Polyarteritis nodosa presenting with frank hematuria

Polyarteritis nodosa (PAN) is an uncommon systemic vasculitis characterized by necrotizing inflammation of small- or medium-sized arteries. The disease normally presents with non-specific symptoms. Urological symptoms at presentation are extremely rare. We report a 65-year-old man who was diagnosed with a polyarteritis nodosa having presented atypically with left testicular pain and swelling, and an intratesticular lesion. He developed painless visible hematuria while under investigation. No gross arterio-venous fistula was seen to suggest a false aneurysm. Subsequently, laboratory studies showed positive anti-neutrophil cytoplasmic antibody levels and a raised erythrocyte sedimentation rate. This was an unusual presentation of PAN diagnosed with multidisciplinary input from the urology, radiology and nephrology teams.Polyarteritis nodosa (PAN) is an uncommon systemic vasculitis characterized by necrotizing inflammation of small- or medium-sized arteries. It was the first vasculitis to be described by Kussmaul and Maier in 1866 and was named “periarteritis nodosa.”¹Signs and symptoms of this disease are usually vague and non-specific. Malaise, weakness, fever, headache, arthralgia, myalgia, chronic renal failure, neuropathy and transient ischemic attacks are common presentations. Angina, myocardial infarction and congestive cardiac failure are rarely seen at the first presentation. Abdominal pain with nausea, vomiting or per rectal bleeding, as well as hepato-pancreatic infarction, have also been described. Many clinical symptoms are related to arterial branch occlusion leading to organ ischemia.² The lungs are usually not involved in systemic PAN.³ It affects 2 to 6 people per 100 000 per year, and can be seen in all ethnic groups. Any age group can be affected, but it is commonly seen in people between the ages of 40 and 60. The incidence is higher in areas where hepatitis B is endemic.⁴Common investigations to diagnose PAN include positive anti-neutrophil cytoplasmic antibodies (ANCA), hepatitis B surface antigen and a raised erythrocyte sedimentation rate (ESR). A full blood count shows raised inflammatory parameters (neutrophils) and gamma globulins.⁵ Angiography shows micro aneurysms, which is considered the gold standard for diagnosis.⁶We report the case of a 65-year-old man who was diagnosed to have polyarteritis nodosa having presented atypically with hematuria. He also had a testicular lesion which was initially presumed to be a tumourous growth.The patient had a history of myelodysplasia (chronic myeloid leukemia), which is currently in remission and under regular follow-up. After being referred by hematologists with a suspected left testicular lesion, he visited the urology clinic. The lesion was cystic on examination and initial imaging with ultrasound suggested the possibility of mycotic aneurysmal lesions in the testicle (Fig. 1).Open in a separate windowFig. 1A testicular ultrasound, suggesting the possibility of mycotic aneurismal lesions.While he was being worked up for the suspected testicular lesion, he developed frank painless hematuria, which was initially managed with catheterization and bladder irrigation. His condition then deteriorated and he became acutely septic with respiratory compromise. Acute severe pneumonia was diagnosed which required prolonged ventilation. During the course of his intensive care unit admission, in spite of anti-biotic administration, his inflammatory parameters remained high. Hematuria continued requiring intermittent bladder irrigation and supplemental blood transfusions.Once his general condition improved, he had a cystoscopic evaluation. There were no gross abnormal lesions in the bladder, but blood was observed coming out of the left ureteric orifice. Ultrasonography suggested a left renal hematoma confirmed on a subsequent computed tomography scan (Fig. 2), which also showed small aneurysms in both kidneys. Renal angiography was undertaken with a view to embolization of any bleeding lesion. Angiography revealed numerous aneurysms in both kidneys measuring up to 15 mm in diameter (Fig. 3). Segmental infarcts were also noted in the lower pole of the right kidney. Further mesenteric angiography revealed small aneurysms in the head of the pancreas (Fig 4). Subsequently laboratory studies showed positive ANCA and raised ESR levels. Taken together, these findings suggested the diagnosis of PAN, and the patient was started on corticosteroid therapy. He made a good clinical recovery.Open in a separate windowFig. 2A computed tomography scan showing a renal parenchymal aneurysm.Open in a separate windowFig. 3The renal angiogram revealing multiple aneurysms and segmental infarct.Open in a separate windowFig. 4The mesenteric angiogram showing an aneurysm in the pancreatic head.Currently, he is on a reducing dose of steroid treatment and remains clinically well. His management continues under the nephrology and hematology teams for his PAN and myelodysplasia, respectively.     

Primary penile malignant lymphoma: report of a rare case

A 73-year-old male presented with a 3-month history of two penile masses: one on the shaft and one on the glans penis. Both lesions were poorly defined, fixated and without tenderness. The sizes were 1.0 × 1.0 × 1.0 cm and 1.2 × 1.5 × 1.5 cm, respectively. The patient underwent lumpectomy of the glans penis; we confirmed malignant lymphoma of the glans penis (B-cell derived; diffused large B-cell) by postoperative pathological examinations. CHOP (cyclophosphamide-hydroxydaunorubicin-oncovin-prednisone) chemotherapy was administered. The patient was tumour-free at the 33-month follow-up.Primary penile malignant lymphoma is very rare, there are only 26 reported cases worldwide.A 73-year-old male presented with a 3-month history of penile mass; he was otherwise healthy. Physical examinations indicated the following: normal vital signs, with no enlarged superficial lymph nodes; no abnormalities in cardiopulmonary examination; flat abdomen; liver and spleen were impalpable, with no palpable mass; kidney was impalpable, with no tenderness. Digital rectal examination revealed a hyperplastic prostate, with no nodule. Two masses were discovered on the shaft of penis and glans penis; the masses were firm, poorly defined, fixated and no tenderness. Their respective sizes were: 1.0 × 1.0 × 1.0 cm and 1.2 × 1.5 × 1.5 cm. Routine blood/urine tests, liver and kidney function tests were normal. His prostate-specific antigen was 3.05 ng/mL. Upon ultrasound and computed tomography (CT) scanning, his prostatic hyperplasia measured 3.0 × 4.0 × 4.5 cm and was 28.08 mL; there were no abnormalities with the liver, gallbladder, pancreas, spleen, kidneys and ureter, and no enlargement of the retroperitoneal lymph nodes. A plain chest radiograph showed no abnormalities.The patient underwent lumpectomy of the glans penis. At surgery, the mass on the shaft was unable to be resected, and thus only a biopsy tissue was taken. The mass on the glans penis was completely resected with some surrounding normal tissue, and primary suturing of the glans was performed. Pathological examinations indicated malignant lymphoma of the glans penis (B-cell derived; diffused large B-cell), and the mass margins on the glans were tumour-free; immunohistochemistry tests (Fig. 1, Fig. 2) indicated: CD3(−), CD792(+), LCA(+), L26(+), BCL6(−), BCL10(−), UCHL1(−), KP−1 cell(+). Postoperative bone marrow biopsy showed trilineage hyperplasia; lymphocyte showed no significant abnormalities in percentage and morphology. Chemotherapy was administered after the operation: CHOP (500 mg/m² cyclophosphamide and 30–50 mg/m² adriamycin and l–2 mg oncovin intravenously infused on day 1; and 40 mg prednisone taken orally on days 1–5, with an interval of 3 weeks between two courses of treatment). Physical examination, routine blood/urine tests, liver and kidney function tests, serum electrolytes, serum tumour makers, plain chest radiograph, abdominal and pelvic CT were examined to exclude recurrence and metastasis at each follow-up (postoperative month 3, 6, 9 and years 1 and 2. The follow-up showed regression of the mass on the shaft of penis and no signs of recurrence.Open in a separate windowFig. 1Hematoxylin and eosin staining of primary penile lymphoma, ×400.Open in a separate windowFig. 2Immunohistochemistry: leukocyte common antigen-positive tumour cells presented brown, ×400.     

Recurrent transitional cell carcinoma of the bladder: A mixed nested variant case report and literature review

Nested variant of urothelial cell carcinoma (NVUC) is a rare histological entity, with about 80 reported cases. It has a deceptively benign appearance with an aspect characterized by confluent small nest or urothelial’s cell tubules. This tumour often resembles inverted papilloma, von Brunn’s nests (VBNs), cystitis cystica, nephrogenic metaplasia and sometimes usual transitional cell cancer. It is very important to be able to distinguish between benign lesions and nested variant bladder cancer because, in spite of its bland morphology, there is evidence that it behaves aggressively.A 70-year-old man with hematuria underwent ultrasound examination and cystoscopy that revealed the presence of a bladder cancer. The trans-uretheral resection of the tumour revealed a solitary high-grade cancer with lamina propria involvement (T1G3). Five months after the operation and after an adjuvant endovesical treatment with Bacillus Calmette-Guérin (BCG), a follow-up cystoscopy revealed a recurrent tumour that was resected. Pathological staging and microscopic examination revealed a high-grade (G3) nested variant transitional cell carcinoma (TCC) with a deep lamina propria involvement (T1b). The tumour was characterized by high expression of the tumour suppressor gene p53 and by immunoreactivity for proliferation marker Ki-67. The patient was then submitted to radical cystectomy and a diagnosis of mixed urothelial nested variant tumour at stage pT2a and grade G3 with a linfatic involvement was made. Twelve months after the first diagnosis of bladder cancer, the patient underwent a cycle of intravenous gemcitabine along with cisplatin.The aggressive behaviour of this neoplasm suggests that the correct indication should be early radical cystectomy with extended lymph adenectomy to avoid the progression into the bladder wall or the metastatic spread.Nested variant of urothelial cell carcinoma (NVUC) is a rare histological entity that was described for the first time in 1989 by Talbert and Young.¹ This variant of urothelial cancer is often not recognized and its clinical and pathological characteristics are not completely defined. At present, correct management is still not defined, as well as its relationship with conventional TCC. Solitary nested variant tumours have been observed, as well as cases synchronous with urothelial bladder cancer.² Furthermore, there are cases with ureter involvement in association or not with the urinary bladder.³ About 80 cases are reported and the largest series (30 cases) is described by Wasco and colleagues.⁴ Nested variant lesions have a deceptively benign appearance with an aspect characterized by confluent small nest or urothelial’s cell tubules infiltrating lamina propria and/or muscular layer. There are urothelial cells with mild pleomorphism, slightly increased nuclear/cytoplasmic ratio and occasionally prominent nucleoli.⁵This variant of urothelial bladder cancer often resembles clinical and histological features of inverted papilloma, von Brunn’s nests (VBNs), cystitis cystica, nephrogenic metaplasia and sometimes usual TCC. It is very important to be able to distinguish between benign lesions and nested variant bladder cancer because, despite its bland morphology, there is evidence of its aggressive behaviour and its capacity to progress to muscle invasive and metastatic disease.A 70-year-old man, a smoker and with no occupational risk factors, consulted the urology department for hematuria and dysuria characterized by increased voiding frequency and nocturia. He underwent a bladder and kidney ultrasound examination and provide three samples for urinary cytology. Both exams revealed suspected bladder cancer, which was confirmed by the cystoscopic detection of a single right-lateral and trigone wall lesion of about 2 cm in diameter. The patient underwent transurethral resection of the bladder tumour (TUR-B) and random biopsies of suspect areas were performed. The pathological evaluation carried out at the pathology department revealed a high-grade tumour with lamina propria involvement (T1G3) and wide consensual phlogosis. A second-look TUR-B was performed 6 weeks after the first one and confirmed the prior diagnosis without muscular-layer spread. The patient started endovesical adjuvant immunotherapy with induction BCG, oncee weekly for 6 weeks; 45 days after endovesical treatment, he had a follow-up cystoscopy that revealed a new transition small lesion (less than 1 cm) that was resected. Pathological evaluation confirmed, again, high-grade a urothelial bladder cancer (G3) confined to lamina propria (T1). The patient repeated endovesical treatment with BCG and, after 5 months, a diagnosis of a recurrent tumour was made. The appearance of the tumour, located on the right wall, 2.5 cm in diameter, in the same area of the first resected lesion, was unusual since it was characterized by a mix of papillary cancer and another part resembling benign morphology, similar to inverted papilloma. Computed tomography (CT) scans showed thickened right bladder wall and neither pelvic lymph nodes swelling nor hydronephrosis were described. An endoscopic resection of the tumour, extended to the muscular layer, was performed. Pathological staging and microscopic examination revealed a high-grade (G3) nested variant TCC with a deep lamina propria involvement (T1b), without muscularis mucosae infiltration (Fig. 1). The tumour was characterized by high expression of the tumour suppressor gene p53 (Fig. 2) and by immunoreactivity for proliferation marker Ki-67 (Fig. 3). Both markers, pathological evaluation and high recurrence rate confirmed the high aggressiveness of this cancer; due to this, the patient was submitted to radical cystectomy with extended lymph nodes dissection. After the surgery, the bladder was studied and superficial muscularis mucosae involvement was observed (pT2a); moreover, 3 of 20 resected lymph nodes were positive for metastasis. Twelve months after the first diagnosis of bladder cancer, the patient was referred to the oncology department and a cycle of intravenous gemcitabine along with cisplatin was started.Open in a separate windowFig. 1.Transitional cell bladder cancer mixed with nests of urothelial cells.Open in a separate windowFig. 2.Tumour cells are strongly positive for p53 in some tumour cell nuclei.Open in a separate windowFig. 3.Immunoperoxidase staining for Ki-67, showing nuclear expression within tumour.     

Malignant peripheral nerve sheath tumour of the bladder associated with neurofibromatosis I

Neurofibromatosis is a hamartomatous disorder of autonomic peripheral nerve sheaths associated with peripheral nerve sheath tumours. Most tumours are neurofibromas; however, the genitourinary system is rarely involved. We present a rare case of a nerve sheath tumour of the bladder in a young patient, which was discovered to be malignant.A previously healthy 17-year-old woman presented with abdominal pain and recurrent urinary tract infections. She also had intermittent difficulty passing urine and had bilateral loin discomfort. Physical examination was unremarkable aside from fullness in the suprapubic area and skin pigmentation in keeping with her known history of neurofibromatosis 1. Laboratory investigations revealed a mildly raised creatinine of 160 mmol/L and raised inflammatory markers with an erythrocyte sedimentation rate of 80 mm/h. Abdominal ultrasonography revealed moderate bilateral hydronephrosis and a pelvic mass. Computed tomography imaging confirmed a large bladder mass and showed evidence of bilateral hydronephrosis (Fig. 1). The patient subsequently had magnetic resonance imaging of her pelvis, which revealed a very large lobulated mass within the pelvis, lying anterior to the uterus and directly involving the bladder (Fig. 2). The patient underwent surgery with cystectomy and ileal conduit formation. At pathological histology, the mass was shown to be a malignant peripheral nerve sheath tumour of the bladder wall (Fig. 3 and Fig. 4).Open in a separate windowFig. 1Postcontrast axial computed tomography image of the lobulated mass within the bladder.Open in a separate windowFig. 2Axial T₁-weighted magnetic resonance imaging demonstrating the heterogenous lobulated bladder mass (A); sagittal T₁-weighted image of the pelvis, demonstrating the extent of the heterogenous mass (B).Open in a separate windowFig. 3Corresponding gross specimen of the bladder expanded by a large white variegated and lobulated tumour, primarily based in the bladder wall.Open in a separate windowFig. 4Histological specimen that stains positive for smooth muscle actin confirming the diagnosis of malignant peripheral nerve sheath tumour (original magnification × 20).     

Soft tissue case 61

A 55-year-old man presented to the emergency department with a 12-hour history of severe crampy abdominal pain, nausea, vomiting and obstipation. The patient had a complex medical history, including coronary artery disease, lupus, hypothyroidism, epilepsy, pancreatitis and renal calculi. However, the patient had no history of a hernia or abdominal surgery. Physical examination revealed a temperature of 38.5°C and a soft distended abdomen that was diffusely tender without signs of peritonitis. The rest of the physical examination was unremarkable. Routine laboratory investigations including a complete blood cell count, electrolytes, liver enzymes and amylase were normal, with the exception of a decreased hemoglobin level of 116 g/L. We ordered a plain abdominal radiograph (Fig. 1) and a contrast-enhanced computed tomography (CT) scan of his abdomen. What is your diagnosis?Open in a separate windowFig. 1Plain erect abdominal radiograph of a 55-year-old man presenting with abdominal pain, nausea, vomiting and obstipation.     

Gougerot-Carteaud Syndrome Treated with 13-cis-retinoic Acid

A 15-year-old Caucasian boy with a diagnosis of confluent and reticulated papillomatosis who had received numerous treatments with minimal responses cleared with a 20-week course of 13-cis-retinoic acid and has remained in remission. It is important to consider the use of oral retinoids in the treatment of this stubborn, unsightly, and psychologically upsetting disease.This case describes a 15-year-old Caucasian boy who presented with a pruritic, brownish eruption of a few years'' duration that was treated with diphenhydramine hydrochloride cream and moisturizers. His past history is unremarkable except for being premature due to abruptio placenta. He is overweight and plays football. His prior skin diagnoses include eczema, for which he was given topical steroids, and tinea versicolor, which responded minimally to oral and topical ketoconazole.On physical examination, the patient had brownish-tan sessile, velvety, hyperkeratotic, coalescing papules on the chest, back, nape of neck, and extensor surfaces of upper arms (Figures 1 and ​and2).2). Scraping produced a powdery scale.Open in a separate windowFigure 1Velvety, brown, hyperkeratotic lesions on the backOpen in a separate windowFigure 2Lesions on the abdomenA biopsy showed horn formation and was difficult to assess (Figure 3). Asteatotic eczema, pityriasis rubra pilaris, acanthosis, and fungus were considered and dismissed. In view of the deeper sections and the history, a diagnosis of confluent and reticulated papillomatosis of Gougerot et Carteaud (also known as Gougerot-Carteaud syndrome) was made.Open in a separate windowFigure 320x magnificationTreatment began with minocycline 100mg daily for three months. The dosage was tapered to 50mg daily over the next three months as the lesions faded and the patient''s skin felt smooth. Unfortunately, the lesions began to return, and isotretinoin was started at 1mg/kg or 60mg twice daily with food for 20 weeks. The patient''s triglycerides were monitored and fenofibrate was added in the third month of the regimen. The patient had an excellent response—the unsightly lesions were eliminated and his skin became smooth (Figures 4 and ​and55).Open in a separate windowFigure 4The patient''s back post-treatment—smooth skinOpen in a separate windowFigure 5The patient''s chest post-treatment     

A Case of a Generalized Lichenoid Tattoo Reaction

Cutaneous reactions to tattoos have been increasing in conjunction with the rise in popularity of tattoos. While localized lichenoid reactions to tattoo inks are fairly common, generalized lichenoid reactions are relatively rare. Herein the authors present a case of a generalized lichenoid reaction to a tattoo containing only black ink. They also present a brief discussion of tattoo reactions and treatment options.A 17-year-old boy with no past medical history presented five weeks after obtaining a professional tattoo with black ink on the left arm. Two weeks after the tattoo, he noted the onset of a generalized, pruritic rash. Examination revealed an African-American male teenager with numerous 1 to 4mm, flat-topped, skin-colored to hyperpigmented papules with a fine white scale, concentrated at the site of the tattoo, but also scattered over the trunk, genitals, and all four extremities, including the dorsal hands and feet (Figure 1-​-3).3). The oral mucosa was spared. A skin biopsy from the right forearm revealed a lichenoid infiltrate with few eosinophils (Figure 4 and ​and5).5). Liver function tests were within normal limits, and hepatitis C antibody was negative. He was treated with triamcinolone acetonide 0.1% cream applied topically twice daily and a two-week oral prednisone taper starting at 40mg. Unfortunately, despite multiple attempts to contact the patient, he was lost to follow-up. Open in a separate windowFigure 2Disseminated lichenoid papules Open in a separate windowFigure 1Lichenoid papules at the site of the tattooOpen in a separate windowFigure 3Disseminated lichenoid papules with koebnerizationOpen in a separate windowFigure 4Histopathologic findings demonstrating a lichenoidOpen in a separate windowFigure 5Histopathologic findings demonstrating eosinophils pattern     

Overcoming the Barrier Treatment of Ichthyosis: A Combination-therapy Approach

Ichthyosis vulgaris is an inherited disorder of keratinization that results in asteatotic scales on extensor surfaces of the arm, legs, and trunk. A combination-therapy approach with a physiological lipid-based barrier repair topical emulsion and ammonium lactate 12% lotion applied topically was shown to be effective at four-week follow up without any untoward side effects. This combination therapy addresses the importance of caring for both the corneocytes (“bricks”) and the intercellular lipid bilayer (“mortar”) for optimal benefit.A 10-year-old boy presented with a long-standing history of very dry skin involving the arms, legs, and trunk (Figures 1 and ​and2).2). Prior treatment regimens for the current condition were limited to use of over-the-counter moisturizers with limited success. The patient was in general good health with no known drug allergies and was using no medications. Family history was noncontributory except for his mother and one sibling having dry skin. Physical examination exhibited dry, fine, scaly patches on the trunk (Figure 1) and both the upper and lower extremities (Figure 2) with sparing of the flexural surfaces, including the popliteal, antecubital, axillary, and inguinal regions. A diagnosis of ichthyosis vulgaris (IV) was rendered based on the clinical features and history. The patient was treated with a combination regimen including ammonium lactate (AL) 12% lotion (Lac-Hydrin 12% Lotion, Ranbaxy Laboratories, Jacksonville, Florida), followed by a physiological lipid-based barrier repair cream (Ec) containing ceramides, cholesterol, and free fatty acids in a 3:1:1 ratio designed to simulate the normal intercellular lipid bilayer (EpiCeram^® Skin Barrier Emulsion, Promius Pharma LLC, Bridgewater, New Jersey). Both formulations were applied sequentially twice daily diffusely to the skin including the affected areas. At the one-month follow-up visit, the patient had essentially complete resolution of ichthyotic scaling and xerosis (Figures 3–5). The patient remained clear with continued use of the ensuing weeks and was subsequently lost to follow upOpen in a separate windowFigure 1Dry, scaly patches on the trunkOpen in a separate windowFigure 2Dry, scaly patches on both the upper and lower extremities with sparing of flexural surfacesOpen in a separate windowFigure 3Response to treatment (one-month follow up)Open in a separate windowFigure 5Response to treatment (one-month follow up)Open in a separate windowFigure 4Response to treatment (one-month follow up)     

Impalement Injury with Transanal Prolapse of Bowel Content Due to Intraperitoneal Rectal Tear

A 48-eight-year-old female patient came with prolapse of small intestines per rectum (Fig. 1) due to impalement injury with iron rod at a construction site. As the patient was hemodynamically unstable, she was taken for emergency laparotomy. A large rent in the intraperitoneal rectum was found (Fig. 2), with prolapse of intestines. Luckily for the patient, there was no other intra-abdominal organ injury. As there was no fecal contamination at all, a decision against proximal colostomy was made. The rent was closed in two layers after re-placing the small bowel contents with thorough lavage. Patient made a swift recovery thereafter.Open in a separate windowFig. 1Patient presented with prolapse of bowel contents per rectum due to injuryOpen in a separate windowFig. 2Intraoperative picture showing the perforation involving the rectum     

A Case of Mycosis Fungoides and Lymphomatoid Papulosis Occurring Simultaneously in a Child

Several studies have reported an association between lymphomatoid papulosis and other lymphomas, such as mycosis fungoides, Hodgkin’s disease, and anaplastic large cell lymphoma. The association between lymphomatoid papulosis and mycosis fungoides has been reported to be between seven and 39 percent. Although a relationship is acknowledged between lymphomatoid papulosis and mycosis fungoides, our understanding is limited. The authors report a case of mycosis fungoides and lymphomatoid papulosis in a child.An 11-year-old girl presented with a six-year history of asymptomatic, light patches on her face, arms, and legs. The patches initially faded in the winter months, but more recently have remained fixed. The patient also reported a three-year history of pruritic papules on her left forearm and wrist, a rash that started during a vacation in Jamaica. Topical corticosteroids and tacrolimus 0.1% ointment have reduced the erythema and scale on her face, but have not helped the pruritic papules on her left forearm. Review of systems was otherwise negative. The patient’s past medical history has otherwise been unremarkable.On physical examination, there were several pink to hyperpigmented papules on her left dorsal wrist (Figure 1). There were also multiple large, hypopigmented patches on her arms and legs (Figure 2). In addition, there were hypopigmented and erythematous patches with slight scale on the bilateral cheeks. Open in a separate windowFigure 1Pink, hyperpigmented papule on the left dorsal wristOpen in a separate windowFigure 2Multiple large, hypopigmented patches on the patient’s armsFour skin biopsies were taken. The left forearm sample showed a wedge-shaped, dense perivascular and interstitial infiltrate of lymphocytes with CD30(+) cells and a few CD20(+) cells. T-cell gene rearrangement studies were positive for distinct monoclonal peaks (Figure 3). The right arm biopsy displayed mild psoriasiform hyperplasia with lymphocytic epidermotropism, mild spongiosis, and necrotic keratinocytes. Few CD30(+) cells and CD20(+) cells were present. T-cell gene rearrangement studies were positive and identical to the left forearm sample (Figure 4). The left anterior thigh sample showed a superficial perivascular lymphoid infiltrate with mild epidermotropism. There were few CD30(+) cells with no CD20 positivity. T-cell gene rearrangement studies were predominantly negative, yet suspicious amplicons with identical size to the first two specimens were present. The left dorsal wrist sample displayed a perivascular infiltrate of lymphocytes accompanied by interstitial neutrophils and mild spongiosis. Some of the lymphocytes were large and pleomorphic with a moderate number of CD30(+) cells and few CD20(+) cells. T-cell gene rearrangement studies were suspicious for a positive and partial match to the left forearm and right arm samples. In addition, flow cytometry was performed and was within normal limits and an antinuclear antibody (ANA) was negative. Open in a separate windowFigure 3A high power view of the biopsy taken from the patient’s left forearm demonstrating a wedge-shaped, dense perivascular and interstitial infiltrate of Iymphocytes with many CD30(+) cellsOpen in a separate windowFigure 4A low power view of the biopsy taken from the patient’s right arm illustrating mild psoriasiform hyperplasia with lymphocytic epidermotropism, mild spongiosis, and necrotic keratinocytes.Thus, the authors diagnosed the patient with mycosis fungoides (MF)—patch stage—and lymphomatoid papulosis (LyP)—type A. She was treated with narrowband ultraviolet B (UVB) phototherapy (28 treatments) that has led to repigmentation of all of the hypopigmented areas, but has not improved the pruritic lesions on her left wrist. The plan is for her to continue narrowband UVB phototherapy for another 12 treatments.     

Inverse Papular Acrokeratosis of Oswaldo Costa: A Case Report

Acrokeratoelastoidosis of Oswaldo Costa, or inverse papular acrokeratosis, is a rare genodermatosis first described in 1952 by Oswaldo Costa, a Brazilian dermatologist. It is characterized by flesh-colored papules on the lateral aspects of the palms and soles and dorsum of hands. The histological features are hyperkeratosis, hyalinized and homogenous collagen, and a decrease in and fragmentation of the elastic fibers (elastorrhexis). In the absence of elastic fiber fragmentation, a similar clinical presentation is diagnosed as focal acral hyperkeratosis. Many cases of inverse papular acrokeratosis of Oswaldo Costa may have been considered focal acral hyperkeratosis since it can be difficult to find the elastorrhexis. The authors report a case of a 51-year-old woman with inverse papular acrokeratosis of Oswaldo Costa with poor response to topical treatments.A 51-year-old black woman from Rio de Janeiro complained of “small bumps on feet,” present for seven years. She sought medical consultation and used several creams with discreet improvement and subsequent worsening. Two years earlier she noticed similar lesions on her hands. She knows of no other cases in the her family and does not have a history of consanguinity. There was no history of exposure to arsenic, and she had no other symptoms.She presented with flesh-colored papules on the medial and lateral borders of the hands and feet (Figures 1 and ​and2).2). She did not present with palmoplantar desquamation, hyperhydrosis, vesicles, crusts or ungual alterations, or dermatological lesions on other sites. Histopathology showed more exuberant hyperkeratosis in small areas of epidermal depression. Orcein stain revealed a decrease and fragmentation of the elastic fibers. The histopathological picture was compatible with acrokeratoelastoidosis (Figure 3).Open in a separate windowFigure 1Flesh-colored papules on the medial borders of the feetOpen in a separate windowFigure 2Similar papular lesions on the handsOpen in a separate windowFigure 3Histopathology: Exuberant hyperkeratosis in a small area of epidermal depression (HE, 10x)     

Antibody and T Cell Response to SARS-CoV-2 Messenger RNA BNT162b2 Vaccine in Kidney Transplant Recipients and Hemodialysis Patients

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Fully Regressive Melanoma: A Case Without Metastasis

Fully regressive melanoma is a phenomenon in which the primary cutaneous melanoma becomes completely replaced by fibrotic components as a result of host immune response. Although 10 to 35 percent of cases of cutaneous melanomas may partially regress, fully regressive melanoma is very rare; only 47 cases have been reported in the literature to date. AH of the cases of fully regressive melanoma reported in the literature were diagnosed in conjunction with metastasis on a patient. The authors describe a case of fully regressive melanoma without any metastases at the time of its diagnosis. Characteristic findings on dermoscopy, as well as the absence of melanoma on final biopsy, confirmed the diagnosis.A 62-year-old man presented to our institution with a pigmented lesion on his back, which he noticed three weeks prior. The patient could not tell when the lesion first appeared.Cutaneous examination revealed a 12mm x 8mm asymmetric, variegated, pigmented patch with large slate-gray and pink dis colorations (Figure 1). No ulceration, crusting, active exsanguination, or granulation was noted. Examination of the axillae was negative for palpable lymphadenopathy.Open in a separate windowFigure 1.A 12mm x 8mm asymmetric, variegated, pigmented lesion consistent with cutaneous melanomaDermoscopic examination revealed scar-like depigmentation and multiple blue-gray “pepper-like” papules overlying a pink macular base (Figure 2). The lesion was surgically excised under local anesthesia with a 3mm margin. The histopathologic examination revealed a flattened epidermis, residual large melanocytes confined to the basal layer of the epidermis, but no obvious nests of melanocytes were noticed, and there was no pagetoid spread. The papillary dermis was slightly edematous with some fibrosis, dilated blood vessels, and a lymphohistiocytic infiltrate, as well as the presence of many melanophages. The reticular dermis and subcutaneous tissue were normal (Figure 3). Immunohistochemical staining with Melan A was negative for nests at the dermal-epidermal junction and dermis (Figure 4). Thus, the definitive diagnosis of fully regressive melanoma was made on basis of the constellation of the clinical, dermoscopic and pathologic findings.Open in a separate windowFigure 2.The demoscopie examination shows a pinkish scar-like macule and a gray-blue to black macule with gray-blue “pepperlike” papules; findings are consistent with regressive melanoma.Open in a separate windowFigure 3.Hematoxylin and eosin (H&E) histopathology: No obvious nests of melanocytes were noted, and there was no pagetoid spread. The papillary dermis shows lymphohlstiocytlc infiltrate and the presence of multiple melanophages. Magnification: 200xOpen in a separate windowFigure 4.Immunohistochemlcal staining with Melan A did not reveal the presence of nests at the dermal-epidermal junction in the dermis, confirming the absence of cancerous melanocytes.A re-excision of the initial lesion with a 1cm margin was performed based on depth of melanophage infiltration (less than 1mm) because Breslow index was not available. The patient underwent extensive work up for metastasis. The work up included cerebral MRI, thoracic and abdominal CT scans, and axillary ultrasonography; all were negative. The patient followed up with our service every three months for two years, followed by yearly thereafter, during which he underwent serial physical examinations with assessment of lymphadenopathy. The patient has had neither a local relapse of disease nor cutaneous metastases.     

Tripartite Separation of Glomerular Cell Types and Proteomes from Reporter-Free Mice

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Generalized Anetoderma after Intravenous Penicillin Therapy for Secondary Syphilis in an HIV Patient

Anetoderma is a rare, benign disorder characterized microscopically by the pan-dermal loss of elastic fibers in the dermis and presenting clinically as circumscribed, skin-colored or grey-white atrophic macules and/or patches on the trunk and/or extremities. Lesions are described as having a “sac-like” appearance, since they bulge or herniate upon palpation. Although the clinical picture is characteristic, a definitive diagnosis requires histological confirmation in order to differentiate this disorder from other conditions of elastolysis, such as cutis laxa and mid-dermal elastolysis. Little is known concerning the pathogenesis of this condition, and treatment attempts have been both diverse and unsuccessful. This article will review a case of generalized anetoderma in a patient with secondary syphilis after being treated with intravenous penicillin, along with a concise literature review.A 43-year-old Caucasian man with a history of human immunodeficiency virus (HIV), receiving highly active antiretroviral therapy (HAART) for seven years, presented to the outpatient dermatology clinic with complaints of “spots” all over his body. Two months prior, during an admission to the hospital for the treatment of secondary syphilis with intravenous penicillin, his skin lesions began to appear. They started on his left arm as red, itchy “bumps” that quickly spread in size and number to affect his trunk and extremities. The patient reported that his scalp, palms, soles, face, and mucous membranes were spared. He also denied any prior skin complaints, change in medications, drug abuse, or recent sexual activity. Physical examination demonstrated numerous skin-colored to white macules with a wrinkled surface on the trunk and extremities (Figure 1). The macules followed skin-cleavage lines and protruded upon forward bending, but disappeared upon lying down (Figures 2A and 2B). Open in a separate windowFigure 1Up-close view of multiple, well-circumscribed, wrinkled-appearing macules following skin cleavage lines on the left trunk and extremity.Open in a separate windowOpen in a separate windowFigures 2A and 2BA) Herniation of macules upon forward bending and B) their disappearance upon lying flat.An atrophic macule from the right arm was biopsied. Hematoxylin and eosin (H&E) staining demonstrated a perivascular lymphoplasmacytic infiltrate (Figures 3A and ​and3B).3B). Verhoeff Van-Gieson (EVG) elastin stain showed absent and fragmented elastic fibers in the dermis, and a Masson''s trichrome stain demonstrated no abnormalities in collagen or smooth muscle (Figures 4A and 4B). Immunohistochemical staining for antitreponemal antibodies was negative. Serum testing for antinuclear antibodies, thyroid autoantibodies, thyroid function, antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibody), and complement levels were all within normal limits. Rapid plasma reagin (RPR) was positive at a titer of 1:240 despite intravenous penicillin treatment in the recent past. Thus, the clinical findings in conjunction with microscopic analysis and negative blood examination confirmed a diagnosis of anetoderma in the setting of HIV and secondary syphilis. Open in a separate windowFigure 3ASuperficial perivascular dermatitis, seen at low magnification (H&E, 2x)Open in a separate windowFigure 3BLymphoplasmacytic infiltrate seen in the papillary dermis (H&E, 40x)Open in a separate windowOpen in a separate windowFigures 4A and 4BSpecial staining shows A) an absence of elastic fibers in the papillary and mid-reticular dermis (EVG, 2x) and B) no collagen (blue) or smooth muscle (red) abnormalities (Trichrome, 2x).     

Missing Self–Induced Microvascular Rejection of Kidney Allografts: A Population-Based Study

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Human Kidney Spheroids and Monolayers Provide Insights into SARS-CoV-2 Renal Interactions

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Inhibiting Focal Adhesion Kinase Ameliorates Cyst Development in Polycystin-1–Deficient Polycystic Kidney Disease in Animal Model

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Muir-Torre Syndrome: A Case Report

Muir-Torre syndrome is an autosomal dominant genodermatosis associated with sebaceous neoplasms and visceral malignancies. Characteristic sebaceous neoplasms include sebaceous adenoma, sebaceous carcinoma, sebaceoma, and keratoacanthoma with sebaceous differentiation. The most common visceral malignancies are colorectal and genitourinary tumors. Investigations into the molecular genetics of Muir-Torre Syndrome have revealed an association with germ-line mutations in hMSH2 and hMLH1 genes. The clinical and histological features of a patient with Muir-Torre syndrome who had two sebaceous adenomas, multiple basal cell carcinomas, and frontal bossing in association with colon cancer are presented in this report.An 81-year-old male presented with an asymptomatic tan papule on his left jaw line. The patient stated that the lesion had been present for several months. The patient had a history of numerous basal cell carcinomas (BCC), actinic keratoses (AKs), and one prior biopsy-proven sebaceous adenoma. In addition to multiple skin cancers, the patient had a history of coronary artery bypass graft surgery and a partial colectomy for colon carcinoma. The patient was unable to provide a family history as he was adopted.Physical examination of the patient’s left jaw line revealed a 0.4cm tan-to-yellow papule on a markedly erythematous base. An incidental finding of frontal bossing was also noted (Figure 1). A shave biopsy showed numerous pyriform sebaceous lobules associated with an increased number of darker, immature sebocytes at their periphery. The lobules were oriented perpendicularly to the skin surface (Figure 2 and ​and33).Open in a separate windowFigure 1Patient at presentation. An incidental finding of an unusually prominent forehead and heavier-than-normal brow ridge known as frontal bossing.Open in a separate windowFigure 2H&E, original magnification x100. Shave biopsy of the left jaw line showing numerous pyriform sebaceous lobules associated with an increased number of immature sebocytes at their periphery. The lobules are oriented perpendicularly to the skin surface.Open in a separate windowFigure 3H&E, original magnification x200. Shave biopsy of sebaceous adenoma showing increased number of darker germinative cells at the periphery of the sebaceous lobules.The patient’s sebaceous adenomas and BCCs were excised, and he was treated with long-term fluorouracil 5% cream applied twice weekly to his face, chest, and back as prophylaxis for AKs and cutaneous malignancies. He had skin examinations every three months as well as annual screening for internal malignancy. The patient developed more than 20 nodular and superficial BCCs on his face and trunk during his lifetime, which were treated by excision or electrodessication and curettage. He did not, however, develop metastases from his colon carcinoma or any additional internal malignancies. The patient died at age 87 of causes unrelated to his skin or colon carcinomas.