Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis.

Clinical case of the month. 3-year-old boy presenting with a hand rash. Juvenile dermatomyositis.

Of the diseases within the spectrum of the juvenile idiopathic inflammatory myopathies, juvenile dermatomyositis (JDM) is the most common. As the name implies, JDM affects the muscles and skin most commonly, but can involve other organ systems as well. Dermatologic manifestations often precede other signs and symptoms by months or even years and frequently are the primary reason the patient seeks medical attention. In the case presented here, a 3-year-old boy initially developed a hand rash that brought him to his primary care physician. By the time muscle weakness had developed, the patient had already been evaluated for dermatomyositis and therapy had been initiated. An understanding of these early clinical findings will enable physicians to make a timely diagnosis and commence therapy promptly in order to prevent life-threatening sequelae of the disease.

Clinical case of the month. A young man with a persistent skin eruption. Mycosis fungoides.

There are many types of skin disease that fit into the classification of cutaneous lymphoma, but mycosis fungoides is by far the most common of this group. It is a non-Hodgkin's lymphoma of T-cell origin that presents in the skin. Mycosis fungoides often evolves for years without a specific diagnosis because it can present as an eczematous or psoriasiform eruption. Patients identified in the early stages and treated appropriately have a normal life expectancy.

Cutaneous sarcoidosis presenting as leonine facies.

Cutaneous sarcoidosis often masquerades as many other disease entities. We describe the case of a 56-year-old African American man with a 1-year history of progressively enlarging nodules and plaques of the face resulting in a leonine appearance and madarosis. The diagnosis of cutaneous sarcoidosis was made after skin biopsy results revealed noncaseating granulomas without evidence of foreign body, mycobacteria, or deep fungal infection. A thorough systemic workup was void of other comorbidities. The reports of tumoral sarcoidosis or sarcoidosis presenting with leonine facies are rare, and those cases that have been reported have been linked to other systemic findings.