Info Jbi
Amyloidosis Systemic, Nodular, and Epidermal Derived S Primary, AL type, myeloma-associated N None S Plasma-cell-derived light chains N Plasma-cell-derived light chains ED Epidermal keratins S Multiple myeloma in 1 3 pts. N 10 with systemic amyloidosis ED None S Hemorrhagic papules plaques N Waxy nodules ED Hyperpigmented macules and lichenoid papules S Fissured hyaline deposits, perivascular deposits N Hyaline deposits c plasma cells ED Hyaline dermal globules S Monoclonal kappa or lambda...
Info Rzo
Glucagon-secreting tumors, usually malignant and MEN I syndrome, Zollinger-Ellison syndrome Rash erythematous, scaly patches intertriginous and acral area angular chelitis, diabetes mellitus, diarrhea Mild epidermal acanthosis with confluent parakeratosis and vacuolar degeneration of the superficial epithelial Malignant pancreatic tumor, weight loss, metastases Necrolytic migratory erythema NME , referred to as the glucagonoma syndrome, is a rare paraneoplastic syndrome consisting of the...
Subcutaneous Panniculitislike Lymphoma
Subcutaneous lymphoma, cytophagic histiocytic panniculitis Hemorrhagic plaques and nodules, abdomen and extremities Dense lymphoid infiltrate in subcutis with hemorrhage and necrosis Systemic workup required to assess extent Subcutaneous panniculitis-like lymphoma SPL occurs primarily in younger adults, but can arise in persons of any age. The usual presentation is that of an ulcerative and hemorrhagic panniculitis, presenting as multiple ery-thematous nodules often involving the lower...
Pancreatic Panniculitis
Synonyms Pancreatic fat necrosis, nodular fat necrosis of pancreatic disease Etiology In part, due to cutaneous enzymatic fat necrosis from circulating pancreatic enzymes Associations Acute and chronic pancreatitis, acinar cell carcinoma Clinical Erythematous subcutaneous nodules favoring lower extremities, may drain spontaneously arthritis, serositis, abdominal pain, nausea, vomiting, lytic bone lesions Histology Subcutaneous fat necrosis with loss of adipocyte nuclei, ghost cells, stippled...
Gardner Syndrome
Synonyms Familial adenomatous polyposis FAP , adenomatous polyposis coli APC Etiology Mutation in adenomatous polyposis coli APC gene, chromosome 5q21 Associations Colonic tubular adenomas and carcinoma, cutaneous epidermoid cysts, fibromas, desmoids, osteomas Histology Epithelial cysts with pilomatrical differentiation, fibrous nodules Evaluation Ophthalmologic examination, panoramic dental radiographs, lower GI tract evaluation, possible APC gene mutation evaluation Treatment Prophylactic...
Scleromyxedema
Lichen myxedematosus, papular mucinosis, Arndt- Paraproteinemia, multiple myeloma, hepatitis C, HIV Increased dermal mucin with increased fibroblasts Slowly progressive increased mortality rate Scleromyxedema is a cutaneous condition that is associated with paraproteinemia in more than 80 of cases 1 . In the vast majority of cases, the paraprotein is an IgG heavy chain and a y light chain. Rare cases of K light chains have been reported, and polyclonal hypergammopathy is also seen in some...
Info Vaw
Gyrate Erythemas Erythema Gyratum Repens and Erythema Chronicum Migrans ECM Infection with Borrelia burgdorferi EGR Internal malignancy, tuberculosis ECM Outdoor activities in endemic areas for Ixodes EGR Truncal arcuate erythematous bands with trailing ECM Expanding erythematous solid or annular patch EGR Mild spongiosis with superficial perivascular ECM Nonspecific superficial and deep lymphocytic ECM Early, early disseminated, chronic late ECM Good with appropriate antibiotic therapy, lt 5...
Info Irb
Lethal Non-Langerhans Cell Histiocytoses Necrobiotic Xanthogranuloma and Xanthoma Disseminatum NXG Necrobiotic xanthogranuloma with NXG Myeloma, Grave's disease, primary biliary XD Myeloma, Waldenstrom's macroglobulinemia, NXG Ulcerating red-orange plaques, particularly of the XD Disseminated red brown papules into plaques NXG Palisading granulomatous dermatitis with bizarre XD Diffuse epithelioid histiocytes, foam cells, and XD Cases associated with myeloma and Waldenstrom's NXG...
Multicentric Reticulohistiocytosis
Mutilating osteoarthritis, visceral malignancies Aggregations of histiocytes with ground glass cytoplasm Multicentric reticulohistiocytosis is a rare syndrome of adulthood characterized by the rapid appearance of multiple papules and nodules arising in conjunction with a severe, mutilating arthritis. When occurring as part of the syndrome, the reticulohistiocytomas are found on mucosal surfaces in up to 50 of cases 1-2 . Visceral reticulohistiocytomas involving lymph nodes, bone marrow, lungs,...
Metastatic Carcinoma
Cutaneous metastatic neoplasm, Sister Mary Joseph Hematogenous or lymphatic spread of primary tumor to Primary malignancies most commonly gastrointestinal Nodule, occasionally ulcerated, scalp most common site Most commonly adenocarcinoma in dermis Cytokeratins 7, 20, carcinoembyronic antigen Implies widespread metastatic disease Less than 1-year survival in most cases None ovarian primary may be associated with slightly Cutaneous metastases occur in approximately 10 of all cancer patients. The...
Cowdens Syndrome
Multiple hamartomas, keratoacanthomas, neoplasms Hyperkeratotic folliculocentric papules Proliferations of pale staining keratinocytes from Trichilemmomas are benign epidermal neoplasms that may serve as an indicator of underlying malignancy. Trichilemmomas appear, largely on the head and neck, as exophytic, small, hyperkeratotic papules Figure 15.1 . There is no particular gender predilection and they usually arise during the second decade. When occurring as isolated lesions, these tumors are...
Info Gbd
Acquired Ichthyosis, Acanthosis Nigricans, Palmar Hyperkeratosis PH Tylosis, Howel-Evans Syndrome, tripe palms, AI Hypogranulosis with hyperkeratosis PH Acanthosis with hyperkeratosis and hypergranulosis Generally poor, associated with advanced internal Dependent upon underlying malignancy type and stage of Serious systemic diseases, including visceral cancer, may be indirectly signaled by the development of distinctive cutaneous eruptions. Important aspects of these eruptions include...
BirtHoggDube Syndrome
Mutation in the folliculin gene, chromosome 17p11.2 Fibrofolliculomas, trichodiscomas, acrochordons, pulmonary cysts with spontaneous pneumothorax, renal carcinoma, and colorectal carcinoma in some kindreds Skin-colored papules of face, neck, ears, and upper trunk, with intertriginous soft papules Trichodiscoma interfollicular ovoid nodule with spindled cells in loose fibrillary stroma Fibrofolliculoma central follicle with extension of irregular epithelial strands into surrounding well-defined...
Pagets Disease
Extramammary Paget's disease, mammary Paget's disease Ductular extension of carcinoma from breast genitourinary, gastrointestinal, or apocrine glands Underlying adenocarcinoma of breast mammary or genitourinary or gastrointestinal carcinoma extramammary or primary appendageal Scaly, erythematous patch on nipple mammary or anogenital region extramammary Paget's Large, atypical cells at all levels of epidermis Essential to workup for underlying adenocarcinoma Excellent if no underlying carcinoma...
Merkel Cell Carcinoma
Trabecular carcinoma of skin, primary small cell carcinoma of skin, cutaneous APUDoma Ultraviolet light, chromosome 1 abnormalities, p53, bcl-2, Aging, immunosuppression, other cutaneous and visceral Painless, solitary rapidly growing nodule on exposed Diffuse or aggregated dermal nests of small blue cells, CK-20 , synaptophysin , S-100 - , Melan-A - , I localized disease, II I and regional node s , Male, head location, mitoses gt 10 HPF, vascular I WLE XRT, II WLE XRT ELND, III XRT CTX ABMT...
Cutaneous BCell Lymphoma
Lymphoma cutis, marginal zone lymphoma, follicular lymphoma, large cell lymphoma, malignant Violaceous nodules, most common on head and neck Malignant lymphocytes in dermis, diffusely or in patchy Lymphocyte surface markers and light chains Radiation, intralesional chemotherapy systemic chemotherapy if systemic involvement Cutaneous B-cell lymphoma is not a single disease, but rather a family of neoplastic processes characterized by a proliferation of malignant B lymphocytes. These lymphomas...
Mast Cell Disease Urticaria Pigmentosa
IHC repertoire Staging Prognosis Adverse variables Treatment Urticaria pigmentosa, telangiectasia macularis eruptive Nausea, vomiting, diarrhea, syncope, mast cell leukemia, Papules or nodules with or without associated hyperpigmentation and telangiectasia positive Darier's Increased dermal mast cells perivascular or as tumor nodules, basilar hyperpigmentation, vascular ectasia CD117 c-kit and mast cell tryptase positive Bone marrow involvement conveys poor prognosis Varies with subtype of...
Langerhans Cell Histiocytosis
Synonyms Histiocytosis X, Langerhans cell granulomatosis, eosinophilic granuloma, Hand-Sch ller-Christian disease, Letterer-Siwe disease, self-healing reticulohistiocytosis, Hashimoto-Pritzker syndrome Etiology Unknown, a clonal or reactive expansion of Langerhans cells infiltrating various organs Associations May coexist, precede, or follow the development of various solid tumors and hematopoietic malignancies Clinical Polymorphous red-brown purpuric scaly papules, lichenoid papules, purpura,...
Granulomatous Slack Skin
Cutaneous T-cell lymphoma, granulomatous mycosis Hodgkin's disease, mycosis fungoides, non-Hodgkin's lymphoma, leukemia, Langerhans cell histiocytosis Pendulous folds in axilla and inguinal regions Epidermotropic, hyperchromatic lymphocytes in epidermis with admixed granulomatous areas and Controversial possibly better than conventional mycosis Anaplasia of T cells, nodal involvement Electron beam irradiation, topical and systemic Granulomatous slack skin GSS is a subtle variant of mycosis...
Leukemia Cutis
Cutaneous leukemia, extramedullary myeloid tumor Erythematous patches, papules, nodules, hemorrhagic and purpuric lesions, blue-green nodules Malignant immature hematopoietic precursor cells in dermis, diffusely or in patchy distribution Lymphocyte surface markers and or markers of specific Histologic subtype of high-grade leukemias No local therapy systemic chemotherapy Leukemia cutis is an uncommon cutaneous eruption that may be difficult to diagnose. In the vast majority of patients, there...
Angiosarcoma
Hemangiosarcoma, lymphangiosarcoma, malignant Ultraviolet light, radiotherapy, lymphedema Treves- Stewart syndrome , preexisting vascular malformations Rapidly expanding bruise-like patch, erythematous Ill-defined anastomosing dermal network of atypical endothelial-lined spaces most common or defined diffusely arranged aggregates of epithelioid or spindled CD-31 most sensitive and specific , CD-34, Ulex Size gt 5 cm, depth of invasion gt 3.0 mm, mitotic rate gt 3 HPF, positive surgical margins,...