. EBV serum test showed EBV-IgA and IgG positive, but serum EBV-DNA was negative. Patient was given α-interferon 300 million units, intramuscularlly, two times a week, plus prednisone 30mg per day, orally. Patients' skin lesions were improved, but present recurrent episodes of papulovesicular eruptions. For the past 3 month patient had a recurrent high fever and exacerbated vesiculopapular eruptions and blisters. Laboratory test showed anemia and neutropenia, serum biochemistry and marrow examination confirmed that a lymphoma associated hematophagocytosis was complicated. Sirolimus was started at an initial dose of 1.5mg, orally, every 12 hours, combined with dexamethasone 10mg/day, intravenously. Three days later the fever begun to resolved and the skin lesion gradually subsided. Two weeks later the skin lesions disappeared and the dose of sirolinmus begun to tapered to 1.0mg, orally, every 12 hours, with prednisone 30mg/day, orally. By three month of follow-up the patient still on stable and be observed. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Inion Bony Solitary Plasmocytoma: A Case Report

2020 ◽  
Vol 6 (2) ◽  
pp. 83-88
Author(s):  
Vivek Agrawal ◽  
◽  
Kunal M.Ch Dholakia ◽  
Keyword(s):  
Multiple Myeloma ◽  
Brain Mri ◽  
Residual Tumor ◽  
Myeloma Cell ◽  
Venous Sinus ◽  
Solitary Plasmacytoma ◽  
Systemic Involvement ◽  
Bony Lesion ◽  
One Year

ackground and Importance: Plasma Cell Dyscrasias (PCD) are a heterogeneous group of diseases having a spectrum from multiple myeloma to solitary plasmacytoma, a rare subtype, which constitutes 2 to 5% of all PCD. It typically involves axial bones but the involvement of skull bone is very rare. Case Presentation: We are reporting a case of inion bony lesion with venous sinus infiltration in a 55-year-old male patient, presented with pain, imbalance in walking, and swelling in the occipital region. Brain MRI suggested an extra-axial lesion with skull involvement and venous sinus infiltration. The tumor was infiltrating into the sinus with patent torcular venous confluence. Tumor decompression followed by radiosurgery of residual lesion was considered in pre-operative surgical planning. It was diagnosed as a case of myeloma cell disease on histopathology. Postoperative myeloma work-up confirmed the absence of any systemic involvement. The patient was given a course of radiotherapy. Conclusion: One-year follow-up with repeated MRI and myeloma investigations in the 3rd month, 6th months, and 1 year did not show any finding suggestive of progression to multiple myeloma. The follow-up of brain MRI showed a complete resolution of the residual tumor.

scholarly journals Therapeutic Challenges for Elderly Patients with Primary Hyperparathyroidism

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Kenneth Sluis ◽  
Hyon Kim ◽  
Yuling He ◽  
Beatrice Wong ◽  
Xiangbing Wang
Keyword(s):  
Primary Hyperparathyroidism ◽  
Chart Review ◽  
Treatment Options ◽  
Case Series ◽  
Retrospective Chart Review ◽  
Long Term Stability ◽  
Feasible Option ◽  
One Year

Primary hyperparathyroidism (PHPT) predominantly affects older adults, and parathyroidectomy can achieve definitive cure in symptomatic PHPT and asymptomatic meeting surgical criteria. As the population continues to age, the treatment of PHPT in octogenarians and nonagenarians presents a clinical conundrum. This case series presents the management of eight patients 85 years of age and older diagnosed with PHPT. A retrospective chart review of patients diagnosed with primary hyperparathyroidism were identified in a single institution. Those patients 85 years of age and older who were followed up for over one year were included in this case series. The literature on treatment options for this age group was also reviewed. Eight cases of PHPT patients aged 88 ± 2.5 years old with a follow-up average of 5.6 ± 4.4 years were reported in our case series. Six PHPT patients were medically managed and two PHPT patients underwent parathyroid resection. Most of the medically managed PHPT patients except for one had long-term stability of disease for over five years. The treatment of PHPT diagnosed in patients over 85 years of age presents a clinical challenge for which there is no clear consensus guideline. Our case series supports that medical therapy is a feasible option for PHPT patients over 85 years old.

Ustekinumab-induced Sarcoidosis in a Patient with Psoriatic Arthritis

2020 ◽  
Vol 15 (2) ◽  
pp. 163-166 ◽  
Author(s):  
Senol Kobak ◽  
Huseyin Semiz
Keyword(s):  
Psoriatic Arthritis ◽  
Skin Lesions ◽  
Chronic Inflammatory Disease ◽  
Acute Phase Reactants ◽  
Caseating Granuloma ◽  
Endobronchial Ultrasonography ◽  
Chest X Ray ◽  
Dose Corticosteroid ◽  
One Year

Background: Psoriatic Arthritis (PsA) is a chronic inflammatory disease that may affect different joints. Sarcoidosis is a Th-1 cell-related chronic granulomatous disease characterized by non-caseating granuloma formation. The coexistence of both the diseases is a rare entity. Ustekinumab, an IL12 / 23 inhibitor, has shown efficacy and safety in the treatment of PsA. Objective: This study presents a case with ustekinumab-induced sarcoidosis in a patient with PsA. Case Report: A 52 years old female patient with complaints of pain and swelling of the wrists, MCP, PIP and DIP joints and skin lesions was referred to our Rheumatology clinic. On her medical history, she had been under follow up for 5 years with the diagnosis of psoriasis and one year ago, she started to receive ustekinumab prescribed by a dermatologist. On physical examination, she had psoriasis skin lesions and arthritis of both wrists, MCP, PIP, DIP joints. Bilateral hilar lymphadenopathies were detected in the chest X-ray and thorax computed tomography. In laboratory tests, acute phase reactants and serum angiotensin-converting enzyme levels were high. Endobronchial ultrasonography biopsy was performed and non-caseating granuloma consistent with sarcoidosis was reported. Ustekinumab was discontinued, methotrexate and low-dose corticosteroid were started. The patient was clinically stable in the 6th month of the treatment and the findings were regressed. Conclusion: Sarcoidosis development appears to be a new paradoxical effect of ustekinumab therapy, being another biological agent.

scholarly journals Pemphigus Vulgaris Presented with Cheilitis

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Zaheer Abbas ◽  
Zahra Safaie Naraghi ◽  
Elham Behrangi
Keyword(s):  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Lower Lip ◽  
Additional Lesion ◽  
Autoimmune Blistering Disease ◽  
Systemic Corticosteroids ◽  
Erosive Lichen Planus ◽  
Blistering Disease ◽  
One Year

Background. Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. In 50 to 70% of cases, the initial manifestations of pemphigus vulgaris are oral lesions which may be followed by skin lesions. But it is unusual for the disease to present with initial and solitary persistent lower lip lesions without progression to any other location.Main Observations. We report a 41-year-old woman with dry crusted lesions only on the lower lip, clinically resembling actinic cheilitis and erosive lichen planus, but histopathological evaluation showed unexpected results of suprabasal acantholysis and cleft compatible with pemphigus vulgaris. We treated her with intralesional triamcinolone 10 mg/mL for 2 sessions and 2 g cellcept daily. Patient showed excellent response and lesions resolved completely within 2 months. In one-year follow-up, there was no evidence of relapse or any additional lesion on the other sites.Conclusion. Cheilitis may be the initial and sole manifestation of pemphigus vulgaris. Localized and solitary lesions of pemphigus vulgaris can be treated and controlled without systemic corticosteroids.

Multicentric reticulohistiocytosis: the Mayo Clinic experience (1980–2017)

Rheumatology ◽  
2019 ◽  
Vol 59 (8) ◽  
pp. 1898-1905 ◽  
Author(s):  
Catalina Sanchez-Alvarez ◽  
Avneek Singh Sandhu ◽  
Cynthia S Crowson ◽  
David A Wetter ◽  
Gavin A McKenzie ◽  
...  
Keyword(s):  
Mayo Clinic ◽  
Treatment Options ◽  
Unmet Need ◽  
Skin Lesions ◽  
Multicentric Reticulohistiocytosis ◽  
Need For Treatment ◽  
Clinic Experience ◽  
Joint Effusions ◽  
Rheumatic Conditions ◽  
Record Review

Abstract Objectives Multicentric reticulohistiocytosis (MRH), a rare histiocytic disease that can mimic other rheumatic conditions, may be associated with cancer and other autoimmune disorders. To better understand the disorder and its other associations, we aimed to evaluate clinical correlates and outcomes of all patients with MRH seen at Mayo Clinic, Rochester between 1980 and 2017. Methods A retrospective medical record review was conducted to identify all patients with MRH between 1 January 1980 and 30 April 2017. Results We identified 24 patients with biopsy-proven MRH (58% female, 75% Caucasian, median age at diagnosis 52 years, median follow-up of 2.3 years). All patients had cutaneous and articular involvement; 23 (96%) patients had papulonodular skin lesions (87% periungual and dorsal hand) and seven (30%) mucosal nodules; and 22 (92%) patients had arthralgias, 21 (88%) joint effusions and 13 (54%) synovitis. Most frequently used therapies included corticosteroids, cyclophosphamide, methotrexate and bisphosphonates. Biologics were used in four patients. Nine patients had symptomatic resolution at 1 year and 12 partial improvement. Radiological findings included erosive changes in three (60%) patients and arthritis mutilans in two patients (40%). Twenty-nine per cent of patients had a concomitant autoimmune disease and 25% malignancy including melanoma, endometrial, peritoneal and lung carcinoma. The 5-year survival rate was 85% (95% CI: 74, 100%). Conclusion To our knowledge, this is the largest single-centre series of patients with MRH highlighting the rarity of the condition and an unmet need for treatment options that can allow sustained disease remission. It also highlights the need for a high vigilance for malignancy and autoimmune diseases.

scholarly journals Hypereosinophilic dermatitis: generalised lichenification and gyrate erythema as the sole manifestation of idiopathic hypereosinophilic syndrome

2019 ◽  
Vol 12 (10) ◽  
pp. e232142
Author(s):  
Deepika Yadav ◽  
Ananya Sharma ◽  
Shipra Agarwal ◽  
Vishal Gupta
Keyword(s):  
Hypereosinophilic Syndrome ◽  
Oral Prednisolone ◽  
Bone Marrow Examination ◽  
Skin Lesions ◽  
Idiopathic Hypereosinophilic Syndrome ◽  
Systemic Involvement ◽  
Cytogenetic Studies ◽  
Atypical Cells ◽  
Peripheral Blood Eosinophilia

A 22-year-old female presented with generalised lichenification and severe pruritus, along with multiple annular papules and concentric plaques over trunk and extremities for the last 3 years. Her haematological investigations revealed leucocytosis with peripheral blood eosinophilia and raised serum IgE levels. Skin biopsy showed perivascular and interstitial infiltrate of eosinophils and lymphocytes in the dermis. Bone marrow examination showed myeloid hypercellularity with increased number of eosinophils, but no atypical cells. Cytogenetic studies did not reveal any chromosomal alterations. No systemic involvement was found on imaging. A diagnosis of idiopathic skin-limited hypereosinophilic syndrome was made. She was treated with tapering doses of oral prednisolone and weekly methotrexate with significant improvement in skin lesions and pruritus in 2 months, which was maintained at 7-month follow-up.

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