Stefano Cavicchini

Roaccutane Treatment Guidelines: Results of an International Survey

BACKGROUND Oral isotretinoin (Roaccutane) revolutionized the treatment of acne when it was introduced in 1982. METHODS Twelve dermatologists from several countries with a special interest in acne treatment met to formally review the survey of their last 100 acne patients treated with oral isotretinoin. The primary purpose of the survey was to identify the types of acne patients who were prescribed oral isotretinoin and how the patients were managed. RESULTS Of the 1,000 patients reviewed, 55% of those who received oral isotretinoin had those indications treated historically, i.e. severe nodular cystic acne or severe inflammatory acne, not responding to conventional treatment. Forty-five percent of patients who were prescribed oral isotretinoin however had either moderate or mild acne. Most patients in this group had moderate acne (85%). However, 7.3% had mild acne on physical examination. The criteria for prescribing oral isotretinoin in this less severe group of patients included acne that improves < 50% after 6 months of conventional oral antibiotic and topical combination therapy, acne that scars, acne that induces psychological distress and acne that significantly relapses during or quickly after conventional therapy. Treatment is usually initiated at daily doses of 0.5 mg/kg (but may be higher) and is increased to 1.0 mg/kg. Most of the physicians aimed to achieve a cumulative dose of > 100-120 mg/kg. Mucocutaneous side-effects occur frequently but are manageable while severe systemic side-effects are rarely problematic (2%). The teratogenicity of oral isotretinoin demands responsible consideration by both female patients and their physicians. Significant cost savings when treating acne patients with oral isotretinoin as compared to other treatment modalities were further proven in this study. CONCLUSIONS Our recommendation is that oral isotretinoin should be prescribed not only to patients with severe disease but also to patients with less severe acne, especially if there is scarring and significant psychological stress associated with their disease. Acne patients should, where appropriate, be prescribed isotretinoin sooner rather than later.

Blue-black rule: A simple dermoscopic clue to recognize pigmented nodular melanoma

Background  Dermoscopy improves melanoma recognition, but most criteria were described in the context of superficial spreading melanoma.

Spitz nevus, Spitz tumor, and spitzoid melanoma: a comprehensive clinicopathologic overview.

Spitz nevus can clinically present either in the classical (reddish pink) or the pigmented (brownish black) variant. Dermoscopy demonstrates that the pigmented variant is much more common than the classical variant; however, none of these show dermoscopic patterns clearly distinguishable from melanoma. Even histopathologically, a clear-cut differentiation between benign and malignant spitzoid neoplasms is often difficult, so that intermediate diagnostic categories (atypical Spitz nevus and Spitz tumor) are admitted. Because of these difficulties in clinical and histopathologic evaluation, surgical excision is recommended for clinically atypical spitzoid lesions of childhood and for all spitzoid lesions of adulthood.

Primary cutaneous pleomorphic T-cell lymphoma expressing CD30 antigen

Pleomorphic peripheral T-cell lymphomas (PPTL) arising primarily in the skin are uncommon. The observation of pleomorphic T-cell lymphoma composed of 100% CD30+ cells is rare. We report the case of a woman in whom such a tumor manifested itself with a single cutaneous lesion. It was composed of medium to large cells expressing markers of helper/inducer T-lymphocytes, as well as activation (CD30) and proliferation antigens (Ki-67), but not the CD25 activation antigen. The lesion was excised and the patient is alive and disease free 2 years after the diagnosis. This case confirms previous reports about the favorable course of some cutaneous pleomorphic lymphomas, and shows that the activation antigen expression and proliferation antigen (Ki-67) expression do not seem to worsen the prognosis.

Clinical and dermoscopic features of atypical Spitz tumors: A multicenter, retrospective, case-control study

BACKGROUND Few studies have described the clinical and dermoscopic features of atypical Spitz tumors. OBJECTIVE We sought to describe the clinical and dermoscopic features of a series of atypical Spitz tumors as compared with those of conventional Spitz nevi. METHODS This was a multicenter, retrospective, case-control study, analyzing the clinical and dermoscopic characteristics of 55 atypical Spitz tumors and 110 Spitz nevi that were excised and diagnosed histopathologically. RESULTS The majority of atypical Spitz tumors presented clinically as a plaque or nodule, dermoscopically typified by a multicomponent or nonspecific pattern. A proportion of lesions (16.4%) exhibited the typical nonpigmented Spitzoid pattern of dotted vessels and white lines under dermoscopy. Nodularity, ulceration, linear vessels, polymorphic vessels, white lines, and blue-white veil were associated with atypical Spitz tumors by univariate analysis, but only nodularity and white lines remained significant after multivariate analysis. In contrast, a pigmented typical Spitzoid pattern was a potent predictor of Spitz nevi, associated with 6.5-fold increased probability. LIMITATIONS Differentiation from Spitzoid melanoma and other nonmelanocytic lesions was not investigated. CONCLUSION Atypical Spitz tumors are polymorphic melanocytic proliferations with a nodular clinical appearance. Dermoscopically they demonstrate a multicomponent and nonspecific pattern. A typical nonpigmented Spitzoid pattern on dermoscopy (with dotted vessels and white lines) does not exclude atypical Spitz tumors.

Xanthoma disseminatum: A case with extensive mucous membrane involvement

A 37-year-old man had red or brownish papules on his face, chest, and groin, where they coalesced into plaques. Endoscopic examination revealed the presence of small xanthomatous papules on the mucous membrane of the stomach. A biopsy specimen revealed a dense infiltrate of histiocytes, foam cells, Touton giant cells, and inflammatory cells. The diagnosis of non-X histiocytosis was confirmed by immunohistochemical studies of the infiltrate.

Hypopigmented atypical Spitzoid neoplasms (atypical Spitz nevi, atypical Spitz tumors, Spitzoid melanoma): a clinicopathological update

The clinicopathologic classification, diagnosis, and management of Spitzoid melanocytic lesions is one of the most problematic topics in dermato-oncology and dermatopathology. After earlier anecdotal reports [1,2], the controversial history of these controversial lesions began in 1948 when Sophie Spitz described 13 cases of what she called “juvenile melanoma,” underlining its presumably good prognosis because only one case of her series had proven fatal [3]. During the following forty years, however, the entity described by Sophie Spitz was thought to be completely benign, with metastasizing cases being intuitively considered as cases of melanomas simulating Spitz nevus (Spitzoid melanoma) [4]. In 1989, Smith and co-worker described the so-called “Spitz nevus with atypia and metastasis” or “malignant Spitz nevus,” i.e., a kind of lesion showing histopathologic features not enough for a diagnosis of malignancy, yet capable of nodal metastasis, usually with no further dissemination [5]. This apparently contradictory concept was then set forth by Barnhill with the diagnostic category of “metastasizing Spitz tumor” [6], or “atypical Spitz nevus/tumor” [7]. To date, while some opinion leaders maintain that there are only two diagnostic categories (nevus and melanoma) and that every “abnormal” behavior is simply a diagnostic mistake [8], some others suggest that Spitzoid lesions are indeed a “morpho-biologic spectrum” of lesions ranging from benignity to full-blown malignancy [9], and sharing a peculiar genetic profile, with chromosome rearrangements involving kinase fusions [10]. Intermediate lesions within such a spectrum eventually show: an equivocal histomorphology, featuring a diagnostic agreement among experts which is consistently lower than for “conventional” (non-Spitzoid) melanocytic neoplasms [7,11]; peculiar clinical features and behavior with a relatively high incidence in prepubescent patients [12] and a higher incidence of regional (sentinel) node involvement [13] but a better prognosis than “conventional” (non-Spitzoid) melanoma of the same thickness/stage [14] (possible low-grade malignancies [11]). We have already pointed out that dermoscopy seems to allow clinicians to increasingly identify and excise pigmented spindle cell Spitz nevus and Reed nevus (which are basically the same clinicopathological entity), to such an extent that the brown-black plaque-like “variant” is surprisingly becoming the most common (and therefore “typical”) Spitz nevus encountered in clinico-dermoscopic-pathologic studies [15–17]. The present paper will focus on the clinicopathological features of papulonodular hypopigmented Spitzoid lesions, which are “atypical” with a much greater frequency than their plaque-like heavily pigmented counterpart [16,17].

Heterogeneity of dermal OKT6+ cells in inflammatory and neoplastic skin diseases

This immunopathologic study of both normal and pathologic skin specimens (contact dermatitis [CD], lichen planus [LP], cutaneous T cells lymphoma [CTCL], and histiocytosis X [HX]) allowed as to differentiate four types of dermal OKT6+ cells: (1) cells with the same morphologic features as epidermal Langerhans cells (LCs), rarely found in either normal or pathologic dermis; (2) cells structurally similar to LCs but lacking Birbeck granules (BGs), found mainly in CD and LP; (3) larger cells rich in cytoplasmic organelles, only 5% of which contained BGs. They were especially common CTCL; and (4) cells typical of HX.

Simultaneous occurrence of multiple trichoblastomas and steatocystoma multiplex

We present a 55-year old man who, since age 21, progressively developed multiple papules and nodules on the face and upper trunk. Light microscopic examination of some of the neoplasms showed trichoblastomas, while others had histopathological features of trichoepithelioma and steatocystoma. Simultaneous occurrence of multiple trichoblastomas/trichoepitheliomas and steatocystomas, not reported previously, could represent multiple neoplasms involving differentiation toward different components of the folliculosebaceous unit.

Pigmented nodular melanoma: the predictive value of dermoscopic features using multivariate analysis

Nodular melanoma (NM), representing 10–30% of all melanomas, plays a major role in global mortality related to melanoma. Nonetheless, the literature on dermoscopy of NM is scanty.