Unilateral multisegmental morphea.

There are 5 subtypes of morphea that are based on disease distribution and presentation, including plaque, localized, generalized, linear, and deep morphea. We report a case of a young patient with morphea lesions in scattered locations confined to 1 side of the body, which we have termed unilateral multisegmental morphea.

Erythematous plaques on the face, trunk, and upper arms.

An 18-year-old woman presented with a one-year history of erythematous to slightly violaceous indurated papules and plaques on the face, earlobes, neck, upper arms, chest, and upper back.

Denuded congenital lesions: recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (Hallopeau-Siemens type) (RDEB-HS) is a rare severe mechanobullous disorder resulting from a defect in collagen VII. Patients with RDEB-HS present with generalized blistering and denudation of the skin at birth and have mucosal involvement. The repeated blistering leads to scarring, which may be deforming and result in serious complications.

Nonmelanoma skin cancer of the head and neck II: surgical treatment and reconstruction.

Surgical treatment of non-melanoma skin cancers (NMSC) is most often straight forward and can be performed as an office-based procedure by non-surgically trained professionals. However, the cases that present to an Otolaryngology practice typically will involve difficult-to-treat lesions around the face and lesions that necessitate an in depth knowledge of how to balance the need for adequate surgical margins with the reconstructive contraints imposed by aesthetic subunits of the face. This article discusses modern concepts of surgical margins for NMSC, including the Moh's micrographic surgical technique, and then reviews the most common reconstructive strategies for difficult-to-treat areas on the face.

Nonmelanoma skin cancer of the head and neck I: histopathology and clinical behavior.

Non-Melanoma skin cancer (NMSC) is the most commonly encountered malignancy in almost every area of practice, but the cases that present to an Otolaryngology practice will be advanced in nature. The major subtypes of NMSC include basal cell carcinoma, squamous cell carcinoma, dermatofibrosarcoma protuberans, merkel cell carcinoma, and adnexal malignancies. In this review, we present the epidemiology, histology, clinical presentation and management of these major subtypes.

Bortezomib-induced histiocytoid Sweet syndrome.

Bortezomib, a proteasome inhibitor approved for the treatment of multiple myeloma, has been reported to be associated with Sweet syndrome. However, careful review of the histopathology of the first reported case and our case revealed similar histologic and immunohistochemical findings (a mononuclear dermal infiltrate) and not the usual neutrophilic infiltrate of Sweet syndrome. We suggest that the dermatitis induced by bortezomib is best classified as "histiocytoid Sweet syndrome.

Novel cryoprecipitate for wound healing and skin grafts in rats.

The authors sought to evaluate the ability of locally administered enhanced cryoprecipitate (eCryo) to improve the wound healing of split thickness skin grafts (STSG) and their donor sites. An STSG (5 x 5 cm) was harvested on the back of 30 rats and divided into four areas that were then treated in one of the following groups: A: 'standard' dressing without STSG; B: eCryo without STSG; C: eCryo with STSG coverage and D: STSG alone. Macroscopic and histological assessments (histomorphometric grading scale and cellular composition) were evaluated at days 7, 14, 21 and 28 for wound healing.

Langerhans cell histiocytosis mimicking malignant melanoma: a diagnostic pitfall.

Langerhans cell histiocytosis (LCH), especially with an involvement limited to the skin, is a rare entity in adults. In formulating a differential diagnosis of a solitary skin lesion, LCH is rarely considered. Morphologically, cells seen in LCH can mimic those seen in a melanocytic tumor; moreover, they both show S-100 protein reactivity with immunoperoxidase staining.

Multicentric reticulohistiocytosis associated with Burkitt lymphoma and adenocarcinoma.

Multicentric reticulohistiocytosis (MRH) is a rare disease of unknown etiology characterized by cutaneous nodules and destructive, sometimes crippling, polyarthritis. The diagnosis is confirmed by histopathologic features of the cutaneous nodules or synovial tissue, including an infiltrate composed of histiocytes, many of them multinucleate, with a ground glass appearance. Multicentric reticulohistiocytosis has been associated with a number of chronic conditions and various malignancies.

Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States.

Background: There are large discrepancies in reported mortality for bullous pemphigoid (BP).

Objective: We sought to determine the mortality of a large cohort of patients with BP and compare this with age-matched control subjects.

Methods: Data were collected on 223 patients with a new diagnosis of BP between 1998 and 2003 through our cutaneous immunofluorescence laboratory databases.

Pruritic bluish-black subcutaneous papules on the chest.

Steatocystoma multiplex (SM) is characterized by multiple dermal cysts involving the pilosebaceous glands. Although most presenting cases are sporadic, there is a rare familial syndrome involving a mutation in keratin 17 (K17) that is inherited in an autosomal dominant fashion. SM often presents concomitantly with eruptive vellus hair cysts (EHVS) and pachyonychia congenital type 2 (PC-2).

Confluent and reticulate papillomatosis.

A 24-year-old man had an asymptomatic rash on his chest and arms for one year. On his chest, there were brown confluent plaques in a reticulate pattern. A scraping for fungus was negative.

Infantile systemic hyalinosis: Case report and review of the literature.

Infantile systemic hyalinosis (ISH) is a rare, progressive autosomal recessive disease, which is usually fatal by the age of 2 years. Clinical onset typically occurs within the first few weeks of life. The disease is characterized by joint contractures, osteopenia, failure to thrive, gingival hypertrophy, diarrhea, protein-losing enteropathy, and frequent infections.

Sweet's syndrome in chronic lymphocytic leukemia associated with neutropenic fever and granulocyte colony stimulation factor.

Granulocyte colony stimulation factor (G-CSF) is commonly used in the treatment of chemotherapy-induced myelosuppression. We report the case of a 62-year-old man with chronic lymphocytic leukemia who presented with neutropenic fever and sepsis. After treatment with G-CSF he developed Sweet's syndrome.

Primary neuroendocrine carcinoma of the vagina with Merkel cell carcinoma phenotype.

We describe a case of primary neuroendocrine carcinoma arising from the anterior vaginal wall of a 67-year-old woman. Primary neuroendocrine carcinoma of the vagina is a rare entity with only 25 previously reported cases in the literature. In previous reports, these tumors have not been distinguished from primary neuroendocrine carcinoma of the skin (Merkel cell carcinoma).

Impact of thorough block sampling in the histologic evaluation of melanomas.

Objective: To determine if changes in histologic parameters obtained from intermittent sampling of the entire block correlated with differences in prognosis and management.

Design: Prospective analysis of skin biopsy specimens.

Setting: Skin pathology laboratory.

Interstitial granulomatous dermatitis secondary to soy.

A healthy 58-year-old woman developed an asymptomatic papular eruption of the neck, cheek, abdomen, arms, and flexures. There was an 8-year history of the lesions, which had erupted when the patient started a strict vegetarian diet. Lesions lasted 3 to 5 days, cleared without scarring, and were associated with burning and increased tearing of the eyes.

A variant of nephrogenic fibrosing dermopathy with osteoclast-like giant cells: a syndrome of dysregulated matrix remodeling?

Nephrogenic fibrosing dermopathy (NFD) is a disorder characterized by dramatic thickening and hardening of skin in the extremities and trunk, which occurs in individuals on dialysis for renal disease. The pathophysiology is unknown. Increased transforming growth factor-beta (TGF-beta) and collagen deposition have been reported in a small group of patients studied by Jimenez et al.