Intravesicular taxane-induced dermatotoxicity in a 78-year-old man with urothelial carcinoma and primary cutaneous anaplastic large cell lymphoma.

Patients treated with intravesical bacillus Calmette-Guérin therapy for urothelial carcinoma often become refractory and experience recurrent disease, thus necessitating alternative intravesical treatment modalities if the patient is to be spared the morbidities associated with radical cystectomy. Intravesical treatment with taxane-based chemotherapy, such as docetaxel, has gained traction in urologic oncology, proving to be an effective salvage therapy in such patients. Systemic taxane-based chemotherapeutic regimens have long been used in several advanced malignancies, and their systemic side-effects and associated histologic correlates have been extensively documented. In contrast to adverse effects associated with systemic administration, intravesical taxane administration has thus far proven to be well-tolerated, with little to no systemic absorption. To our knowledge, features of taxane-induced systemic effects have not been reported in this setting. Herein, we report a case of a patient with recurrent urothelial carcinoma treated with intravesical docetaxel, along with primary cutaneous anaplastic large cell lymphoma, who developed characteristic dermatotoxic histologic findings associated with intravenous taxane administration. As such histopathologic findings often represent close mimickers of neoplastic and infectious etiologies, knowledge of the potential for systemic manifestations of taxane therapy in patients treated topically may prevent potentially costly diagnostic pitfalls.

Delayed-Onset Sclerema Neonatorum in a Critically Ill Premature Infant.

Sclerema neonatorum is extremely rare in the 21st century. We report a premature infant managed in a neonatal intensive care unit with delayed development of sclerema neonatorum.

Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature.

Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder presenting in middle adulthood that nearly always affects the lungs and shows cutaneous involvement in up to 50% of cases. Skin lesions are present at the time of diagnosis in roughly one-third of patients and may precede the development of lung lesions in as many as 10-15%. Recognition by both the dermatologist and dermatopathologist is therefore crucial for early and accurate diagnosis. While skin involvement is grossly and microscopically diverse, the disease most commonly presents as erythematous subcutaneous and dermal nodules showing the classic histopathologic triad of transmural lymphocytic angiitis, atypical B-lymphocytes in a polymorphous T-cell background, and necrotic foci within lymphoid aggregates. We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature.

Disseminated Scopulariopsis-culture is required to distinguish from other disseminated mould infections.

Disseminated fungal infections are a major cause of mortality in severely immunocompromised bone marrow transplant (BMT) patients. Scopulariopsis is a soil saprophytic mould that is typically associated with onychomycosis and only rarely associated with disseminated infection with cutaneous findings. We describe a case of fatal disseminated Scopulariopsis infection in a 56-year-old neutropenic male with chronic myelogenous leukemia status post peripheral blood stem cell transplant that was clinically and histologically indistinguishable from disseminated Aspergillus, Fusarium or zygomycosis infection. Distinguishing the above listed fungi by tissue culture is crucial because disseminated Scopulariopsis is difficult to eradicate and associated with a high mortality rate in the immunocompromised BMT patient population.

Penicillamine-induced elastosis of the mucosal lip.

Long-term penicillamine therapy has been associated with alterations in dermal elastic tissue. Well-described associated dermatoses include pseudo-pseudoxanthoma elasticum, acquired cutis laxa, elastosis perforans serpiginosa, and anetoderma. Histologically, "lumpy-bumpy"" or "bramble-bush"" morphologic changes of elastic fibers in the dermis are characteristic. Previous reports of these findings in normal-appearing skin and internal organs suggest a systemic elastolytic process. Here we report an unusual case of penicillamine-induced elastosis affecting the mucosa of the lip with characteristic histologic features.

The utility of PU.1 as an immunohistochemical marker for histiocytic and dendritic lesions of the skin.

PU.1 is a transcription factor restricted to the hematopoietic system. It is expressed in myeloid lineage and B lymphocytes but is absent in mature T cells and nonhematopoietic cells. Among myeloid lineage-derived cells, PU.1 is overexpressed in monocytes, histiocytes, and dendritic cells. We evaluated PU.1 expression in 78 cases of primary skin neoplasms, including 9 reticulohistiocytomas, 9 Langerhans cell histiocytoses, 7 juvenile xanthogranulomas, 9 fibrous papules, 8 dermatofibromas, 12 dermatofibrosarcoma protuberans, 9 Spitz nevi, and 15 malignant melanomas. Strong nuclear staining for PU.1 was seen in all cases of histiocyte and dendritic cell origin, including 9/9 reticulohistiocytomas, 9/9 Langerhans cell histiocytoses, and 7/7 juvenile xanthogranulomas. No staining for PU.1 was seen in any studied cases of fibrous papules, dermatofibrosarcoma protuberans, dermatofibromas, Spitz nevi, or malignant melanomas. This study indicates that PU.1 is a valuable immunohistochemical marker for identifying cutaneous histiocyte- and dendritic cell-derived lesions. PU.1 staining is easily interpreted due to the sharp nuclear staining as compared with the irregular and often variable cytoplasmic staining seen with CD68.

Severe erythema multiforme responding to interferon alfa.

Erythema multiforme (EM) is a targetoid eruption with interface pathology often triggered by a hypersensitivity response to a variety of infections, most commonly herpes simplex virus. Hepatitis C virus is rarely associated with EM. We present a 37-year-old man with an 8-year history of severe EM unresponsive to valacyclovir, acitretin, and cyclosporin, and marginally responsive to high-dose prednisone. The eruption had cleared 6 years previously during treatment with interferon for his concurrent hepatitis C virus. Although his viral titer was undetectable, we initiated therapy with interferon and ribavirin. The patient responded dramatically within 2 months and remained clear of EM after 1 year of continued interferon therapy. This is the third case reported in the world literature documenting a response of EM to interferon, and the first case in which hepatitis C virus was undetectable in serum prior to interferon therapy.

Langerhans cell histiocytosis presenting as blueberry muffin baby.

Blueberry muffin baby is a descriptive term for purpuric lesions reflective of extramedullary hematopoeisis. The clinical lesions most commonly result from intrauterine infections, such as rubella and cytomegalovirus, and less commonly with malignancy and hematologic disorders. Langerhans cell histiocytosis is a clonal proliferation of dendritic histiocytes in the skin. This has very rarely been associated with a blueberry muffin presentation. We report the case of a newborn with typical lesions of cutaneous hematopoiesis and lytic bone lesions related to Langerhans cell histiocytosis. At birth, approximately 40 2 mm to 5 mm purpuric, nonblanching macules were scattered on the trunk, extremities, and soles of our patient. Laboratory studies were unremarkable and cultures were negative. Skin biopsy showed a dermal proliferation of histiocytes staining positive for S100 and Cd1a. Pediatric bone surveys, chest radiographs, and computed tomography scans of the head were normal. Six months later, the skin lesions had resolved, but radiographs revealed lytic bone lesions of the right tibia, right ilium, and left pubic ramus, consistent with skeletal Langerhans cell histiocytosis.

Nephrogenic fibrosing dermopathy with systemic involvement.

BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). Nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. Autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.

Viral exanthems.

Viral exanthems are mostly associated with self-limited diseases. However, in some cases diagnosis of an exanthem may be crucial to patients and their contacts. Certain exanthems have fairly characteristic morphology, but in many cases an accurate diagnosis cannot be made on the basis of morphology alone. Historical factors may be helpful when evaluating these patients, specifically their disease contacts, immunization record, previous exanthematous illnesses, and associated prodromal symptoms. Some illnesses are seasonal and this knowledge may be useful. This manuscript reviews a number of common childhood exanthems. We included the most common viral exanthems encountered by primary-care physicians and dermatologists.

Benign cutaneous Degos disease in a 16-year-old girl.

Degos disease is a rare disorder, characterized by the development of typical atrophic porcelain-white macules on the trunk and extremities. It most commonly occurs in middle-aged Caucasian adults and eventuates in fatality in the majority of patients. A minority of reported patients have a more benign course and a favorable outcome. Degos disease is extremely rare in children. We report Degos disease in a teenager with an extended survival and the absence of internal involvement. We discuss the concept of benign cutaneous Degos and review the 34 patients (30 adults and four children) identified in the world literature with skin biopsy and at least 1 year of follow-up; the average age was 37.6 years; 26 were female and eight were male. As opposed to the malignant form of Degos disease, benign cutaneous Degos is more commonly reported in women (3:1). No laboratory abnormalities have been predictive of a benign versus malignant course. We also discuss the 24 reported instances of Degos disease (malignant and benign) in the pediatric population, of which 14 (58%) were fatal, with death occurring on average 3.6 years after diagnosis.