En coup de sabre.

En coup de sabre (ECDS) is a rare form of localized scleroderma that typically manifests in children and women. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution on the frontoparietal scalp with surrounding scarring alopecia. Many patients have comorbid central nervous system involvement in addition to the cutaneous findings. En coup de sabre is a rare entity that should be delineated from Parry-Romberg syndrome, as both entities share some common features and may coexist. Corticosteroids remain the treatment of choice, but other modalities such as methotrexate, excimer laser, and grafting have been used with varying success. We report a case of an elderly woman who presented with an asymptomatic alopecic plaque consistent with ECDS.

Panniculitis, pancreatitis, and polyarthritis: a rare clinical syndrome.

A rare triad composed of lobular panniculitis in the setting of pancreatitis and polyarthritis is termed panniculitis, pancreatitis, and polyarthritis (PPP) syndrome. Pancreatic panniculitis is a rare form of subcutaneous fat necrosis associated with underlying pancreatic disease. We describe a case of PPP syndrome and review the relevant literature associated with this rare clinical syndrome. Despite numerous adjuvant therapies, definitive treatment of PPP syndrome requires correction of the underlying pancreatic disease.

Granulomatous pigmented purpuric dermatosis.

Granulomatous pigmented purpuric dermatosis (GPPD) is a rare histologic variant of pigmented purpuric dermatosis (PPD). It includes classic histology changes of PPD with superimposed granulomas. This variant is thought to be associated with hyperlipidemia and is found predominantly in individuals in the Far East; however, a review of the literature that included 26 documented cases of GPPD revealed these associations might be becoming less clear. We report a case of GPPD in an elderly white man who had an eruption involving the majority of the lower legs.

Subintimal hemorrhage in an unruptured superior cerebellar artery aneurysm: prelude to rupture: case report.

OBJECTIVE AND IMPORTANCE: An intracranial aneurysm that hemorrhages into the subintimal layer of the blood vessel is a rare clinical entity. Such a hemorrhage is distinct from dissecting aneurysms or pseudoaneurysms of the cerebral circulation and may represent an aneurysm in the process of rupturing. The authors report their experience in the management of a patient who presented with a subintimal hemorrhage of a superior cerebellar aneurysm. CLINICAL PRESENTATION: A 54-year-old man presented with a progressively worsening headache over the course of 3 days. A noncontrast computed tomographic scan did not demonstrate subarachnoid hemorrhage but was suggestive of a posterior circulation aneurysm. INTERVENTION: In the absence of subarachnoid hemorrhage on the computed tomographic scan, negative findings for xanthochromia in the cerebrospinal fluid, and no evidence of subarachnoid blood on magnetic resonance imaging, a conventional four-vessel cerebral angiogram was performed, which revealed a right superior cerebellar artery aneurysm. Further evaluation on magnetic resonance imaging demonstrated abnormal signal conforming to the aneurysm wall, suggesting the presence of a contained subintimal hemorrhage. The patient underwent a right frontotemporal craniotomy (half-and-half approach) for clip ligation of the superior cerebellar artery aneurysm. CONCLUSION: Subintimal hemorrhage of an aneurysm in the absence of subarachnoid hemorrhage may be conceptually considered an aneurysm in the process of rupturing. Thus, aneurysms found in the acute setting may benefit from further workup, such as magnetic resonance imaging or highly selective angiographic evaluation of the aneurysm itself. Timely surgical intervention may prevent otherwise imminent rupture into the subarachnoid space.