bluuebunny/crossref_metadata_2025 huggingface dataset:
Interstitial Granulomatous Dermatitis with Plaques
Authors: Farmer AJ , Wang LC , Medenica MM
Published: August 2010

A 74‐year‐old woman presented with an asymptomatic, 20 by 10 cm, erythematous, indurated plaque on the sacrum with similar lesions in the folds of both legs. Laboratory studies revealed an elevated ANA and ESR but normal rheumatoid factor, TSH, Lyme, anti‐DNA, and SSA/SSB antibodies. Biopsies from the sacrum and left leg showed a perivascular and interstitial infiltrate of foamy histiocytes, epitheliod cells, and multinucleated giant cells, as well as lymphocytes and eosinophils in the entire dermis. The patient was diagnosed with interstitial granulomatous dermatitis (IGD) with plaques. The lesions resolved spontaneously. IGD with plaques is a rare but benign disorder of uncertain etiology. Its characteristic histopathology and clinical appearance distinguish it from other granulomatous dermatitis.
Incidental Acantholytic Dyskeratosis and Hailey‐Hailey Like Acantholysis
Authors: Gaertner E. , Groo S.
Published: August 2010

Materials and Methods: 247 consecutive skin specimens covering a three‐month period (1/04–3/04) were reviewed to identify incidental foci of Hailey‐Hailey (HH) like acantholysis and focal acantholytic dyskeratosis (FAD). Cases in which these patterns comprised the primary process were excluded. All cases were formalin fixed, paraffin embedded, and hematoxylin‐eosin stained. Results: Six cases were identified: three shave biopsies (chest, back, face), two excisions (back, face), and one punch biopsy (scalp). This represented 2.4% of total reviewed. Four specimens had a HH like pattern (1.6%); two had FAD (0.8%). The average patient age was 68 years (range 41–86). Four patients were male; two were female. Three cases were associated with epidermal neoplasia: basal cell carcinoma (HH), actinic keratosis (HH), and malignant melanoma in situ (HH). Two were associated with significant inflammation: seborrheic keratosis (HH) and bullous lichen planus (FAD). One biopsy was of lichen simplex chronicus (FAD). The average diameter of acantholysis was 0.3 mm. Four cases showed associated prominent solar elastosis. Discussion: Incidental acantholysis with FAD and HH like patterns is uncommon. This has previously been postulated to reflect possible pre‐neoplastic epidermal damage. The discussed cases show an association with epidermal neoplasia, solar change, and inflammation.
Spitzoid Melanomas in Children, Like Spitz Nevi, Lack Common Activating Mutations in BRAF and NRAS
Authors: Gill M. , Cohen J. , Renwick N. , Mones J. , Silvers D. N. , Celebi J. T.
Published: August 2010

Spitzoid melanoma in children is a rare tumor that can mimic Spitz nevus both clinically and histologically. Recently, dysregulation of the RAS‐RAF‐MAPK signalling pathway was reported in 89% of sporadic melanomas due to activating mutations in the BRAF or NRAS gene. BRAF mutations are found in 53–80% of primary melanomas and 70–90% of common melanocytic nevi, whereas none are present in Spitz nevi. NRAS mutations occur in 5–37% of melanomas and 9–56% of common melanocytic nevi. The mutational status of NRAS in Spitz nevi is unknown. This is the first study analyzing the mutational profile of BRAF and NRAS in Spitzoid melanomas occurring in prepubertal children (10 years of age or younger). Primary tumor cells were isolated via laser capture microdissection from 10 typical Spitz nevi and 10 metastatic Spitzoid melanomas. Exons 11 and 15 of BRAF and exons 2 and 3 of NRAS were amplified by PCR and directly sequenced. Common activating mutations were not identified in BRAF or NRAS. Absence of the V599E hot‐spot mutation in BRAF was further confirmed by allele‐specific PCR. We conclude that Spitzoid melanomas in children share clinical, histological and genetic similarities to Spitz nevi, and are genetically distinct from other melanoma subtypes.
Ultrasmall Dysplastic Nevi: Atypia can be Seen in Moles Less than 2 MM in Diameter
Authors: González‐Serva Aldo , Muñoz‐Garcia Liliana
Published: August 2010

The recognized breadth of dysplastic nevi has quickly shrunk. We now present very small dysplastic nevi below any described breadth. A cluster of mostly punched‐out nevi derived from a single clinician who recognizes as ‘atypical’ even very small lesions was also assigned histologic dysplasia on architectural and cytologic basis. From 536 junctional nevi with dysplasia ranging from mild to moderate, 136 (25.3%) were 1 mm. in vitro or less while 371 (69.2%) were 1.1–2 mm. Among 155 compound nevi with the same range of dysplasia, 19 (12.2%) were 1 mm or less and 95 (61.2%) were 1.1–2 mm. Moderate or, less so, severe atypia was even seen in more than half of either 18 junctional and 18 compound nevi of dysplastic type between 2.1 and 3 mm. While the above threshold for dysplasia may not be similar to the one than many dermatopathologists could use, it is still evident that sliding down our higher‐grade nevi to meet milder degrees of perceived atypia in a more conservative diagnostic scale, the breadth of these nevi still remains minuscule in general. In sum, we adhere to the idea that dysplastic nevi are not only common but can be extremely small: “bonsai nevi.”
Immunohistochemical Characterization of Hyaline Globules in Kaposi’s Sarcoma
Authors: Gupta N. , Inamdar K. , Chaffins M. , Ma C. , Ormsby A. , Lee M.
Published: August 2010

Hyaline globules (HG) are frequently found in neoplastic cells of Kaposi’s sarcoma (KS). Presence of HG, characteristic of but not specific of KS, has been considered to represent erythrophagocytosis. In order to further characterize the nature of HG, we studied 10 cases of cutaneous KS with distinct HG using immunohistochemistry for hemoglobin A, Kappa and Lambda. All KS cases were of nodular type. Also included were 3 cases of glomeruloid hemangioma with similar HG in endothelial cells for the comparision. In selected cases electron microscopic examination was performed. PAS stain with diastase digestion was also done in all cases. Results: HG were diastase resistant and PAS positive. All HG in neoplastic cells of KS and endothelial cells of GH were negative for hemoglobin A. There was variable staining for light chains: focal but strong positivity for kappa, weak and variable staining for Lambda. Some HG was positive for both kappa and lambda. Electron microscopic study demonstrated HG as homogeneous electron dense inclusions with internal small vacuoles, whorled or granular materials. Conclusions: All HG in neoplastic cells and endothelial cells of GH represent phagolysosomes. Negative stain for hemoglobin A in KS and GH do not support the inclusions being fragmented RBC’s.
Aberrant Glycosylation and its Correlation with Expression of GNTV and Matriptase in Malignant Melanoma
Authors: Handerson T. , Miyoshi E. , Ueda M. , Taniguchi N. , Pawelek J.
Published: August 2010

Acetylglucosoaminyltransferase V (GNTV), an enzyme responsible for branching of asparagine‐linked oligosaccharides is associated with tumor invasion and metastasis. Beta1–6 branched oligosaccharides, detected with the lectin, LPHA, are associated with tumor progression in human breast and colon cancer. They were found to be common to 22 types of human cancers, and were particularly expressed in metastases. Matriptase is a proteolytic inducer of both the HGF/cmet and plasminogen activator pathways, highly associated with metastasis. A potential prometastatic effect of GNTV was found to be due to modification of active matriptase by adding beta1–6 GlcNAc branching. We investigated expression of LPHA, matriptase and GNTV in pathology specimens of primary invasive and metastatic malignant melanomas. Sequential sections were stained with anti‐GnT‐V, anti‐matriptase, or biotinylated LPHA. Our results showed variable staining patterns, from involving only small regions, to encompassing the entire tumor. The expression of all three markers was closely associated, suggesting that metastatic pathways might have been activated in the staining areas of these tumors. To our knowledge, this is the first report of matriptase expression in malignant melanoma, as well as the first demonstration of an association between matriptase, GNTV and beta 1,6‐branched oligosaccharides in vivo.
Aggressive Peripheral T‐cell Lymphoma Presenting as Cutaneous Nodules with a Dense Infiltrate of Eosinophils
Authors: High W.A. , Houpt K.R. , Hoang M.P. , Cockerell C.J. , Kroft S.H.
Published: August 2010

A 65 year‐old, well‐appearing and asymptomatic man presented with a small non‐healing ulcer on the right palm present for 6 weeks. He reported a 15 year history of hand and foot “eczema” treated with topical steroids. Six months earlier his right 4th toe had been amputated for “infection”, but additional information was not immediately forthcoming. Punch biopsy from the palm demonstrated superficial ulceration, dermal eosinophils and possible cytoplasmic inclusions within keratinocytes, leading to suspicion of parapox infection. Over the ensuing two months his condition deteriorated markedly, and he developed multiple fungated and ulcerated cutaneous nodules. Incisional biopsy of the nodule on the right palm revealed a dense dermal inflammatory infiltrate with a predominance of eosinophils, and a perivascular infiltrate of medium to large lymphocytes with irregular nuclei and mitoses. These atypical lymphocytes were positive for CD3, but were negative for CD4, CD8, CD30, CD56, TIA‐1 and EBER in‐situ hybridization. TCR‐gamma gene rearrangement revealed a clonal population. Retrospective analysis of the amputated toe demonstrated similar features. He declined conventional treatment, and has been reluctant to pursue follow‐up. Our case highlights the fulminant course and dense mixed inflammatory cell infiltrate with eosinophils often present in peripheral T‐cell lymphoma that may complicate diagnosis.
Lipedematous Alopecia: Could it Sometimes be an Unusual Sequela of Discoid Lupus Erythematosus?
Authors: High W.A. , Hoang M.P.
Published: August 2010

Lipedematous alopecia is a rare entity of uncertain etiology characterized by a diffusely thickened and boggy scalp, with varying degrees of alopecia. Six of eight cases in the literature have occurred in African American women. Classic histopathologic findings include increased subcutaneous tissue, varying degrees of inflammation and fibrosis, and an absence of significant mucin deposition. Herein, we report an additional case of lipedematous alopecia occurring in an African American woman who also demonstrated clinical, historical, and histopathologic findings reminiscent of discoid lupus erythematosus. Explicit observation of such an occurrence has not been made previously, although hyperkeratosis, follicular plugging, mild perivascular or periadnexal infiltrates, and fibrosing alopecia have been reported in lipedematous scalp, possibly the residua of other cases with associated discoid lupus‐like features. For further comparison, we contrast our case with a second patient with lipedematous scalp, which maintained histological similarities to lipedematous alopecia, but was lacked any significant hair loss. It is possible that some cases lipedematous alopecia may represent an unusual sequela of discoid lupus erythematosus. Nevertheless, we cannot exclude the possibility of two diseases occurring independently, nor that early changes of lipedematous alopecia might share histopathological and/or clinical findings with discoid lupus erythematosus.
Cutaneous Manifestations of Juvenile Hyaline Fibromatosis
Authors: Hilliard N.J. , Hammers Y. , Martin E. , Howard P. , Omura E.M. , Kelly D.R.
Published: August 2010

An 11‐year‐old adopted male presented with deforming scalp and bilateral posterior auricular nodules, joint contractures, and gingival hypertrophy. Histopathologic examination of the skin nodules revealed a normal epidermis with areas of extracellular eosinophilic ground substance surrounding benign spindle‐shaped (myo)fibroblasts in the dermis and subcutis, alternating with hyalinized, acellular zones. The spindle cells did not show cytologic atypia. A diagnosis of juvenile hyaline fibromatosis (JHF) was given. The patient had been seen multiple times in the past for similar recurring, disfiguring dermal and subcutaneous nodules. The patient had a history of osteolytic lesions of the distal radius and ulna and hyaline deposition involving the sigmoid colon and rectum. JHF is a rare autosomal recessive hyalinosis with mutations of the Capillary Morphogenesis Gene‐2 (CMG‐2) at chromosome 4q21, a region that encodes a binding protein that concatenates capillary collagen IV to laminin. Other genetic abnormalities include mutations in dermal collagen (types I and III). Disease onset occurs early in life. Organ involvement is an ominous sign usually resulting in death during infancy or early childhood. Surgery is the only presently available treatment.
Acid‐Fast (Ziehl‐Neelsen) Staining Facilitates Follicular Counting in Horizontally‐Sectioned Scalp Biopsies.
Authors: Hoss D. , Berke A. , Murphy M. , Kerr P. , Grant‐Kels J.
Published: August 2010

Transverse sectioning of scalp biopsies was first proposed by Headington in 1984. Horizontal sections allow easy analysis of follicular density, accurate determination of hair shaft diameter, and quantification of the anagen:telogen ratio. The terminal to vellus hair (T:V) ratio is an important parameter in the differential diagnosis of non‐scarring alopecias. A vellus hair is defined as having a cross‐sectional diameter of 0.03 mm or less, and a diameter not exceeding the thickness of its root sheath. It is tedious to tally the T:V ratio in a standard 4 mm punch biopsy because the entire biopsy can only be visualised using the 2x and 4x objectives. It is difficult to identify vellus hairs with this magnification, and one must switch between high and low power, often losing track of which hairs have already been counted. All hair shafts are strongly acid‐fast and stain bright red, making it easy to count terminal and vellus hairs with 2x and 4x magnification. We show acid‐fast (Ziehl‐Zeelsen) stained slides from various types of non‐scarring alopecias cut at the level of the infundibula, and contrast these to hematoxylin and eosin stained slides at the same level, to demonstrate how acid‐fast staining greatly facilitates follicular counting.
Benign Cutaneous Mixed Tumor with Combined Folliculosebaceous and Eccrine Differentiation
Authors: Iwenofu O.H. , Lamar W.L. , Clark S.H. , Crowson A.N.
Published: August 2010

Cutaneous adnexal tumors manifesting divergent differentiation are a rare but recognized subset of adnexal neoplasms. We present a 42‐year‐old woman with a cystic tumor of the skin above the left eyebrow. A biopsy revealed a tumor comprising eccrine tubules lined by a single row of columnar epithelium surrounded by a peripheral small cuboidal layer and containing, adhered to the internal cuticle, mucin‐like material. In some areas, the eccrine tubules were associated with sheets of small plasmacytoid myoepithelial cells embedded in a chondroid matrix. Elsewhere, there were cysts containing central keratinous debris associated with a peripheral basaloid epithelial proliferation showing the formation of buds mimicking abortive hair follicle structures. No polarizable terminal or vellus hairs were seen. These two elements were geographically separate in the biopsy but collided in the center of the tissue element. A diagnosis of benign cutaneous mixed tumor with combined folliculosebaceous and eccrine differentiation was rendered. Such tumors present a diagnostic challenge and are frequently confused with neoplasms including the benign mixed tumor (chondroid syringoma) and a variety of eccrine and folliculosebaceous neoplasms and harmatomata. This case illustrates the potential multilineage differentiation of pluripoential germinative cells of the cutaneous adnexae.
S‐100 Protein Positivity in Merkel Cell Carcinoma: An Uncommon Finding
Authors: Jensen S. , Stefanato C.M.
Published: August 2010

Merkel cell carcinoma (MCC) is a small round cell tumor which does not characteristically stain with S‐100 protein. We report a case of an 83 year‐old male with a two‐month history of an ulcerated nodule on the right arm. Histopathology revealed small blue round cells arrayed in a trabecular pattern and in diffuse sheets, with scattered mitoses and necrosis. The cells were negative for vimentin, positive for NSE, focally positive for high molecular weight cytokeratins and diffusely positive for low molecular weight cytokeratins, including CK20. The findings were diagnostic of MCC, however, foci of S‐100 protein positive cells were also present in the tumor. Upon reviewing cases from Skin Pathology Laboratory at BUSM received between 1/1/2000 to 7/30/2004, we documented a total of 34 cases diagnosed as MCC; 24 were stained with S‐100 protein and this case was the only one that stained positively. S‐100 protein positivity of MCC, while having been previously reported, is an uncommon finding. We report our case to stress the importance of morphological and immunoperioxidase features for the diagnosis of Merkel cell carcinoma, particularly in the exceptional cases which exhibit S‐100 protein positivity.
Chronology of Cellular Blue Nevus
Authors: Jin L. , Arai E. , Tsuchida T. , Ogawa F. , Mitsuhashi T. , Ban S. , Shimizu Y. , Hirose T. , Shimizu M.
Published: August 2010

Cellular blue nevus is composed of dendritic melanocytes, together with islands of epithelioid and plump spindle cells. We examined 58 cases of blue nevi. Fourteen cases were common blue nevi. The remaining 44 blue nevi were divided into three types according to its silhouettes. These were nodular (type 1, 18 cases), nodular with notch (type 2, 16 cases), and rounded pushing patterns (type 3, 10 cases). Well circumscribed top heavy nodule was observed in type 1, and it was limited to the dermis. Type 2 showed budding appearance into the subcutaneous tissue. Type 3 revealed bottom heavy with downward pushing margin. Both type 2 and type 3 were demonstrated biphasic patterns of epithelioid and spindle cells. Perineural distribution of melanocytes was also observed in type 2 and 3. The size of these types was 3.3 mm in type 1, 4.7 mm in type 2, and 7.4 mm in type 3. The thickness of these types was 1.6 mm in type 1, 2.5 mm in type 2, and 5.2 mm in type 3. Our results suggest that cellular blue nevi may be divided into three stages. Type 1 is its early stage, and may be misinterpreted as common blue nevi.
A Case of Malignant Melanoma with Multiple Primary Lesions
Authors: Kato M. , Taguchi M. , Kuramochi A. , Suzuki T. , Tsuchida T.
Published: August 2010

A 49‐year‐old Japanese male first visited our clinic at May 13th, 2003. A bean‐sized black macule had been noticed in the parietal region since childhood. The lesion had increased in size gradually and had become 30 mm in diameter. The lesion was diagnosed as malignant melanoma clinically and also dermoscopically. Sentinel node navigation using radioisotope revealed the metastases. We performed wide local excision and radical neck dissection, and the definite diagnosis of malignant melanoma and regional nodal metastases was made. TNM staging was stage?b, that is, pT4b, N2c, M0. The patient also had nine small black freckles on the trunk and extremities. All of them were excised, and eight lesions of them were diagnosed as malignant melanoma, because proliferation of atypical melanocytes and formation of abundant abnormal nests in epidermis were observed histopathologically. Chemotherapy was performed, but metastases of skin and visceral region were found in February, 2004. These lesions had put up strong resistance to chemotherapy and the patient died on June, 6th, 2004. Autopsy revealed multiple metastases including heart, kidneys, liver and large intestine and so on. No other family members suffered from malignant melanoma. The origin of this patient’s melanoma remained unclear.
Nodular Clear Cell Hidradenoma: A Case Report with Immunohistochemistry, Cytogenetics and Flow Cytometry
Authors: Khoury Thaer , Wallace Paul , Sait Sheila , Richard  , Cheney T.
Published: August 2010

Background: Nodular Hidradenoma (NH) is an uncommon neoplasm with controversial cell origin (myoepithelial, eccrine and more recently apocrine), and few cytogenetic and DNA flow Cytometry data. Material and Methods: We report a case of NH on the left ankle of a 77 year‐old man. The tumor was located subcutaneously and measured 2.5 × 2.0 cm, with no throbbing, tenderness, edema, or groin lymphadenopathy. A wide local excision was performed. Immunohistochemistry using monoclonal antibodies to Smooth Muscle Actin (SMA), S‐100 protein, p63 and Calponin were examined. A fresh central portion of the tumor was examined for karyotyping and DNA flow cytometry. Six months later the patient was healthy without local recurrence. Results: The tumor was positive for SMA, Calponin and p63 with focal staining for S‐100. The karyotype showed four aberrant, unrelated clones, t(1;8)(p22;p23), del(11)(q14q23), t(1;10)(p13;q22) and del (6)(q23q26). DNA flow cytometry and cell cycle analysis showed that the tumor is 100% diploid with 6.55% S‐phase. Conclusions: Expression of SMA, p63, Calponin, and S‐100, which are myoepithelial markers, support a myoepithelial origin of this tumor. The multiclonal pattern observed in this neoplasm seems to reflect cytogenetic divergence during neoplastic progression. DNA diploidy with a low S‐phase predicts a benign clinical course.
Epithelioid Cell Histiocytoma with Granular Cells
Authors: Lee J.
Published: August 2010

Epithelioid cell histiocytoma (ECH) is regarded as a variant of fibrous histiocytoma. It is not readily recognized as fibrous histiocytoma because of its unconventional histologic features and its resemblance to Spitz nevus. The ECH reported herein was from a 50‐year‐old man who presented with an asymptomatic, 1.5 cm, polypoid mass of unknown duration on his posterior shoulder. Histologic examination revealed epidermal collarette and dermal sheets of epithelioid cells with granular cytoplasm. Both fine and coarse granules were noted in the cytoplasm. They were PAS positive and diastase resistant. Many dilated vessels were also noted in the lesion. A panel of immunostains including S100, vimentin, CD68, and factor 13A was done. The lesional cells were reactive with vimentin, CD68, and factor 13A, and non‐reactive with S100. These results favored exclusion of Spitz nevus and granular cell tumor, and supported the histologic impression of ECH. Both fine and coarse cytoplasmic granules were reactive with CD68. The latter finding supports that the cytoplasmic granules in this ECH represent lysosomes as in granular cell tumors.
Spectrum of Histological Features in Infantile Digital Fibromatosis
Authors: Liang C. , Capaldi L. , Ney A. , Gray J. , Katarincic J. , Lapidus C. , Robinson‐Bostom L.
Published: August 2010

Infantile digital fibromatosis (IDF), also called Reye tumor or inclusion body fibromatosis, is a benign proliferation of myofibroblasts, with characteristic perinuclear eosinophilic cytoplasmic inclusion bodies. The inclusions stain pink with hematoxylin‐eosin, red with Masson’s trichrome, and purple with phosphotungstic acid‐hematoxylin. This intradermal neoplasm is formed by interlacing bundles of collagen and spindle cells. Positive immunohistochemical staining with vimentin, cytokeratin, desmin, and actin indicate myofibroblastic cell origin. Clinically, patients present at birth or infancy with asymptomatic smooth, flesh colored nodules, typically on lateral or dorsal digits. IDF is a benign neoplasm that may spontaneously regress and treatment is observation. Lesions recur in 60% of excisions; surgery is only recommended in cases of functional impairment or aggressive growth. We report three cases of IDF. The patients, ranging 8 months to 8 years old, presented with pink to flesh colored nodules on the feet and fingers. A spectrum of histological features was observed. All cases revealed dermal proliferation of spindle cells with blunt nuclei and eosinophilic intracytoplasmic inclusions. Pattern of spindle cells ranged from loose haphazard arrangement to well‐organized interlacing bundles. The quantity of eosinophilic inclusions varied. For diagnostic and treatment purposes, all three patients underwent excision, with subsequent recurrence in two cases.
Fatal Subcutaneous Panniculitis‐Like T‐Cell Lymphoma (Sptcl) with Interface Change and Dermal Mucin, A Dead‐Ringer for Lupus Erythematosus
Authors: Ma L. , Bandarchi B. , Glusac E. J.
Published: August 2010

We report a 48‐year‐old male who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3+CD4−CD8+/−CD56+. Monoclonal rearrangements of T‐cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is amongst rare reports of subcutaneous panniculitis‐like T‐cell Lymphoma (SPTCL) with systemic involvement.
Cutaneous T‐cell Lymphoma of 12 Years Duration Initially Presenting in a Two Year Old
Authors: Mark L. , Billings S.D.
Published: August 2010

A 14‐year‐old Caucasian girl presented with an eczematous patch on the left proximal inner arm of 12 years duration. She had previously been treated with emollients and topical steroids without complete resolution. The patient denied constitutional symptoms but did note eczematous patches on her buttocks of one‐year duration and new eczematous patches on her inner left thigh and right inner upper arm. Upon exam, all lesions were slightly scaly without induration. The primary lesion was a poikilodermatous 4 × 10 cm rough plaque. No lymphadenopathy was noted. A total of five lesional biopsies were obtained. All of the biopsies demonstrated a superficial infiltrate of atypical lymphocytes with cerebriform nuclei and prominent epidermotropism including the formation of Pautrier’s microabcesses. The diagnosis of mycosis fungoides was rendered. This case is significant for its onset at two years of age, the benignity of its course over a 12 year period and the long duration before definitive diagnosis was obtained. Mycosis fungoides needs to be considered in the differential diagnosis of recalcitrant eczematous disease even in young children.
Direct Immunofluorescence Results of a Case of Railway Track Dermatitis
Authors: Mecca P. , Carlson J. A.
Published: August 2010

A 40 year‐old female with sero‐negative arthritis presented with annular and cord‐like configurations (rope sign) of asymptomatic papules and plaques along her flanks, suspected to be granuloma annulare. Medications at the time of presentation included methotrexate, prednisone, Vioxx (rofecoxib), Arava (leflunomide) and Remicade (infliximab). High‐potency topical corticosteroids did not alleviate the eruption. Biopsy was performed demonstrating a deep dermal, interstitial and focally palisading granulomatous infiltrate with eosinophils. Dermal lymphangiectases were present, and the epidermis was unaffected. Direct immunofluorescence (DIF) examination showed deep dermal, interstitial IgG, C3, and IgM deposits coating collagen bundles centered in the granulomatous infliltrate and IgG antinuclear keratinocyte reactivity. Serologic tested also revealed anti‐nuclear antibodies. No other auto‐antibodies were detected. Based on these findings, this patient was diagnosed with “interstitial granulomatous dermatitis (IGD) with cords and arthritis”. Thirteen cases of IGD with cords were identified in a literature review of which 7/12 (58%) were sero‐(rheumatoid factor)‐negative, 3/5 (60%) were anti‐nuclear antibody positive, and 12/13 (92%) had arthritis. One theory of pathogenesis is that immune‐complex deposition in the dermis initiates a granulomatous reaction; DIF findings in this case support this proposed mechanism. IGD with cords is a singular clinicopathologic entity associated with sero‐negative, non‐erosive arthritis and antinuclear antibodies.
Subclinical Chronic Lymphocytic Leukemia Associated with a 13Q Deletion Presenting Initially in the Skin: A Propos of a Case.
Authors: Seilstad K , Khandelwal A , Gupta K , Byrd J , Magro C
Published: August 2010

Introduction: B‐cell chronic lymphocytic leukemia (B‐CLL) represents a low grade B cell lymphoproliferative disease with overlapping features with small lymphocytic lymphoma. The neoplastic cell is an autoreactive CD5 CD23 B lymphocyte. B‐CLL may involve the skin, typically in the context of known disease. We present a case of subclinical B‐CLL presenting initially in the skin. Results: A 73‐year‐old male developed a lesion on his right cheek in April 2003 compatible with basal cell carcinoma. The re‐excision specimen contained a well differentiated atypical lymphocytic infiltrate consistent with B‐CLL along with residual carcinoma. Subsequent laboratory studies revealed slight thrombocytopenia and mild peripheral blood lymphocytosis. A diagnosis was made of stage 0 CLL. Chromosomal studies on peripheral blood showed a deletion at 13q14.3. Conclusion: This case represents a cutaneous presentation of subclinical B‐CLL. There are two prior reports describing B‐CLL presenting initially in the skin. In one case the infiltrates were incidental on a re‐excision specimen. The second report suggests 16% of B‐CLL patients have cutaneous manifestations as the first sign of disease. Data indicates that 13q14 deletion in B‐CLL in early stage disease may portend a more aggressive course, possibly explaining its early presentation in the skin in our case.
Febrile Ulceronecrotic Mucha‐habermann Disease: a Rare, Severe Variant
Authors: Thompson Michele M. , White Clifton R.
Published: August 2010

A 56 year old Hispanic man presented with extensive ulcerative skin lesions, involving his lower trunk, groin and upper legs, severe pain and a temperature of 38.7 degrees Celsius. He was admitted to the Medical Intensive Care Unit for empiric intravenous antibiotics. Several biopsies were performed. In the following days his condition worsened and ulcerative lesions involved nearly all of his skin. Previous biopsies were consistent with pityriasis lichenoides et varioliformis acuta (PLEVA), however, neither this, nor others in the histological differential diagnosis, fit his severe and worsening clinical picture. Histology revealed vacuolar alteration with dyskeratotic keratinocytes and a superficial perivascular mixed infiltrate of lymphocytes and eosinophils. There was confluent parakeratosis containing neutrophils, and a diminished granular layer with pallor in the upper portion of the spinous layer. Immunofluorescence studies were negative. These findings were consistent with PLEVA. A clinical diagnosis of febrile ulceronecrotic Mucha‐Habermann disease was made. Febrile ulceronecrotic Mucha‐Habermann disease is a rare and severe variant of PLEVA characterised by high fever and papulonecrotic skin lesions. Twenty‐five cases have been previously reported. We present the clinical and histological findings in this unusual clinical presentation.
Über Schlundlähinung
Authors: ANTONI N. R. E.
Published: August 2010

Die Schlingmuskulatur ist in der Vagus‐kernsäule gesondert von den Kehlkopfmuskeln repräsentiert und ist bei bulbären Zerstörungen isoliert lädierbar.

Die Gehörigkeit des Musc, crico‐thyreoideus zur Pharynx‐muskulatur, indem ihre zentrale Repräsentation nach. experimentellen Ergebnissen kranial von der übrigen Kehlkopfmuskulatur und sogar vom Velum palati, zusammen mit der Schlundmuskulatur gelegen ist, kann vielleicht auch klinisch festgestellt werden, indeni bei die Recurrensmuskeln des Kehlkopfs verschonender, bulbärer Schlinglähmung Symptome gesehen wurden, die auf Lähmung dieses Muskets bezogen werden können (Kraftlosigkeit der Stimme, Kreuzung der Arywulste).

Einseitige Lähmung der Schlundmuskulatur ist sowohl bei zentralen (bulbären) wie peripherem (hohe Vagusunterbrechung) Angriff imstande, hochgradiges, anfangs sogar absolutes Schling‐unvermögen zu erzeugen.

Dieses Schlingunvermögen beruht nicht nur auf unvollständiger Obliteration des Pharynxlumens beim Schlingact, sondern auch und vor allem auf Fortbestehen des Tonus am Oesophagusmunde (Killian'schen Muskel), also auf einer Hemmungslahmung.

Versuche mit Adrenalin und Atropin ergeben, dass der Tonus am Oesophagusmund wahrscheinlich autonomer Herkunft ist.

Die Hemmung des Sphinetertonus im Sehlingact muss peripher angreifen, da die Lähmung dieser Hemmung auch bei extracranieller Vagusunterbrechung vorhanden ist.

Ist der Recurrens der hemmende Innervator des Oesophagus‐mundes, dann muss das Fehlen jeglicher Schlinglähmung bei Recurrenslähmung darauf bezogen werden, dass die postganglionären Sympathicusfasern auch im Recurrens verlaufen.

Wie die thoracale Vagusunterbrechung eine Erschlaffung der Speiseröhre zugleich mit Krampf der Cardia erzeugt (was von Kraus als die Ursache der spindelförmigen Ectasie betrachtet wird), so kann ein Krampf des Oesophagusmundes durch Lähmung der Schlundmuskelnerven verursacht sein.

Wie am unteren, so sind auch am oberen Oesophagusende leichtere Grade dieser Kombination von Schwäche mit Krampf (am unteren Ende seit langem als Cardiospasmus bekannt) bei nervösen Personen sehr häufig, was ja mit dem Wesen der Neurasthenie als reizbare Schwäche gut harmoniert. Das Globusgefühl (Globus hystericus), der m. E. durch Spasmus des Killianschen Muskels am Oesophagusmunde ausgelöst wird, ist meistens nur eine Teilerscheinung einer derartigen, mehr weniger ausgebreiteten visceralen Neurose, die seinerseits mehr weniger selbständig auf dem Boden der konstitutionellen, endocrinnervösen Schwäche oder als »Reflexneurose» durch organische Erkrankungen der Baucheingeweide ausgelöst wird.
De quelques essais d'administration de l'insuline par voie buccale spécialement au point de vue de Pâge des patients et de l‘état fonctionnel de Festomac.
Authors: Gjertz. Emil
Published: August 2010

Resumé

Les expériences ont porté sur 6 patients; 3 étaient des diabétiques dont l'âge variait de 9 à 21 ans; les 3 autres, âgés de 41 à 47 ans avaient présenté de l'achylie ou de l'hypochylie. Deux de ces derniers étainet diabétiques.

Entre ces deux groupes on constate une différence remarquable: alors que le taux du sucre du sang, trois heures (dans un cas, deux heures seulement) aprè l'ingestion d'insuline dans l'huile ou dans l'eau, s'était abaissé pour le premier groupe de 0.040 ou de 0.058 %, il ne s'abaissait pour le second que de 0.016 ou de 0.020 %.

Cette grosse différencene peut s'expliquer uniquement par une réaction différiences faites montrent que, chez les jeunes diabétiques par l'action de l'insuline s'est manifesté une diminution du sucre du san, alors qu'une diminution analogue ne se manifeste pas chez les malades d'âge moyen et dont la sécrétion gastrque est altérée.

Chez un des patients de chacun des deux groupes on a essayé de voir si des doses plus fortes d'insuline entraînent une diminution plus considérable du sucre du sang; mais on n'a rien constaté de pareil.
De l'anosognosie dans l'hémiplégie cérébrale; contribution clinique à l‘étude de ce symptôme.
Authors: BARKMAN ÅKE
Published: August 2010

Chez quelques patients atteints d'une hémiplégie gauche aiguë, hémiplégie s'accompagnant de troubles sensitifs dans la moitié paralysée du corps et se compliquant le plus souvent d'hémianopsie gauche, en même temps que de déviation conjuguée vers la droite, on a noté du côté des jacultés psychiques un signe particulier se traduisant par: 1° Un défaut de l'attention à l'égard de cette moitié du corps, par suite de quoi les malades vivent dans l'ignorance de leur incapacité fonctionnelle (anosognosie); 2° Une fixation de la mémoire concernant l'état fonctionnel de la région du corps en cause au point même où elle se trouvait avant l'apparition de la maladie, d'où il suit que les malades estiment que cette moitié gauche du corps est absolument saine.

 Les conditions de l'apparition de ce syndrome sont: 1° Une obscurcissement aigu ou une perte aiguë de la conscience (apoplexie); 2° L'atteinte contemporaine du système sensitij (voies de la sensibilité‐éventuellement voies visuelles) qui transmet les impressions nées ou provenant de cette moitié du corps.

 Il n'est pas impossible que, dans tous les cas où apparait ce symptôme, nous rencontrions une localisation du processus pathologique dans le système de la couche optique droite — y compris une partie du lobe pariétal droit.

 Jusqu'ici l'anosognosie n'a été décrite que dans les hémiplégies gauches, ce qui ne veut pas dire qu'elle soit exclusivement liée à des lésions de l'hémisphère cérébral droit.
Zur Klinik und Pathologie der geheilten Lungentuberkulose nebst einigen Worten über deren Röntgenologie.
Authors: LINDBLOM ADOLF F.
Published: August 2010

Verfasser unterscheidet folgende drei Formen von Heilung: klinische Heilung, pathologisch‐anatomische Heilung und bakteriologische Heilung. Verfasser berichtet über drei Fälle von pathologisch‐anatomischer Heilung. Zwei von diesen Fällen waren auch bakteriologisch abgeheilt. In alien drei Fällen waren über den affizierten Stellen harte oder halbharte Rasseln zurückgeblieben. Diese Fälle zeigen daher, dass harte oder halbharte Rasseln nicht nur bei klinischer Heilung, sondern auch bei pathologisch‐anatomischer und bakteriologischer Heilung nachbleiben können.

Verfasser berührt endlich die Frage bezüglich der Beziehung zwischen dem Pathologisch‐anatomischen und dem Röntgenologischen bei geheilter Lungentuberkulose.
Über eine eigenartige, mit Thrombenbildung verbundene Reaktion des Gefässendothels.
Authors: HENSCHEN FOLKE
Published: August 2010

In gewissen kapillären und venösen Gefässgebieten mit Stauung des Blutes und schleichender Entzündung des umgebenden Gewebes fand Verf. eine eigenartige, mit Fibrinoidbildung verbundene Wucherung des Gefässendothels, die zu einer mehr oder weniger umfangsreichen Thrombenbildung führen kann. Die Veränderung dürfte als eine neue, bisher nicht beschriebene Form von produktiver Thrombovaskulitis bezeichnet werden können.

Die gereizten Endothelien wacbsen als lange schlanke Verbände in das stauende aber flüssige Blut hinein und können dabei Teile der Blutmasse vom übrigen Blute abtrennen. Die eingefangenen Erythrozyten geben dann in eine hyaline Masse über, welche sich morphologisch‐tinktoriell wie Fibrin verhält. Am meisten ausgeprägt waren die Veränderungen in den sog. Harnröhrenkarunkeln.

Verf. hebt die Analogie mit der bei einigen allgemeinen Infektionskrankheiten wie Typhus, gewissen Kolibazillosen und Sepsis lenta beschriebenen sog. Endovasculitis verrucosa hervor. Auch bier treten eigenartige hyaline Thrombosen auf, die durch Intimawucherung und fibrinoide Umwandlung von kernhaltigen Zellen und roten Blutkörpern zustande kommen.
Die Beziehung der β‐Lysine der Sera zu den bakteriziden Leukozytenstoffen.
Authors: PETTERSSON ALFRED
Published: August 2010

Es gibt keinen Anhalt für die Annahme einer Identität der β‐Lysine und der baktericiden Leukozytenstoffe, die wahrscheinlich verschiedene Körper sind. Auch der das inaktive Serum reaktivierende Bestandteil der Leukozyten dürfte von den bakteriziden Leukozytenstoffen getrennt sein.
Ein Fall von Diabetes mellitus mit bemerkens‐werter Remission.
Authors: ÅKERRÉN Y.
Published: August 2010

Bericht über einen Fall von Diabetes mellitus mit ziemlich akut einsetzenden Symptomen bei einem 46 Jahre alten Mann, wo der zuerst beobachtete Blutzuckerwert 0.30 % betrug (der erste Nüchternwert nacht ca 20 Stunden Hunger war 0.19 %). Während der ersten Pflegetage lag ausserdem eine bedeutende Ketonurie und während der ersten zwei Pflegewochen eine bedeutende Urobilinurie vor. Die Hyper‐glykämie ging bei Behandlung rasch zurück und während einer Nachbeobachtung von 2 1/2 Jahren ist der nüchterne Blutzuckerwert trotz freier Diät mit starker Kohlehydratbelastung, normal gewesen. Der Harn ist auch zuckerfrei gewesen. Eine vollständige, voraussichtlich dauernde Remission des diabetischen Zustandes ist demnach eingetreten.
Yersuche über mikrochemische Reaktionen.
Authors: HAMMARSTEN EINAR , HAMMARSTEN GRETA , TEORELL TORSTEN
Published: August 2010

Zusammenfassung II.

Die fällende Wirkung von Sulfosalizylsäure und Lanthannitrat auf Eiweiss, Nucleinsäure und Eiweiss‐Nucleinsäuregemische bei verschiedenen Wasserstoffionenkonzentrationen wurde quantitativ untersucht. Es wurde gezeigt, dass, während Eiweiss von Sulfosalizylsäure, Nucleinsäure von Lanthannitrat (aber nicht umgekehrt) gefällt wurden, Eiweiss‐Nucleinsäure‐Gemische mit beiden Reagentien Fällungen gaben.

Mit gepufferten Lösungen von Sulfosalizylsäure und Lathannitrat konnte gezeigt werden, dass rote Blutkörperchen von Eidechsen in einem gewissen Funktionsstadium, das möglicherweise mit der Zellteilung zu tun hat, eiweissfreie oder sehr eiweissarme Zellkerne hatten, während die Zellkerne der eigentlichen Zellteilungsprozesse stark eiweisshaltig waren.
Zwergwuchs mit unsicherer Pathogenese bei drei Brüdern.
Authors: DAM. EINAR
Published: August 2010

Es werden über Fälle von Zwergwuchs bei 3 Brüdern einer sonst normalen Kinderschar berichtet.

Die Ursache muss anscheinend in einer Infektion oder Intoxikation, welche in der Kindheit ihr endokrines System getroffen und dadurch—ohne übrigens andere Spuren zu hinterlassen—auf ihr Knochensystem eingewirkt hat, gesucht werden.

Die Veränderungen in diesem sind ausgesprochen niedrige vertebrae columnae, was in wesentlichem Grade den Zwergwuchs bedingt; ferner Veränderungen in den Fuss‐ und Kniegelenken mit Abflachung der Gelenkenden, nebst Deformation des Collum femoris, anscheinend auf herabgesetzte Widerstandskraft der Knochen, dem Gewichte des Körpers widerstehen zu können, begründet; schliesslich mehr oder weniger ausgesprochene osteoartroitische sekundäre Veränderungen der Hüftgelenke als Folge der hier entstandenen statischen Missverhältnisse.

Die 3 Brüder, die jetzt 27, 28 und 31 Jahre alt sind, können in keine der gewöhnlich bekannten Zwergtypen eingeordnet werden. Die Möglichkeit eines früher nicht beobachteten Krankheitsbildes scheint nicht ausgeschlossen werden zu können.

On cite des cas de nanisme chez 3 frères dont les autres frères et sœurs éAtaient normaux.

La cause en doit probablement éCtre cherchéAe dans une infection ou intoxication qui pendant l'enfance a frappéA leur système endocrin et qui a ainsi exercéA une influence sur le système osseux, sans d'ailleurs laisser d'autres traces.

Le changement de celui‐ci consiste dans des vertebrae columnae distinctement bas, ce qui est une condition essentielle du nanisme; en outre dans le changement des pieds et des articulations des genoux avec un aplatissement des bouts des articulations, ainsi qu'une déAformation de collum femoris, probablement due à une réAduction de la force de réAsistance des os à supporter le poids du corps; enfin dans des changements osteoartroitiques secondaires plus ou moins prononcéAs aux articulations du féAmur par suite des disproportions statiques y produites.

Les 3 frères, à préAsent âgéAs de 27, 28 et 31 ans, ne peuvent éCtre classéAs dans aucun des types de nains géAnéAralement connus. On est d'avis que la possibilitéA d'une maladie pas encore observéAe ne peut pas éCtre exclue.
Über einen Fall essentieller aplastischer Anämie1
Authors: HOLBØLL S. A.
Published: August 2010

1. Es wurde die Krankengeschichte einer Patientin mit einem perniciosa‐ähnlichen Blutbild beschrieben, das sich der Lebertherapie gegenüber refraktär zeigte.

2. Die Differentialdiagnose wird erörtert, und es wird nachgewiesen — durch die Autopsie bestätigt — dass die Diagnose auf essentielle aplastische Anämie zu stellen ist.

3. Es wird hervorgehoben, dass die essentielle, aplastische Anämie, im Gegensatz zur früheren Auffassung, ein jahrelanges Vorstadium mit Herabsetzung der Anzahl der Erythrocyten, Leukocyten und Thrombocyten, allein ohne Regenerationsformen haben kann.

4. Es wird nachgewiesen, dass die essentielle, aplastische Anämie von der Leberbehandlung nicht beeinflusst wird.
Über das Verhalten der Ketonkörper in Blut, Urin und Spinalflüssigkeit, unter besonderer Berücksichtigung des Coma diabeticnm1
Authors: BÜLOW‐HANSEN VICTOR
Published: August 2010

1. Der Verfasser weist nach, dassdie Niereninsuffizienz die Ursache des Coma diabeticum ist.

Die Niereninsuffizienz führt zur Ketonämie.Die Ketonämie wird durch die Lungen auszulüften versucht — »die grosse Atmung». »Kompensierte Niereninsuffizienz». Wenn Nieren und Lungen zusammen die Ketonämie nicht in Schranken halten können, kommt es zumComa diabeticum.

2. Der Verfasser erweist eine dreifache Wirkung des Azetons auf den Organismus:

a) DasAzetonhämolysiert das Blut.

b) » » fällt die Eiweissstoffe des Blutes.

c) » » lähmt die Peroxydasen des Blutes.

3. DasInsulinhebt den lähmenden Einfluss des Azetons auf die Peroxydasen des Blutes auf.

4. Je näher ein Patient sich dem Coma diabeticum befindet, urn so höhere Ketonwerte findet man in der Spinalflüssigkeit im Verhältnis zu denen des Blutes.

5. Die Ketone der Spinalflüssigkeit scheinen von Insulin weniger beeinflusst zu werden als die Ketone des Blutes.
Contribution a l‘étude clinique de Pintoxication par la caféine.
Authors: ERHARDT RICHARD
Published: August 2010

L'auteur du présent travail attire l'attention sur quelques aspects, semble‐t‐il, négligés, des effets psychiques ultérieurs du café chez l'homme; il insiste sur l'importance pratique qui s'attache à cette connaissance. En principe, on ne peut les considérer comme essentiellement différents de ceux de l'alcool; bien plus, dans certaines conditions, ils partagent avec ce poison la propriété »de supprimer la réflexion et d'affaiblir les facultés de la raison». Ces constatations amènent l'auteur à étudier quelles sont les régions de l'organisme qu'on peut supposer en rapport avec la production de ces effets.
Zwerchfellhernie und Magenkarzinom22
Authors: MÖLLER FOLKE
Published: August 2010

Schilderung eines Falles von Zwerchfellhernie (Hiatusbruch) bei gleichzeitiger Existenz eines Magenkarzinoms, einer hochgradigen Anämie nach früheren Blutungen (wahrscheinlich Polypen der Magenwand) sowie einer schweren Ataxie.

Die chronische, mit Anämie einhergehende Blutung scheint bei Zwerchfellhernienträgern nicht selten Au sein und dürfte fast stets auf eine bleibende Veränderung der Schleimhaut Zurückzu‐füher sein, meist auf ein Magengeschwür.

Das Bid der funikulären Myelitis kam hier zusammen mit einer hypochromen Anämie vor.
Ein starkes Topf‐Kurare von den Tucuna‐(Ticuna‐) Indianern des oberen Amazonas.
Authors: SANTESSON C. G.
Published: August 2010

Der deutsche Forschungsreisende Curt Nimuendajú hat (Nov. 1929) die Tucna‐Indianer im oberen (westlichen) amazonasgebiete besucht und davon ein interessantes Material mitgebracht, welches von Prof. Erland Nordenskiöld für das etnographische Museum in Gothenburg eingekauft worden ist. Unter den gegenständen war auch ein kleiner Topf mit Kurare. Da die benutzen, ist sowohl die Bereitung als die Anwendung von Kurare im Bergiff verdirängt zu werden. Es ist daher berechtigt, die vorliegende Probe zu untersuchen, besonders weil sie zugleich sehrstark ist. Die Lösung der Droge zeigt die gewöhnlichen Alkaloidreaktionen, gibt auch mit Ammoniak einen Niederschlag, was nach R. Boehm für das Topfkurare bezeichnend ist. Bei Fröschen (Esculenten) geben noch 0.3 mg eine tötliche Kurarelähmung, 0.01 mg deutliche Parese, die 2 bis 3 Stunden dauerte, 0.008 mg sind unwirksam. Die Letalgfabe für Kaninchen liegt Zwischen 2 und 3 mg per Kilo. Das stärkste Topfkurare Boehm's zeigte für Kaninchen eine Letalgabe von 0.8 mg per kilo, das darin vorhindeutet, dass etwa 1/3 seiner stärksten Rohdroge aus dem entsprechenden Kurarin bestanden hat. Da in unserem Kurare wohl auch Protokurarin vorkommt, ist aus der Letalgabe (3 mg pr Kilo) zu entnehmen, dass das Protokurairn etwa 8 Proz. davon ausmacht. In drr am Frosch eben wirksamen Gabe der Rohdroge (0.01 mg) durfte also etwa 0.0008 mg Protokurarin eingehen.