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.

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."

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. Transmission electron microscopy is currently the gold standard for diagnosis of RDEB-HS.

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. Dermatologic manifestations include thickened skin, hyperpigmentation, perianal nodules, and facial papules. Histopathology shows hyaline deposits in the dermis and visceral organs. We describe a patient with ISH confirmed by clinical and histopathologic findings, as well as DNA sequence analysis, which revealed a novel homozygous T118K mutation in the CMG2 gene.

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. We report a case of MRH in a patient with Burkitt lymphoma and metastatic adenocarcinoma of the gastrointestinal tract.

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. A biopsy showed papillomatosis, orthokeratosis, and melanin pigment at the basal layer of the epidermis. The patient was started on a six-week course of minocycline twice/day. Six weeks later, the patient was completely clear of the rash. Confluent and reticulate papillomatosis is an uncommon dermatosis that tends to occur on the chest. The pathogenesis is unknown. Minocycline has been reported to work well in the treatment of this dermatosis.

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). We report a sporadic case of SM in a 21-year-old man.

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. All wound beds as well as STSGs healed well without any complications. Eighty per cent of the STSG showed a histological graft take of >75% after 28 days. There were no statistically significant differences of macroscopic or histological results between the groups at any time point. Preparation of eCryo is easy and effective. Its use as an adhesive for STSGs is safe and shows similar results as controls. The theoretical benefits of eCryo did not show significant differences. Possible reasons as well as important findings for future research on wound healing are discussed.

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). The tumor was composed of cells that showed neuroendocrine-type nuclear features with hyperchromasia, nuclear molding, occasional small nucleoli, and a chromatin pattern that was finely granular. The tumor cells were positive for cytokeratin 20 (CK20), neuron specific enolase, pancytokeratin, epithelial membrane antigen, and chromogranin A expression. Ki-67, a marker of proliferation, was also positive in>90% of cells. The tumor cells showed intense expression of Bcl-2 oncoprotein and mild to moderate expression of c-KIT. Synaptophysin, neurofilament, CD45, CD56, CD10, S-100, HMB-45, cytokeratin 7, and thyroid transcription factor 1 were negative. This pattern of staining is consistent with a Merkel cell carcinoma. This is the first report of a primary neuroendocrine carcinoma of the vagina with a Merkel cell phenotype. Previous studies have not distinguished primary neuroendocrine carcinoma of the vagina from Merkel cell carcinoma of the skin. Positive expression of CK20 in primary small cell carcinoma of the vagina might represent a Merkel cell carcinoma subtype of this tumor.

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. Patients One hundred consecutive patients with an unequivocal diagnosis of melanoma. Interventions Two initial slides were prepared from serial sections of 5-mum thickness. When evaluation of the initial slide revealed melanoma, 5 additional slides were obtained by sectioning at levels through the entire block. Breslow depth, Clark level, ulceration, tumor infiltrating lymphocytes, vascular invasion, regression, presence of a precursor lesion, and histologic type of melanoma for the first slide and the additional 6 slides were analyzed and compared. RESULTS: Review of the additional 6 slides from level sectioning revealed a greater maximum tumor thickness than was evident from the original slide in 43% of the cases. In 10 of these cases, the new maximum tumor thickness measurements changed the surgical management of the patients. Ulceration was observed in 6% of cases on the initial slides, and an additional 3% of lesions were found to have ulceration on levels. The level of invasion was deeper than originally found in 10% of the cases. CONCLUSIONS: Level sectioning through an entire block of a melanoma specimen provides additional information in the classification and management of melanomas. Extensive block sampling will result in more accurate information regarding histologic parameters of melanoma, but the yield must be balanced with the extra cost of materials, time, labor, and the potential disadvantage of not retaining tissue for future use.

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. The biopsy specimen showed an interstitial granulomatous dermatitis without vascular injury, collagen alteration, or mononuclear atypia. The eruption cleared when the patient omitted soy products from her diet. It subsequently recurred with intake of even minimal amounts of soy. Interstitial granulomatous dermatitis is a histologic pattern of inflammation that generates a broad differential diagnosis. No previous reports of interstitial granulomatous dermatitis related to soy products are available in the literature.

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. Sweet's syndrome is a rare disorder but has been associated with cancer recurrence as well as administration of G-CSF. We present clinical and pathologic images that highlight the salient features of this entity.