[Basal cell carcinomas in radiotherapy-treated port wine stains. An ideal indication for Mohs surgery]. / Basalzellkarzinome auf mit Radiotherapie vorbehandelten vaskulären Malformationen. Eine ideale Indikation für mikroskopisch kontrollierte Chirurgie.

BACKGROUND: Early treatment of port wine stains with ionizing radiation can lead to the development of often multifocal basal cell carcinomas (BCC) after decades. In most cases it is clinically impossible to distinguish between the tumor and the underlying vascular malformation and to decide where to set surgical margins. PATIENTS AND METHODS: We report on a series of three patients with BCC overlying a port wine stain that had previously been treated with radiation therapy in early childhood. In all patients Mohs surgery was performed to insure complete excision of the BCC. RESULTS: In our patients, development of BCC occurred about 20 to 40 years after radiation therapy. Clinically - without the help of Mohs surgery - the borders of the BCC could not have been detected due to the underlying nevi flammei in all cases. CONCLUSIONS: BCC overlying a port wine stain previously treated with radiation therapy is a rare but ideal indication for Mohs surgery. In addition we recommend regular clinical follow-ups to detect recurrent or additional BCC as early as possible.

The immunohistochemical localization of lysozyme in human axillary apocrine glands.

Lysozyme has been observed in intraluminal secretory products of apocrine glands in specimens of normal human axillary skin. Lysozyme was also observed in an occasional apocrine secretory cell, as well as in leukocytes within vascular lumina and dermal histiocytes. Lysozyme was not observed in sebaceous glands, eccrine glands, or cells of the epidermis. These observations support an epithelial origin of cutaneous lysozyme and suggest a means of further characterization of the origin and/or differentiation of tumors of appendageal origin.

Localization of factor VIII-related antigen in vascular endothelial cells using an immunoperoxidase method.

Using an immunoperoxidase method, factor VIII-related antigen was localized in vascular endothelial cells. This method provides a marker for endothelial cells at the tissue level and may be used to confirm the vascular nature of a variety of hyperplastic and neoplastic lesions of skin, soft tissues, and other locations.

Persistent cutaneous lymphoid hyperplasia of the face with conjunctival involvement.

Lymphocytic infiltration of the orbital conjunctiva has not, to our knowledge, been previously reported as a phenomenon of secondary involvement following long-standing cutaneous lymphocytic infiltration of the face. We encountered a woman with facial cutaneous lymphocytic infiltration pursuing a benign but persistent course, with eventual involvement of the conjunctiva after 15 years, which responded to low-dose radiation therapy.

Immunohistochemical identification of lysozyme in cutaneous lesions of alleged histiocytic nature.

Histiocytosis X, multicentric reticulohistiocytosis, juvenile xanthogranuloma, the "fibrous" type of dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma are all characterized by dermal and/or subcutaneous infiltrates composed at least partially of cells having morphologic features suggestive of histiocytes. Paraffin-embedded tissues representing these conditions were stained for lysozyme (muramidase) with a peroxidase-antiperoxidase technic. The cells of juvenile xanthogranuloma were rich in lysozyme. Some of the cells of histiocytosis X showed a positive pattern, and the cells of the other three conditions were essentially negative. This study confirmed the histiocytic nature of juvenile xanthogranuloma and multicentric reticulohistiocytosis, supported the interpretation that there is a histiocytic component in the lesions of histiocytosis X, and cast some doubt on the alleged histiocytic nature of "fibrous" dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma.

Immunohistochemical identification of factor VIII-related antigen in endothelial cells of cutaneous lesions of alleged vascular nature.

Immunohistochemical staining for factor VIII-related antigen (FVIII-RAG) with the peroxidase-antiperoxidase technic was used as a marker for endothelial cells in a variety of nevoid, reactive, and malignant vascular cutaneous proliferations. The endothelial cells of small normal cutaneous vessels gave the strongest reaction. The cells of hemangioma and angiokeratoma generally were stained, but with less consistency. In lymphangioma, pyogenic granuloma, and pigmented purpuric dermatosis, positivity was either patchy or of lesser intensity. Numerous strongly positive endothelial cells lining well-formed blood vessels were present in lesions of Kaposi's sarcoma, but the proliferating spindle cells forming "vascular slits" were uniformly negative. The cells of angiosarcoma were essentially negative, except for isolated elements in the better-differentiated areas. Plasma was also strongly positive; this may aid in distinguishing vascular from lymphatic channels. Anti-FVIII-RAG immunoperoxidase staining is a helpful aid in evaluating cutaneous vascular proliferations.

Benign mucous membrane pemphigoid in a wife and husband.

Benign mucous membrane pemphigoid, in the form of desquamative gingivitis, occurred in a 57-year-old woman. The disease then developed in her 59-year-old husband 16 months later. The clinical manifestations were mild and similar in both cases. Direct immunofluorescence microscopy tests were consistent with the diagnosis. Sequential occurrence of this disease in spouses suggests the possibility that it is transmissible.

Negative immunoperoxidase staining for lysozyme in nodular subepidermal fibrosis.

Nodular subepidermal fibrosis (NSF) is a clinical entity, the histogenetic origins of which remain unclear. More than 200 such lesions were examined with light microscopy and subdivided into four types based on their relative degree of cellularity. Five examples of each subtype were stained for lysozyme with the peroxidase-antiperoxidase technique. None of the 20 lesions contained cells with lysozyme. We conclude that the basic cell type in NSF is not lysozyme-containing macrophage; it is a different cell, perhaps one of mesenchymal origin.

Lupus erythematosus with oral and perianal mucous membrane involvement.

A 57-year-old man with lupus erythematosus (LE) had mucous membrane involvement that included the oral and perianal mucosa. Microscopic findings of the oral and perianal mucous membrane lesions were not diagnostic of LE, but immunofluorescent microscopy of biopsy specimens of the lip and perianal mucosa showed the lupus band.

Perianal apocrine gland adenoma.

A 59-year-old man had a pedunculate perianal tumor initially thought to be a condyloma acuminatum. Since it failed to respond to topical podophyllum resin, the growth was excised and found to be an apocrine gland tumor. Histological features included a duct extending from the surface epidermis to multiple tubules, which led to cystic spaces with papillary projections of columnar epithelium. Decapitation secretion and fibrous stromal proliferation were prominent. The clinical and histological similarities to other apocrine gland adenomas are briefly presented.

Solitary congenital indeterminate cell histiocytoma.

BACKGROUND: Indeterminate cell proliferations are rarely described in the literature. We present a case of a patient who presented at birth with a solitary lesion that spontaneously regressed. OBSERVATIONS: Five cases of indeterminate cell proliferations have been described in the literature. Their clinical patterns display some common features including adult onset, usually with multiple lesions and a relatively benign clinical course. This patient presented at birth with a single lesion and spontaneous regression, as one would expect with congenital self-healing reticulohistiocytosis. Biopsy confirmed a dermal histiocytic nodule that was predominantly CD1 and S100 positive. Unlike patients with congenital self-healing reticulohistiocytosis, however, electron microscopy failed to demonstrate Birbeck granules. CONCLUSION: Indeterminate cell proliferations are a distinct entity. This presentation expands the range of clinical manifestations reported. It also suggests a close relationship between indeterminate cells and Langerhans cells.

Cutaneous involvement in Lennert's lymphoma.

Lennert's lymphoma is a fairly distinct clinicopathologic entity that may rarely involve the skin. Patients tend to be elderly women with prominent cervical adenopathy; they often have a rapidly fatal disease course. We describe here an 85-year-old woman with lymphadenopathy and erythematous cutaneous nodules who had malignant lymphohistiocytic infiltrates in both her skin and lymph nodes, with a high concentration of epithelioid histiocytes in the latter.

Vasculitis in cytomegalovirus infection.

A 7-year-old girl had fever, arthralgia, and a mild cutaneous vaculitis with papules, nodules, and livedo. A biopsy specimen of a papule showed lymphocytic small-vessel vasculitis, with some atypical lymphocytic nuclei. Splenomegaly developed, and results of subsequent studies supported a diagnosis of cytomegalovirus (CMV) infection, with atypical peripheral blood lymphocytes and a characteristic pattern of complement-fixing antibodies to CMV antigen. The CMV mononucleosis syndrome is rarely reported in children, and the cutaneous manifestations are usually rubelliform. Distinctive cutaneous pathologic characteristics have been described previously only in neonates ("blueberry muffin" syndrome) and in immunosuppressed patients (viral inclusion bodies in endothelial cells).

Norwegian scabies in Bloom's syndrome.

Norwegian scabies is an unusual Sarcoptes scabiei infestation. It has been reported often in patients who are either severely debilitated or who have congenital or iatrogenic suppression of their immune responses. We report the occurence of Norwegian scabies in a 13-year-old boy with Bloom's syndrome who had impaired humoral and cell-mediated immunity.

Serum lysozyme in patients with localized and generalized granuloma annulare.

Serum lysozyme (Muramidase) levels in patients with localized and generalized granuloma annulare were measured by a turbidometric method. More lysozyme is present in the serum samples of patients with generalized granuloma annulare than patients with the localized form or normal controls. The mean level of patients with generalized disease was 9.27 mg/L compared with 5.96 mg/L for patients with localized disease and 6.8 mg/L for controls.

Dermal mucinosis in discoid lupus erythematosus. Report of two cases.

Two patients with discoid lupus erythematosus (DLE) had lesions exhibiting prominent atrophy and telangiectasia. At the time of biopsy, the skin specimens were grossly mucinous. Light and immunofluorescence microscopy findings confirmed the diagnosis of DLE in both cases. Abundant mucin deposition throughout the dermis was also seen microscopically in both cases. Although secondary mucinosis is known to occur in LE, cases with massive mucinous change, ie, those reported herein, have apparently not been recorded. Mucinosis is not considered important in the histologic diagnosis of LE but may create some confusion with other mucinous disorders.

Progressive nodular histiocytoma.

Progressive nodular histiocytoma is a clinically distinct variant in the general group of normolipemic histiocytic proliferations. Its cardinal feature is the presence of two different lesions--a superficial yellow-brown papule and a deep nodule. We describe herein a 29-year-old man with such lesions. Histologically, a variety of fibrous and cellular patterns were seen, mimicking the wide spectrum of findings in ordinary dermatofibromas. On ultrastructural examination, the most striking feature of the preponderant cell was lipid phagocytosis.

Concordance between telepathologic diagnosis and conventional histopathologic diagnosis: a multiobserver store-and-forward study on 20 skin specimens.

OBJECTIVE: To study the validity and feasibility of transferring images of cutaneous biopsy specimens via e-mail to remote physicians active in dermatopathology for teleconsultation. DESIGN: Twenty skin specimens previously diagnosed at the Department of Dermatology, University of Graz, Austria, were subsequently sent for teleconsultation using the store-and-forward method. For each case, 3 or 4 images at different magnifications were sent by e-mail to 16 colleagues (11 dermatopathologists and 5 pathologists) in 15 centers in 6 different countries. Six weeks later each observer received the hematoxylin-eosin-stained specimens to render a conventional diagnosis. SETTING: Dermatopathology and pathology units within institutional and private settings. MATERIAL: Twenty small skin biopsy specimens of cutaneous diseases were selected randomly from a study set of 80. MAIN OUTCOME MEASURE: Concordance between telepathologic diagnoses and conventional histopathologic diagnoses of 20 skin specimens. RESULTS: On average, 78% of the telediagnoses were correct (range, 60%-95%), whereas 85% of the conventional diagnoses were correct (range, 60%-95%). A perfect diagnostic concordance was obtained in 7 (35%) of 20 cases, and a significant difference was identified in only 1 case. CONCLUSIONS: Results suggest that telepathology performed by physicians active in dermatopathology may serve as a reliable technique for the diagnosis of cutaneous diseases when experts in dermatopathology are not available locally. Furthermore, teledermatopathology is attractive because it provides an opportunity to obtain timely consultation on difficult cases.