Mycosis Fungoides with Dermal Mucin Deposition: An Unusual Variant of the Most Challenging Primary Cutaneous Lymphoma.

Mycosis fungoides (MF) is defined as an epidermotropic primary cutaneous T-cell lymphoma composed of small-to-medium-sized T lymphocytes with cerebriform nuclei and with a T-helper phenotype. LeBoit first described an unusual variant of MF with dermal acid mucin deposition. Such a variant was still considered in the list of clinicopathological variants of MF by Cerroni and colleagues. We herein report a case of patch-stage MF with abundant papillary dermal mucin deposition in a clinical setting of an erythematous patch on the lower abdomen and thigh.

Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby "Spectrum".

A case of congenital self-healing Langerhans cell histiocytosis (CSHLCH), also known as Hashimoto-Pritzker disease, is reported. The newborn presented as blueberry muffin baby at birth, showing numerous non-blanching blue-purplish and dark-red papular, nodular lesions without documented infections and systemic involvement. Histopathological and immunohistochemical findings were suggestive for Langerhans cell histiocytosis. During the first 12 weeks of life, the cutaneous lesions progressively and spontaneously regressed with some atrophic scars. One-year follow-up is negative for relapse of cutaneous lesions or systemic involvement, confirming the diagnosis of CSHLCH.

Human Papilloma Virus-Associated Lips Verrucous Carcinoma in HIV-Infected Male.

Human papillomavirus (HPV) infection, widely known as the necessary cause of cervical cancer, has been established as a major etiologic factor for head and neck cancer (HNC). HIV-infected individuals are at higher risk of HPV-associated cancers than the general population. We describe a 45-year-old man with HIV and HPV coinfection, who presented progressively enlarging verrucous neoformations of the lips. The final diagnosis of verrucous carcinoma was delayed. Early detection of HPV lesions in oral mucosa and HPV screening activities could be important in improving the diagnostic sensitivity for the HIV-infected patients with oral cancer.

Prior knowledge of the clinical picture does not introduce bias in the histopathologic diagnosis of melanocytic skin lesions.

A common debate among dermatopathologists is that prior knowledge of the clinical picture of melanocytic skin neoplasms may introduce a potential bias in the histopathologic examination. Histologic slides from 99 melanocytic skin neoplasms were circulated among 10 clinical dermatologists, all of them formally trained and board-certified dermatopathologists: 5 dermatopathologists had clinical images available after a 'blind' examination (Group 1); the other 5 had clinical images available before microscopic examination (Group 2). Data from the two groups were compared regarding 'consensus' (a diagnosis in agreement by ≥4 dermatopathologists/group), chance-corrected interobserver agreement (Fleiss' k) and level of diagnostic confidence (LDC: a 1-5 arbitrary scale indicating 'increasing reliability' of any given diagnosis). Compared with Group 1 dermatopathologists, Group 2 achieved a lower number of consensus (84 vs. 90) but a higher k value (0.74 vs. 0.69) and a greater mean LDC value (4.57 vs. 4.32). The same consensus was achieved by the two groups in 81/99 cases. Spitzoid neoplasms were most frequently controversial for both groups. The histopathologic interpretation of melanocytic neoplasms seems to be not biased by the knowledge of the clinical picture before histopathologic examination.

Surgical solutions for the reconstruction of the lower eyelid: canthotomy and lateral cantholisis for full-thickness reconstruction of the lower eyelid.

The lower eyelid is the most common site of tumors of the orbital region, usually of basocellular skin carcinoma, whose incidence is increasing with population aging in Western countries. The treatment must achieve the complete removal of the neoplasm in order to prevent recurrences, thus requiring eyelid and periocular reconstruction. Several techniques are described for eyelid defect reconstruction, aiming for direct suture eased by flaps, or considering the use of grafts. We report our experience with 22 patients operated on for basocellular carcinoma of the lower eyelid, for wich defects were repaired by means of a full-thickness reconstruction using a canthotomy and lateral cantholisis; in 13 patients the canthotomy was combined with a lateral-canthal flap. We achieved good results in terms of orbital function and aesthetic outcome, as long as radical removal of the tumor without local recurrences was attainable.

B-cell lymphofollicular infiltrates in mycosis fungoides.

AIMS AND BACKGROUND: The histopathological picture of mycosis fungoides can be characterized by heavy reactive infiltrates. These are rarely composed of B lymphocytes with formation of reactive follicles. METHODS AND STUDY DESIGN: We collected three cases of mycosis fungoides with a reactive B-cell lymphofollicular reaction at the bottom of the epidermotropic infiltrate. RESULTS: Case 1 showed flat lesions (patches) with a CD4+ neoplastic phenotype; case 2 presented infiltrated lesions (plaques) with a CD8+ immonophenotype; case 3 was characterized by nodular lesions (tumors) with a CD4+ neoplastic component. In all three cases, no clonal gene rearrangement was found with the polymerase chain reaction technique. CONCLUSIONS: Among the protean clinicopathological features which mycosis fungoides can show, we underline that a B-cell lymphofollicular component can be encountered at the base of the epidermotropic infiltrate even in clinically flat ("patchy") lesions.

The single-photon emission computed tomography/computed tomography: a new procedure to perform the sentinel node biopsy in patients with head and neck melanoma.

The aim of this study was to define and validate a new technique to detect the sentinel node (SN) in patients treated for head and neck melanoma. In a small series of 23 head and neck melanoma patients, lymphatic mapping was followed by SN biopsy, using in 12 patients a new diagnostic imaging technique, single-photon emission computed tomography/computed tomography. The procedure is described and the major problems encountered are discussed. The preliminary data show that identification of SN using single-photon emission computed tomography/computed tomography never failed in 12 patients, and biopsies performed, compared with those in a standard group, took significantly less time (Mann-Whitney test P=0.006). In conclusion, the authors underline the possibility of a wide use for this technique.

Clinical improvement of psoriasis in an AIDS patient effectively treated with combination antiretroviral therapy.

Psoriasis has been classified as a T-cell-mediated inflammatory disease. The paradoxical exacerbation of psoriasis in AIDS has not been fully explained. We describe a case of a 45-y-old male with AIDS whose coincidental psoriasis resolved following antiretroviral therapy. Dramatic improvement was seen 4 weeks after combination antiretroviral therapy including enfuvirtide was started. In advanced HIV disease psoriasis could represent a clinical index of progression of HIV disease. Several pathogenetic factors involved in the clinical manifestations of psoriasis in the HIV-infected population are discussed.

Palatal tremor suppressed by mouth opening: clinical and neurophysiological correlations in two patients.

We report clinical and neurophysiological findings in two patients with palatal tremor (PT). In both patients a prompt and persistent suppression of palatal movements and clicking sounds is caused by slight passive or active mouth opening. One patient has a typical essential palatal tremor (EPT) according to current classification criteria. The other one has a PT characterized by involuntary activation of levator veli palatini and genioglossus muscles. Objective clicking sounds were observed late during the course of the disease. A symptomatic aetiology was excluded, suggesting a diagnosis of atypical EPT. Our observations further confirm that unusual features of some cases may not fit current PT classification criteria. These two cases have several features that distinguish them from voluntary PT and from psychogenic PT. We emphasize that clear cut modulating phenomena may be relevant features in both typical and atypical involuntary EPT. We finally focus on the significance of jaw posture in determining PT suppression in our patients.