Uzma Farooq

Frozen-section biopsy analysis for acute invasive fungal rhinosinusitis

OBJECTIVE: To evaluate sensitivity and specificity of frozen-section biopsy in the diagnosis of acute invasive fungal rhinosinusitis (AIFRS). STUDY DESIGN AND SETTING: Retrospective chart review of all patients treated for AIFRS at the University of Miami between 1993 and 2005. Twenty patients with the clinical diagnosis of AIFRS were identified. Histopathological data were collected to evaluate the use of frozen-section biopsy to diagnose the disease. RESULTS: Permanent pathology sections were positive for AIFRS in all specimens. Frozen-section biopsies were positive for fungal features in all but five cases (four with Mucor and one with Aspergillus). High positive predictive values were found for both fungal types and for both sets of patients (bedside and intraoperative). CONCLUSION: Frozen-section biopsy is a useful tool for rapid and effective diagnosis in patients with suspected AIFRS. A positive frozen-section result is highly predictive of invasive disease and merits prompt surgical intervention.

Reactivation of dormant cutaneous Leishmania infection in a kidney transplant patient

Background:  Leishmaniasis is an infection caused by a protozoan parasite belonging to genus Leishmania and transmitted by the Phlebotomus sandfly. Clinical presentations of infection include visceral, cutaneous, and mucocutaneous forms. Leishmaniasis is endemic in Africa, Asia, Europe, South America, and southern part of North America. This infection is extremely rare in the US and is mostly found among travelers coming from endemic areas. Cases of cutaneous and visceral leishmaniasis have been reported in organ transplant recipients in endemic areas.

Management of advanced laryngeal and hypopharyngeal plexiform neurofibroma in adults.

Laryngeal neurofibromas are rare. The plexiform subtype is less common and often presents during childhood in association with neurofibromatosis type I. Because it is unencapsulated and more aggressive, plexiform neurofibroma presents a management dilemma. Imaging, particularly magnetic resonance imaging, can aid in diagnosing neurofibroma, differentiating the nonplexiform and plexiform subtypes, and planning the subsequent operative approach. The importance of conservative surgery to relieve symptoms, but preserve laryngopharyngeal function, and close follow-up are stressed. We report 2 adult cases of plexiform neurofibroma involving the larynx and hypopharynx and discuss the controversies in management.

Coma blisters in two postoperative patients.

Coma blisters are self-limited cutaneous bullae that occur in the setting of loss of consciousness because of a drug, illness, or accident, with the most common settings being barbiturate overdose and neurological disorders. The etiology behind coma blisters is poorly understood and is not related to underlying infections or autoimmune conditions. The clinical presentation consists of bullae, erosions, and violaceous plaques usually involving sites of pressure. The skin lesions usually occur within 48-72 hours of the start of a coma and resolve within 2-4 weeks. We present one case of a 5-month-old infant with severe valvular disease who required surgical repair. He was placed on extra corporeal membrane oxygenation and developed multiple tense coma blisters during the course of therapy. Skin biopsy revealed a noninflammatory subepidermal blister with necrosis of the overlying epidermis and necrosis of the eccrine ducts. We also present a second case of an 18-year-old female patient who underwent surgical resection of a benign mandibular tumor. She subsequently developed bullae on both arms 4 days after surgery. The skin biopsy showed a necrotic epidermis, a subepidermal blister, and diffuse necrosis of the eccrine coils.

Cutaneous endometriosis: diagnostic immunohistochemistry and clinicopathologic correlation

Endometriosis is a condition where endometrial glands and stroma are ectopically located in sites other than the uterine cavity. Cutaneous endometriosis is very rare, representing approximately 1.1% of cases of extrapelvic endometriosis. We report a case of a 44‐year‐old female with no prior surgical history who presented with multiple tan brown periumbilical nodules. Histopathological examination revealed multiple glandular structures in the dermis with surrounding stroma. Immunohistochemistry cinches the diagnosis, as CD10, estrogen receptor and progesterone receptor are strongly positive in our case. The mainstay of treatment of cutaneous endometriosis is surgical excision of the lesion.

Cutaneous aspergillosis masquerading as Sweet's syndrome in a patient with acute myelogenous leukemia.

To the Editor, Aspergillosis may present as primary cutaneous lesions or as secondary cutaneous emboli.1 The former typically appear as plaques with black crust, while the latter consists of necrotic papules and plaques, often with ulceration and rapid progression. Pulmonary disease with multiple, bilateral cavitating infiltrates and nodules may appear in those with disseminated cutaneous emboli.1 In hosts with neutropenia or an immunocompromised status, such as our patient, the fungus can disseminate widely. Here we present a case of a 68-year-old male with newly diagnosed acute myelogenous leukemia (AML) who developed multiple pink Sweet’s-like papules; the tissue stained positive with Gomori methenamine silver (GMS) stain, consistent with aspergillosis. A 68-year-old male with newly diagnosed AML status post-induction chemotherapy presented with a 2-day history of multiple, painful skin lesions that were indurated and erythematous. The lesions were distributed on his right finger, right neck, right foot and left cheek and spread to his left leg, left neck and occiput. Over the next few days, he developed left eye pain, periorbital swelling, decreased visual acuity and left-sided nasal congestion. Associated symptoms included recent neutropenic febrile episodes, night sweats, chills, shortness of breath and progressive fatigue. Physical examination at this time revealed rounded, well-defined pink papules with central pustules that were tender to palpation (Fig. 1). Two lesions had a blackish, eschar-like appearance in the center surrounded by an erythematous base (Fig. 2) and one lesion contained a central Fig. 1. Discrete erythematous papule and macule on the left posterior thigh.

Extramammary Paget disease of the vulva with underlying mammary-like lobular carcinoma: a case report and review of the literature.

Extramammary Paget disease of the vulva accounts for 1%–2% of the neoplasms of the anogenital area. Very rarely, extramammary Paget disease of the vulva has been associated with an underlying mammary-like carcinoma, usually ductal, extremely rarely mixed ductal and lobular. We report the case of a 60-year-old female with a recurrent extramammary Paget disease of the vulva. Pathological examination of the wide excision of the vulva revealed an extramammary Paget disease with an underlying invasive carcinoma composed of medium size cells organized in single files, a morphology similar to that of an invasive lobular breast carcinoma. Immunohistochemical staining showed a comparable profile in the Paget cells and in the invasive tumoral cells: CEA and CK7 positivity; GCDFP-15, ER focal positivity. E-cadherin and HER2 were diffusely positive. S100 and CK20 were negative. HER2-CISH was amplified. The diagnosis of extramammary Paget disease of the vulva with an underlying mammary-like lobular carcinoma was made. Despite the characteristic lobular features, the immunohistochemical profile differs from the typical profile of a lobular carcinoma of the breast. The implications in term of prognostic and therapeutic significance need to be further studied.

Post-kala-azar dermal leishmaniasis in HIV-infected patients with AIDS: a report of two cases diagnosed in the USA

Post‐kala‐azar dermal leishmaniasis (PKDL) is an uncommon complication of visceral leishmaniasis (VL) but is emerging as an increasingly frequent and serious complication of acquired immunodeficiency syndrome (AIDS). It manifests as a macular, morbilliform, or nodular eruption in a patient who has recovered from VL.

Elafin: A possible new biomarker and immunohistochemical stain for pre-engraftment syndrome

To the Editor: Engraftment syndrome (ES) and pre-ES (PES) are poorly defined, yet they are increasingly reported complications of hematopoietic stem cell transplantation. ES is usually diagnosed in an autologous, rather than allogeneic, context. The cause is likely multifactorial involving a complex interaction among cytokines, complement, and other cellular and molecular elements of the immune system, such as T cells, monocytes, and other effector cells. The presentation usually involves an unexplained fever and an erythematous rash mimicking graftversus-host disease (GVHD). Suspicious clinical findings include: diarrhea, pulmonary infiltrates, hypoxemia, renal and hepatic dysfunction, weight gain, and transient encephalopathy. High numbers of hematopoietic cells infused, patient age, conditioning regimen, rate of engraftment, and underlying disease are risk factors. Elafin is an endogenous protein secreted in response to interleukin 1 and tumor necrosis factoralfa that is predominantly expressed in epithelial tissue and is an important biomarker that identifies GVHD. Distinction of ES and GVHD is a common dilemma after bone-marrow transplantation. Clinically, the rash of ES after autologous stem cell transplantation mimics that of acute GVHD. In the setting of GVHD, there are elevated plasma concentrations of elafin and there is also immunohistochemical overexpression of elafin in skin biopsy specimens. A 69-year-old man with a history of recurrent primary central nervous system lymphoma presented with fever and a rash after undergoing autologous peripheral blood stem cell transplantation days earlier. On physical examination there were diffuse, erythematous macules and patches on the upper aspect of the chest, abdomen, and back (Fig 1). Associated findings included bilateral lower extremity edema, left lower lobe consolidation, and bilateral pleural effusions. The presence of all 3 major criteria (ie, noninfectious fever, erythrodermatous rash involving[25% of body surface area, and diffuse pulmonary infiltrates) established a diagnosis within the PES/ES spectrum. The patient had not reached an absolute neutrophil count of 500 cells/ L at the time of symptomatic presentation, thus he was given the diagnosis of PES. The patient was treated with intravenous dexamethasone with resolution of his eruption. Two skin specimens were taken from the abdomen and upper aspect of back. Histologic evaluation showed vacuolar alteration of keratinocytes at the dermoepidermal junction along with a sparse superficial lymphocytic infiltrate and necrotic keratinocytes at the basal layer (Fig 2, A). Immunohistochemical staining with elafin was performed, demonstrating significant cytoplasmic staining in the superficial keratinocytes with focal full-thickness epidermal staining (Fig 2, B), along with perifollicular staining. The specificity and sensitivity of elafin revealed that it was the single best discriminator compared with other biomarkers for diagnosing GVHD in patients with bone-marrow transplantation and a rash, and its presence correlated with disease severity. These findings and histopathologic similarities suggest an important clinicopathologic overlap among PES, ES, and GVHD. In summary, the identification of increased elafin expression may establish a novel finding that may contribute important information and facilitate future therapeutic decisions for patients with ES or PES. We would like to conclude that because GVHD, PES, and ES stain with elafin in tissue with a similar staining pattern, elafin may not be helpful in differentiating among these entities. However, in the setting of GVHD, elafin is elevated in the serum, a finding yet to be reported, to our knowledge, in the PES and ES spectrum. Further evaluation is warranted to determine the diagnostic potential of this new biomarker and stain.

Clear cell hidradenocarcinoma with helpful immunohistochemistry: a case report

Clear cell hidradenocarcinomas are rare, malignant sweat gland neoplasms. This tumor usually arises de novo but can also arise from its benign and more common counterpart, nodular hidradenoma. These tumors have been described with many names, including nodular hidradenocarcinoma, malignant acrospiroma, malignant clear cell hidradenoma, solid-cystic adenocarcinoma, malignant clear cell myoepithelioma, and clear cell eccrine carcinoma. We would like to report a case of clear cell hidradenocarcinoma with a helpful immunohistochemical profile to aid in making the diagnosis.