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Key Clinical Message Generalized lichen planus pigmentosus significantly improved with the daily administration of Tofacitinib at a dosage of 15 mg.

Key clinical message Subungual Onycholemmal Cyst (SOC) is a rare nail abnormality with different clinical presentations which can mimic different nail malignancies, such as melanoma, SCC, or glomus tumor. It is necessary for dermatologists and dermatopathologist to be aware of this pathology to make the proper diagnosis and treatment. SOC is a rare nail abnormality which affects the dermis of the nail bed. SOC has different clinical presentations, including onychodystrophy, ridging, clubbing, thickening, pigmentation, or even normal appearance. It can mimic different nail malignancies, such as melanoma, SCC, or glomus tumor. In this report, we describe a 54‐year‐old man with unilateral second right finger nail onychodystrophy and onycholysis for 1 year. He did not have any history of recent trauma, pain, or bleeding. It was completely resected by surgery. Nail biopsy can contribute to the early diagnosis of SOC and improvement of treatment outcomes.

This study focused on a rare case of an isolated renal hydatid cyst. A 58-year-old asymptomatic woman was referred to the urologic clinic after an abdominal computed tomography (CT) scan revealed a renal cystic mass. While immunologic tests were normal, the final diagnosis was a renal hydatid cyst after radical nephrectomy of the affected kidney. We believe isolated occurrence of hydatid cysts is rare.

Malignant melanoma in the urethra is a rare tumor that is difficult to diagnose and treat, leading to a poor prognosis. In this paper, we present the case of a 36-year-old woman with history of invasive rectal adenocarcinoma (PT2N0Mx) who was tumor free for 5 years presented to urology outpatient with history of poor stream, dysuria, and dyspareuria. On examination, there was a huge mass in the meatus of urethra. Urethral malignant melanoma shows a high rate of local recurrence, about 60 % in 1 year. Overall survival in a series of 11 cases at 3 years was 27 %.

Hereditary progressive mucinous histiocytosis (HPMH) is an extremely rare progressive non-Langerhans cell histiocytic disorder presenting with only cutaneous manifestations. Patients typically present with multiple asymptomatic dome-shaped erythematous papules, usually involving the face and upper extremities. Twenty-six cases have been reported worldwide, with no spontaneous regression. Treatment with thalidomide stopped the progression of the disease in two cases. We report a case of progressive mucinous histiocytosis in a 31-year-old female patient with a history of tuberculosis who presented papular lesions on the face that later extended to the hands. She was treated with isoniazid for tuberculosis and isotretinoin for the skin lesions; the improvement was minimal during the next two months, with new lesions appearing on both hands. Thalidomide stopped the progression of the disease. The cause and pathogenesis of HPMH are undetermined. The pathogenesis of HPMH may be similar to that of lysosomal storage disease, considering the intra-cytoplasmic phospholipid deposition in both diseases, in addition to the likelihood of a role of macrophages in triggering the disease. In our patient, tuberculosis may have contributed.

Key Clinical Message Here, we report a case of granulomatosis with polyangiitis presenting with unilateral parotid gland enlargement and later developed skin lesions on the lower extremities and abdomen. Although rare, salivary gland enlargement may be the presenting sign of Wegener's granulomatosis or other ANCA associated vasculitides.

Key Clinical Message Pyoderma gangrenosum (PG) is a neutrophilic dermatosis associated with underlying disorders. The association between PG and solid organ tumors (SM), including gynecologic cancers, has been previously reported. Here, we report a case of a 61‐year‐old woman with pyoderma gangrenosum on the posterior auricular region associated with an underlying fallopian tube carcinosarcoma: a rare and aggressive gynecologic malignancy. The patient's ulcer responded favorably to treatment, and surgical resection of the tumor was performed. The patient was then referred for further cancer management. No new lesions or recurrences were found over the 18 months of routine follow‐up.

Key Clinical Message Waldenström's macroglobulinemia may begin with constitutional symptoms that are common in primary care settings and it is crucial for physicians to be aware of the potential complications of hyperviscosity syndrome and to employ the appropriate diagnostic methods in order to achieve better outcomes. Abstract Waldenström's macroglobulinemia (WM) refers to a type of lymphoplasmacytic lymphoma distinguished by the hyperproliferation of plasma cells, lymphocytes, and plasmacytoid lymphocytes. The disease is primarily diagnosed by increased monoclonal immunoglobulin M (IgM) levels and lymphoplasmacytic cell infiltration into the bone marrow. Individuals exhibit a high risk for hyperviscosity syndrome (HVS) as immunoglobulin levels increase. In addition to constitutional symptoms (fever, night sweats, and unintentional weight loss), clinical findings such as cytopenia, hepatosplenomegaly, and lymphadenopathy, this condition may cause hyperviscosity‐related organ failures. Here we discuss a patient with WM who presented with neurological complaints and blurry vision and developed necrosis at distal portions of his body during the 6‐month course of the disease.

Synovial Sarcoma (SS) is a rare soft-tissue malignancy. Only about 15% of SS originates from the retroperitoneum. Retroperitoneal SS (RSS) is usually diagnosed incidentally due to the anatomy of the retroperitoneum. The most common complaints of patients are abdominal and low back pain. Other common symptoms of RSS are palpable abdominal mass, weight loss, and anemia. In this study, we will describe a 29-year-old white Asian man with a diagnosis of RSS after radical nephrectomy. He was admitted to the Urology Department of Shohada-e Tajrish hospital, Tehran, Iran in March 2019. The distinguishing feature of this case is the size of the mass, which has never been reported so much for retroperitoneal synovial sarcoma. Radiologic imaging showed a huge retroperitoneal mass originating from the kidney. Based on the pathologic features and immunohistochemistry (IHC) study, the diagnosis was consistent with synovial sarcoma. Accurate diagnosis of RSS is usually based on the pathological findings. Therefore, in case of doubt, a biopsy can be employed. Surgical resection of the tumor and lymph nodes dissection is the main and most important part of the treatment. Aggressive resection with free margin is recommended. The role of adjuvant and neoadjuvant chemotherapy in RSS is not certain to date, but it is recommended according to the patient’s condition.

Retinoblastoma (RB) is one of the most common childhood cancers caused by RB gene mutations (tumor suppressor gene in various patients). A better understanding of molecular pathways and the development of new diagnostic approaches may lead to better treatment for RB patients. The number of studies on ceRNA axes is increasing, emphasizing the significance of these axes in RB. Circular RNAs (circRNAs) play a vital role in competing endogenous RNA (ceRNA) regulatory axes by sponging microRNAs and regulating gene expression. Because of the broadness of ceRNA interaction networks, they may assist in investigating treatment targets in RB. This study conducted a systematic scoping review to evaluate verified loops of ceRNA in RB, focusing on the ceRNA axis and its relationship to circRNAs. This scoping review was carried out using a six-step strategy and the Prisma guideline, and it involved systematically searching the publications of seven databases. Out of 363 records, sixteen articles were entirely consistent with the defined inclusion criteria and were summarized in the relevant table. The majority of the studies focused on the circRNAs circ_0000527, circ_0000034, and circTET1, with approximately two-fifths of the studies focusing on a single circRNA. Understanding the many features of this regulatory structure may help elucidate RB’s unknown causative factors and provide novel molecular potential therapeutic targets and medical fields.

Abstract Keratosis lichenoides chronica (KLC) is a rare dermatosis which represents different clinical characteristics between adult‐ and pediatric‐onset cases. We described a childhood case of KLC with features typical for adult‐onset disease. Acitretin led to partial improvement of her skin, but not mucosal, lesions.

Abstract Necrobiosis lipoidica‐like lesions, in known cases of sarcoidosis, can be considered as a member of the broad spectrum of histologic changes in sarcoidosis.

Psoriasis is a chronic immune-related disorder in which both genetic and environmental parameters are involved. Recent studies have demonstrated dysregulation of long non-coding RNAs (lncRNAs) and microRNAs (miRNAs) in the peripheral blood or skin lesions of patients with psoriasis. While a number of lncRNAs such as MEG3, AL162231.4 and NONHSAT044111 have been down-regulated in the course of psoriasis, others including PRINS, MIR31HG, RP6‐65G23.1, MSX2P1, SLC6A14-1:1, NR_003062 have been up-regulated. Moreover, expressions of several miRNAs have been dysregulated in this disorder. Among dysregulated miRNAs are miR-126, miR-143, miR-19a and miR-155 whose diagnostic roles in the psoriasis have also been assessed. Dysregulated non-coding RNAs in this disorder participate in the regulation of chemokine signaling pathway and immune response, control of epidermal development and skin barrier as well as modulation of function of certain subsets of T cells. Besides, these transcripts possibly regulate activity of NF-κΒ, mTOR, MAPK and JAK-STAT signaling pathways. Besides, expression levels of circRNAs have been decreased in the psoriasis lesions. Massive alterations in the levels of lncRNAs and miRNAs in the psoriasis lesions or peripheral blood of affected individuals show participation of these transcripts in the pathogenesis of this disorder.

Abstract Background: Clinical and histological features may overlap between lichen planopilaris-associated and discoid lupus erythematosus-associated scarring alopecia. Objectives: The aim of this study was to demonstrate the cutaneous infiltration of plasmacytoid dendritic cells and to compare their distribution pattern in discoid lupus erythematosus and lichen planopilaris. Methods: Twenty-four cases of discoid lupus erythematosus and 30 cases of lichen planopilaris were examined for immunostaining of the CD123 marker. The percentage and distribution pattern of plasmacytoid dendritic cells and the presence of the plasmacytoid dendritic cells clusters were evaluted in the samples. Results: The number of plasmacytoid dendritic cells was higher in the discoid lupus erythematosus specimens. Aggregations of 10 cells or more (large cluster) were observed in half of the discoid lupus erythematosus specimens and only 2 lichen planopilaris, with 50% sensitivity and 93% specificity for differentiating discoid lupus erythematosus from lichen planopilaris. Study limitations: Incidence and prevalence of discoid lupus erythematosus-associated scarring alopecia in the scalp are low, so the samples size of our study was small. Conclusions: We suggest that a plasmacytoid dendritic cells cluster of 10 cells or more is highly specific for distinguishing discoid lupus erythematosus from lichen planopilaris. It also appears that CD123 immunolabeling is valuable in both active and late stages of the disease.

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm of the sweat glands. It is believed to arise from the malignant transformation of syringocystadenoma papilliferum (SCAP). The majority of cases present on the head and neck and up to 17% of cases show metastatic progression. These tumors seldom occur in the anogenital area and, to date, only one case has been reported on the penis. Here, we report a rare case of SCACP in a 65-year-old man who presented with an erythematous, non-healing, ulcerated lesion on the penis.

Abstract Observing a metastatic malignant melanoma and its primary lesion at the same time is rare. The histopathological detection of any unusual pigmented lesion is critical.

Abstract COVID‐19 should be considered as a new triggering factor for autoimmune disorders like DM‐lupus overlap syndrome. We recommend that patients presenting with dermatomyositis during this pandemic be screened for COVID‐19.

Abstract During the COVID‐19 pandemic, physicians must maintain a high index of suspicion for COVID‐19 in cases of urticarial vasculitis or other forms of urticaria. This is particularly important for acute presentations in otherwise asymptomatic individuals and pregnant women, where a prompt approach to the patient can prevent undesirable complications.

Background and Objective: Infertility refers to the failure in achieving pregnancy of a couple after one year of regular sexual intercourse without using a protection method. The purpose of this research work was to evaluate the current status of the test and quality control performance in semen analysis in selected laboratories. Material and Methods: The semen analysis was performed in the Laboratory of Andrology in terms of macroscopic examination which include volume, color, viscosity, pH and acidity, and in terms of microscopy: the rate of sperm movement, the exact number of sperms per ml of semen, the percentage of sperm viability and movement, the presence of germ cells and white blood cells. Several questions for each part of the test were selected and answered by the director of the laboratories or andrology section supervisor. Results: There was a wide range in the performance of selected medical laboratories in Tehran regarding the standards of semen analysis according to the World Health Organization (WHO) Laboratory Manual for the examination and processing of human semen, fifth edition in 2010. They followed the instructions related to the sample collection in about 70% of the evaluated parameters, initial macroscopic examination in about 87% of the selected subjects, and the microscopic evaluation of sperm in about 65% of the test parameters. Conclusion: some laboratories do not follow the instructions of the WHO in performing semen analysis, and most of them do not follow the suggested methods in all parts of the test.

A 34-year-old man presented with bloody urethral discharge, dysuria, cough and right testicular mass. He had a history of anterior urethral stricture and multiple urethral dilation procedures. Radical orchiectomy and urethral mass biopsy were performed. The pathologist reported both of specimens revealed embryonal carcinoma. Abdominal and chest CT scan showed multiple metastasis. chemotherapy was started with the Bleomycin, Etoposide, and Cisplatin (BEP) regimen and this cycle was repeated every 3 weeks up to four times. Unfortunately, this patient died of brain metastasis.

Background and Objectives: Gliomas are the most prevalent subgroup of primary brain tumors with a relatively high mortality. However, oligodendrogliomas have a better prognosis compared to other subtypes due to their sensitivity to chemotherapy. Considering the low incidence and the resulting lack of information about oligodendrogliomas, particularly in Iran, this study aimed at assessing their basic characteristics. Methods:In this descriptive retrospective study, patients with definite diagnosis of oligodendroglioma were identified by reviewing the archives of pathology reports at the department of pathology of Shohada-e Tajrish Hospital during years 2008 to 2014. Age, gender, location, and the grade of the tumor were extracted and entered to the SPSS statistical software for analysis. Results: A total of 182 patients, including 115 males (63.2%) and 67 females (36.8%), were included with a mean age of 38.5±13.36 years. Frontal lobe was involved in 53 patients (29.1%), parietal lobe in 31 (17.0%), temporal lobe in 22 (12.1%), frontoparietal area in 15 (8.2%), parieto-occipital area in 11 (6.0%), temporoparietal and frontotemporal areas each in 9 subjects (4.9%), occipital lobe in 5 (2.7%), and the brainstem in 4 (2.2%). Furthermore, 108 cases (59.3%) had grade-2 and the remaining74 patients (40.7%) had grade-3 anaplastic oligodendrogliomas. The mean age of subjects with brainstem oligodendrogliomas was significantly lower than the other patients (p=0.025). Conclusion: Oligodendrogliomas commonly effects the frontal lobe, followed by the parietal and temporal lobes. The mean age of subjects with brainstem lesions was significantly lower than other patients. Age, gender or location of the tumor did not independently predict a higher grade lesion.

Superficial acral fibromyxoma (SAF) is a slow growing soft tissue tumor that mainly appears in the acral areas. Here, we report a case of a SAF with distinctive immunophenotype charachteristics. An 18-year-old female was referred to our clinic with the complaint of painless subungual nodule of great toe for a few months. The diagnosis of SAF was made according to histopathology and immunohistochemical (IHC) study, however, the IHC assessment showed positive staining with vimentin, focal reaction with smooth muscle actin, negative reaction with CD34, and positive staining pattern with CD99. These IHC findings are unusual for SAF. This reported case of SAF supports the fact that, although CD34 expression is characteristic for SAF, it is not always present.

Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and middle-aged adults. There is no sex predilection for this entity. The lesions could heal spontaneously, but usually recur and become chronic. Our patient was an 11-year-old girl suspected to have primary combined immunodeficiency complicated by chronic recurrent vegetating pustular lesions on the face and postauricular area since one year of age. The histological features of the lesions were consistent with pyoderma vegetans. This is the first case of PV beginning from early infancy in the setting of primary immunodeficiency and in an unusual location.

Primary signet-ring cell adenocarcinoma of bladder is a rare neoplasm, usually seen in middle age adults. We report the case of an 18-year-old man who presented with intermittent gross hematuria. Computed tomography imaging showed multifilling defects in the bladder. The patient underwent a transurethral resection of the bladder tumor. Histological findings were consistent with poorly differentiated mixed mucinous and signet-ring cell adenocarcinoma. We ruled out other possible origins of tumor by gastrointestinal endoscopy and colonoscopy. The patient was treated with radical cystectomy with prostate and seminal vesicle sparing technique and orthotopic diversion using “W” ileum pouch with pelvic lymphadenectomy to the bifurcation of the aorta was done. Six-month follow-up of patient showed normal conditions without metastatic spread or any recurrence.

A man with a history of urothelial carcinoma is presented here. According to investigations, he had bilateral hydronephrosis due to the pressure effect of a large mass in his bladder. The patient underwent surgical procedure including mass resection and ureter reimplantation. The final pathology report was only fat necrosis.

Synovial sarcoma is a type of malignancy which usually occurs near the joints of the arm, neck, or leg. It is a sarcoma of soft tissue and accounts for 5-10% of all adult soft tissue sarcomas in the world. We present a case with primary superficial cutaneous synovial sarcoma without involvement of the underlying knee joint. It is a very rare condition, and to the best of our knowledge, it is the second report of this topic. Although it is rare among soft tissue tumors, the dermatologists should have precise attention to this skin tumor, as early diagnosis is associated with lower metastatic rate and therefore better prognosis.