Melissa M. Mauskar

MYC amplification in angiosarcomas arising in the setting of chronic lymphedema of morbid obesity

Angiosarcoma is a malignancy of vascular endothelial cells which may arise secondarily as a complication of lymphedema, including chronic lymphedema of morbid obesity. Amplifications in MYC are frequently present in secondary angiosarcoma (arising in irradiated sites and chronic lymphedema) and less frequently in primary cutaneous angiosarcoma.

Fever and a Pustular Rash

A previously healthy man in his 40s presented with 4 days of fever and a rash. He noticed multiple acneiform papules and pustules around the neckline that progressed to involve the face, torso, and limbs. He took no prescribed medications but had taken an ibuprofen pill a week prior for a mild headache; his symptoms started 3 days after that. He had no sick contacts or recent travel and reported no use of alcohol or tobacco. On examination, he was febrile (38.9°C) and tachycardic but normotensive. Clusters of pustules, approximately 1 mm in diameter, were present over an erythematous base overlying the face, neck, chest, back, and arms (Figure). Laboratory analysis revealed values within normal ranges for hemoglobin (13.1 g/dL) and platelet count (186 ×109/L); an elevated white blood cell count (22.3 x109/L; 82% neutrophils, 9% lymphocytes, 5% eosinophils, 4% monocytes); erythrocyte sedimentation rate of 58 mm/h (reference range, 0-20); C-reactive protein level of 232 mg/L (2210 nmol/L) (reference, <5 mg/dL [48 nmol/L]); and normal renal and hepatic function. Gram stain of the pustule fluid revealed no organisms, and cultures of the blood and pustule fluid were without growth. A punch biopsy of the skin lesions showed subcorneal pustules containing neutrophils and eosinophils, with epidermal spongiosis. Figure. Face, neck, and chest of the patient.

Acute syndrome of pan-epidermolysis and thrombotic storm arising in a patient with systemic lupus erythematosus

ASAP: acute syndrome of apoptotic panepidermolysis IV: intravenous IVIg: intravenous immunoglobulin SLE: systemic lupus erythematosus TEN: toxic epidermal necrolysis INTRODUCTION The vast variety of clinical presentations in systemic lupus erythematosus (SLE) contributes to its reputation as one of the great masqueraders. Here we report a case of a 39-year-old woman with a history of SLE in whom a fatal combination of acute syndrome of apoptotic pan-epidermolysis (ASAP) and thrombotic storm developed. Differentiating between drug-induced toxic epidermal necrolysis (TEN) in an SLE patient and TEN-like SLE is challenging; SLE patients are often on chronic medications that increase their risk of drug-induced TEN, and TEN is known to occur more commonly in patients with connective tissue disease. Additionally, both can have near-identical histologic findings, with full-thickness epithelial necrosis along with a sparse superficial lymphocytic infiltrate. The unifying concept of ASAP was first introduced in 2004 to describe the clinical constellation that encompasses the many life-threatening clinical situations presenting with massive epidermal cleavage owing to hyperacute apoptotic injury. Thrombotic storm is a clinical presentation characterized by the rapid onset of multiple thromboembolic occlusions affecting multiple vascular beds including the arterial, venous, and microvascular circulation. Typically, there is an inciting trigger such as infection, trauma, or pregnancy, and around half of patients are positive for antiphospholipid antibodies. These patients have a progressive unexplained recurrence of thrombi that are refractory to acute therapy; therefore, management in the acute setting is extremely difficult.

Urticaria, Urticarial Vasculitis, Angioedema, and Related Diseases

Purpose of ReviewDermatologists are frequently called to evaluate patients with urticaria in the emergency room, urgent care clinics, and hospitals. Many acute urticarial eruptions will resolve without long-term sequelae; however, there are red flags that clinicians must be aware of.Recent FindingsFirst-line treatment for acute urticaria is regular dose H1 antagonists; however, the dose can be increased up to fourfold for refractory disease. Short courses of corticosteroids should be avoided as rebound urticaria is common upon discontinuation. Urticarial vasculitis presents with persistent, atypical urticaria, burning, and residual lesions. The most common extra-cutaneous manifestation of urticarial vasculitis is musculoskeletal involvement. Schnitzler syndrome is a rare, severe condition but new evidence provides promise for use of biologic therapies.SummaryAcute spontaneous urticarial eruptions are commonly encountered in hospitalized patients. This review provides readers with the tools needed to delineate benign eruptions from more concerning conditions.

Cutaneous extranodal NK/T-cell lymphoma mimicking cellulitis in a HIV positive patient without lymphopenia.

We present the case of a 28‐year‐old male with a history of human immunodeficiency virus (HIV) with a 1‐month history of a steadily enlarging, firm painful lesion on the right posterior shoulder. The patient was initially treated for cellulitis given his clinical picture. Histopathologic examination revealed an angiocentric and dermal proliferation of markedly atypical lymphoid cells with numerous mitoses and apoptotic bodies along with broad zones of necrosis. Biopsy revealed the presentation to be consistent with NK/T‐cell lymphoma. The cutaneous lesions from NK/T‐cell lymphoma can often be initially mistaken for cellulitis, therefore this malignancy should be included on the differential in a patient HIV/acquired immune deficiency syndrome (AIDS).

An exophytic nodule on the scalp

From So Fund Conf Corre D Awoman in her 50s presented for evaluation of a nodule on her scalp that had grown gradually over the past 7 years. She denied any pain, pruritus, or other symptoms associated with the lesion. She denied any bleeding or ulceration of the nodule. She had no significant medical history and no personal or family history of skin cancer or other malignancy. She denied any family history of similar lesions. The physical examination revealed a 2-cm exophytic, spherical, pink nodule with a smooth surface on the left frontal scalp (Fig 1). The nodule was firm and nontender. There were no other lesions on the scalp. A biopsy specimen of the nodule was submitted for histopathologic evaluation (Figs 2 and 3).