Metastatic vulvovaginal Crohn disease in the setting of well-controlled intestinal disease.

The cutaneous manifestations of Crohn disease (CD) are varied and include pyoderma gangrenosum, erythema nodosum, and metastatic CD (MCD). The latter is defined as the occurrence of granulomatous lesions at a skin site distant from the gastrointestinal tract. Metastatic CD involving the vulva and perineum is rare and thus often is difficult to diagnose. It may precede, coincide with, or develop after the initial diagnosis of intestinal disease. A variety of clinical presentations have been described, including widespread nonspecific pain and swelling, erythematous papules and plaques, and nonhealing ulcers. The diagnosis often is delayed because of a low index of suspicion brought about by the rarity of the disease and its diverse and confusing manifestations. A skin biopsy usually confirms the diagnosis by revealing noncaseating granulomas in the dermis. Multiple oral and parenteral therapies are available, with surgical intervention reserved for resistant cases. We present a case of vulvovaginal MCD in the setting of well-controlled intestinal disease.

Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoproliferative disorder: Report of a case and review of the literature.

Primary cutaneous small/medium-sized T-cell lymphoma (PCSM-TCL), which was included in the World Health Organization - European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for cutaneous lymphomas as a provisional entity in 2008, has recently been reclassified as primary cutaneous small/medium-sized T-cell lymphoproliferative disorder (PCSM-TCLPD) because of its indolent behavior and uncertain malignant potential. Treatment with local therapies is usually curative, although there have been reports of aggressive, systemic disease. This spectrum of disease behavior evokes the consideration that this entity may actually be multiple diseases with a shared clinicopathologic features rather than a singular disease process with a variety of behaviors. PCSM-TCLPD retained its designation as a provisional entity under the updated WHO-EORTC guidelines; however, additional cases of PCSM-TCLPD are needed to shed more light on this rare disorder.

Evaluation and Management of the Hair Loss Patient in the Primary Care Setting.

Alopecias represent a heterogeneous group of disorders with different etiologies, presentations, and treatment options. The evaluation of the hair loss patient includes a comprehensive clinical history and physical examination; appropriate laboratory testing; and if indicated, a scalp biopsy. Treatment methods vary depending on the type of alopecia, and include watchful waiting, topical and systemic formulations, surgery, and treatment of any underlying or associated conditions. Referral to a dermatologist is helpful in diagnostically challenging and difficult to treat cases. Alopecia can cause emotional, mental, and social distress to patients. Early diagnosis and timely institution of appropriate treatment are helpful and comforting to those affected by this disease.

Skin Biopsy, the Allergists' Tool: How to Interpret a Report.

Inflammatory dermatoses are frequently encountered by the allergist, and histologic evaluation achieved through skin biopsy can be of tremendous value clinically. There is no substitute for a thorough history and physical exam; however, the skin biopsy is a simple, in-office procedure with little risk of complication that can provide invaluable information when a diagnosis is uncertain. Histopathologically, many inflammatory eruptions can look similar or overlap, but information provided by the dermatopathologist can help the clinician render or refine the clinical diagnosis and guide management. This review will discuss descriptive elements contained in the pathology report to provide a framework that can be used by the allergist to comfortably and confidently diagnose inflammatory dermatologic conditions.

Angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE), also named epithelioid hemangioma (EH), is an inflamed vascular tumefaction of uncertain pathogenesis, characterized by proliferation of histiocytoid endothelial cells with prominent lymphocytic and eosinophilic infiltration. Although considered a benign condition, it may recur in up to one-third of cases in the absence of complete surgical excision. The pathogenesis of ALHE/EH is still controversial. However, reaction to trauma and arteriovenous shunting are considered relevant. Histologically, ALHE/EH may be differentiated from other vascular neoplasms by its several unique characteristics including prominent proliferation of plump endothelial cells, and accompanying eosinophilic and lymphocytic inflammation, often with formation of lymphoid follicles. Surgery is the mainstay of treatment and various other treatment strategies have been used with varying results.

New world cutaneous leishmaniasis: obstacles in initiating treatment with sodium stibogluconate in 2 travelers from Texas.

New World cutaneous leishmaniasis (CL) is considered in the differential diagnosis for patients with nonhealing ulcers and a history of travel to high-risk areas. For patients at risk for progression to mucocutaneous leishmaniasis, first-line treatment in the United States entails the use of sodium stibogluconate (SSG), which is obtained from the Centers for Disease Control and Prevention (CDC) under an investigational drug protocol. We report 2 cases of New World CL in travelers to endemic areas who were diagnosed and treated with SSG. These cases demonstrate the logistics of coordinating with the CDC to definitively diagnose New World CL and initiate the necessary treatment.

New side effect of TNF-alpha inhibitors: morphea.

An otherwise healthy 45-year-old man with a 3-year history of poorly controlled psoriasis (no arthritis) was treated with etanercept 50-mg subcutaneous injections twice weekly for 3 months and then once weekly. Alternative treatment options were either unavailable (long commute for phototherapy) or contraindicated (history of alcohol abuse). The patient initially tolerated etanercept well with significant clinical improvement and had an uneventful course; however, approximately 18 months after initiating therapy, he abruptly developed dusky, indurated, and tender plaques on his abdomen and thighs at the sites of etanercept injections (Figure 1). There was also diffuse woody induration involving his flanks and back where injections had not been performed. His only recent prior exposure to an injectable medication was rabies vaccination in his arm 1 year earlier. The patient denied any systemic symptoms. Upon noting these findings, etanercept was immediately discontinued. Biopsy of an indurated plaque on his right lower abdomen revealed a superficial and deep perivascular lymphoplasmacytic inflammatory infiltrate in a background of thickened and hyalinized collagen fibers with notable loss of perieccrine fat (Figure 2). These features were most consistent with the inflammatory stage of morphea. Further work-up revealed a negative antinuclear antibody, anti-double-stranded DNA, anti-Scl-70, and anti-centromere. Borrelia titers were not obtained. The differential diagnosis included scleredema and scleromyxedema; serum and urine protein electrophoresis were within normal limits. The sites were treated with intralesional corticosteroids. During the next 3 months, there was minimal progression of disease although the plaques of morphea had not yet resolved.

MART-1 is a reliable melanocytic marker in lichen planus-like keratosis: a study on 70 cases.

Recent studies have proposed that MART-1 may falsely stain clusters of intraepidermal nonmelanocytic cells in lichenoid dermatitides. This may become an issue especially in isolated lesions of lichen planus-like keratosis (LPLK), a condition also known as benign lichenoid keratosis, and one that is often mistaken clinically for a malignant cutaneous neoplasm. LPLKs are known to exhibit basal epidermal pseudonests, mimicking a regressing melanocytic lesion histologically, and often may prompt the pathologist to obtain a MART-1 stain. If MART-1 is falsely positive, it may seal an incorrect diagnosis. To determine whether or not pseudonests in LPLK decorated with MART-1, we reviewed 70 cases from our institution, stained them with MART-1 (Thermo Fisher-Lab Vision, Ab3 clone, 1:400 dilution, heat-induced epitope retrieval with 0.02M citrate buffer at pH 6.0), and evaluated them for the presence or absence of staining within pseudonests. Four cases demonstrated an occasional MART-1-positive junctional nest. In these cases, microphthalmia transcription factor was also positive, confirming a true melanocytic origin. None of the other cases showed a MART-1 pattern that would have been suspicious for a melanocytic lesion. We propose that this discrepancy between our study and prior ones may be explained by differences in staining protocols or by a very low incidence of non-specific staining. Our study suggests that MART-1 is a useful marker in differentiating melanocytic nests from pseudonests in LPLK.

Sclerosing spindle cell rhabdomyosarcoma in an adult: report of a new case and review of the literature.

Sclerosing spindle cell rhabdomyosarcoma (SSRMS) is a newly recognized entity in adults. The authors report a new case of SSRMS in a 31-year-old woman who presented with a large right leg mass. Biopsy revealed a malignant spindle cell neoplasm with focal sclerotic areas. A diagnosis of monophasic synovial sarcoma was favored initially. The tumor cells in the resection specimen were positive for myosin, myogenin, and MyoD1. Fluorescence in situ hybridization performed on the resection specimen showed no evidence of SYT gene rearrangement in the neoplastic cells, ruling out monophasic synovial sarcoma. A diagnosis of SSRMS was established. The patient succumbed to widely metastatic disease 16 months after initial diagnosis. This case highlights the utility of skeletal muscle markers and cytogenetic testing in distinguishing SSRMS from its mimic, monophasic synovial sarcoma. It is hoped that this case will expand the literature on adult SSRMS and help clinicians and pathologists better understand this newly described entity.

Atopic dermatitis, patch testing, and house dust mites: a brief review.

The association between house dust mites (HDMs) and atopic dermatitis (AD) has long been a contested issue in both dermatology and allergy/immunology. As AD continues to burden millions of individuals and even whole societies, the commercial availability of a HDM allergen mix for epicutaneous testing has revived efforts to identify a definitive link between HDMs and AD. Conclusive evidence of a role for HDMs in the pathogenesis of AD would justify patch testing with mite allergens and would further the use of allergen-specific immunotherapy in the management of such patients; an absence of involvement would refocus our investigative efforts on other conspirators. We present a thorough review of the dissenting scientific evidence and expert opinions on this controversial topic. The association of HDMs with AD has remained elusive, and additional investigation is necessary to resolve this vexing riddle.

Atypical distinctive dermal clear cell mesenchymal neoplasm arising in the scalp.

Distinctive dermal clear cell mesenchymal neoplasm (DDCCMN) is a newly described entity characterized by a dermal proliferation of neoplastic cells with clear to reticulated cytoplasm and vesicular nuclei. Atypical features in the form of nuclear pleomorphism and mitoses may be found. The histogenesis of these neoplastic clear cells is not currently known but they are thought to be mesenchymal in origin. Immunohistochemically, they stain positively for NKI-C3 and negatively for melanocytic, epithelial and lymphoid markers. All five cases originally reported by Lazar and Fletcher occurred in the lower extremities. We report herein a case of DDCCMN with atypical features arising in the scalp.

Metastatic rhabdoid melanoma: report of a case with a comparative review of the literature.

Melanoma has a propensity to mimic the morphologic appearances of a wide variety of epithelial and soft tissue neoplasms. Although rarely encountered, these unusual morphologic variants of melanoma are often treacherous and may be difficult and challenging to diagnose. Thus, recognition of these variants is of paramount importance in the evaluation of any kind of epithelial or soft tissue neoplasm. Rhabdoid melanoma is one such example of these melanoma variants. Very few cases of rhabdoid melanoma have been reported, most of which were found in metastatic lesions. We wish to expand this rather sparse body of literature by reporting a case of metastatic rhabdoid melanoma arising subcutaneously in the right thigh of a 64-year-old man with a remote history of primary toe melanoma. Moreover, we present a comparative review of the clinical, histologic, immunohistochemical and ultrastructural features of previously reported cases. We found significant heterogeneity in the extent of rhabdoid change, expression of melanocytic and non-melanocytic markers and ultrastructural filamentous patterns in the cases we reviewed. It is our hope that this case report and literature review will help pathologists in the evaluation of neoplasms with rhabdoid morphology.

Identification and expression profiling of a human C-type lectin, structurally homologous to mouse dectin-2.

A number of C-type lectins on antigen-presenting cells play an important role in regulating innate immunity. Previously, we identified the mouse C-type lectins (dectin-1, and dectin-2) and human DECTIN-1. To identify human DECTIN-2, we employed degenerative polymerase chain reaction-based cDNA cloning using RNA from human Langerhans cell (LC)-like dendritic cells (DCs). This process yielded a cDNA encoding a C-type lectin with 66.5% amino acid sequence homology to mouse dectin-2, the same gene reported by Kanazawa et al. (J Invest Dermatol 2004: 122: 1522-1524) using the disparate approach of analyzing coding sequences in chromosome 12. Similar to their findings, we found gene expression in lung, spleen, and lymph node. Among resting leukocytes, it was expressed at highest levels by CD14+ monocytes, at lower levels by CD19+ B cells, and not at all by CD4+ T cells. Activation of CD19+ B cells with pokeweed mitogen down-regulated gene expression, whereas expression in CD4+ T cells was induced by Con A. Among our novel findings are an alternatively spliced transcript lacking exon 2, expression in bone marrow and tonsil, expression in CD8+ T cells that is abrogated following activation with phytohemagglutinin, restricted expression to CD1a+ LC within epidermis, and preferential expression by plasmacytoid (rather than myeloid) DC. Finally, we found that treatment with interleukin-4 (IL-4), IL-10, or UVB down regulated gene expression in CD14+ monocytes, whereas granulocyte-macrophage colony-stimulating factor, transforming growth factor-beta1, or tumor necrosis factor-alpha treatment up-regulated it. Our findings may form the basis for understanding the function of human DECTIN-2 in innate immunity.