Clear cell sarcoma of soft tissue with eccrine differentiation: A case report and review of the literature.

Clear cell sarcoma of soft tissue (CCSST) is a deep soft tissue tumor presenting in the extremities of young adults. Histopathologically, nests and sheets of polygonal cells with clear to eosinophilic cytoplasm separated by fibrous septa as well as occasional "wreath-like" giant cells are visualized. However, CCSST has been noted to have atypical histopathological features, such as epidermotropism or myxoid differentiation, or occurrence at unusual sites. Here, we present a case of eccrine ductal differentiation in CCSST. The patient, a 21-year-old woman, presented with a lump of 10-year duration sized 3 × 5 cm on the plantar surface of the fourth and fifth interdigital spaces. There had been an increase in size as well as pain and redness over 6 years. Besides the characteristic findings, there were ductal structures in continuity with the upper dermis indicative of ductal differentiation. The tumor stained positively for S100, HMB45, and succinic dehydrogenase; ducts stained positively for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). CCSST was confirmed with cytogenetic analysis showing the translocation associated with EWSR1-ATF1 fusion gene. Therefore, ductal differentiation is a unique finding that should be considered when evaluating for CCSST.

Histopathological characteristics and CD163 immunostaining pattern in fibrous papule of the face.

BACKGROUND: Signs of inflammation including epidermal interface changes, spongiosis, and dermal inflammation as well as pagetoid dyskeratosis are rarely described in fibrous papule (FP). We aimed to describe the inflammatory parameters, the rate of pagetoid dyskeratosis, along with CD163 immunohistochemical staining as an adjunctive diagnostic tool in FP. METHODS: Histopathology samples of all biopsy-proven FP cases were retrieved from archives and investigated for inflammatory parameters, presence of pagetoid dyskeratosis, as well as CD163, CD10, and CD34 immunostaining pattern of dermal spindle/stellate or multinucleate cells (graded from 0 to 4). RESULTS: Thirty-two cases of FP were identified. A high rate of inflammatory parameters including interface changes (20/32), spongiosis (31/32), and dermal lymphocytic inflammation (31/32) were detected. Pagetoid dyskeratosis was identified in eight out of 32 cases (25%). A grade 4 staining revealing a strong dendritic pattern was confirmed in all FP cases with CD163 immunohistochemistry including atypical variants such as granular FP, compared with CD10 (11/32) and CD34 (3/32). CONCLUSION: The dendritic cellular proliferation in FP may represent an inflammatory response to various stimuli; pagetoid dyskeratosis is a relatively common and underrecognized epidermal feature and CD163 immunostaining may be used as an adjunctive diagnostic tool in unusual histopathological subtypes.

Demographic, clinical and dermoscopic features of melanocytic nevi on the scalp.

BACKGROUND: The scalp is a special anatomical area and dermoscopic findings of this region may significantly differ from other body parts. OBJECTIVE: To investigate and compare the clinical and dermoscopic patterns of scalp melanocytic nevi in patients ≤15 years of age and above, and to analyse their relevance to demographic features, atypical mole syndrome (AMS) and total body nevus count (TBNC). METHODS: In this retrospective cohort study, the clinical data and dermoscopic images of patients with scalp melanocytic nevi were retrieved, reviewed and analysed. Demographic, clinical and dermoscopic features were compared in patients ≤15 years of age and above. RESULTS: A total of 196 scalp melanocytic nevi in 126 patients (female/male:64/62; ≤15/>15 years of age: 49/77) with a median age of 18.5 years (range 0-72) were evaluated. Statistically, the globular pattern was significantly higher in all age groups, and the papillomatous pattern was significantly lower in patients ≤15 years of age (P = 0.008 and P = 0.005, respectively). The eclipse pattern was significantly higher, and the homogenous pattern was significantly lower in patients ≤15 years of age with AMS (P = 0.003 and P = 0.014, respectively). Finally, patients ≤15 years of age with 50 to 100 TBNC had a higher eclipse pattern than those with 0 to 25 TBNC. CONCLUSION: The findings of this retrospective study might implicate that children with eclipse pattern of scalp melanocytic nevi might be 'moley' in the future with an impending risk of AMS. This hypothesis requires confirmation in future prospective studies on a larger cohort of patients.

Dermatoscopy as an adjunctive diagnostic tool in aquagenic syringeal acrokeratoderma.

Aquagenic syringeal acrokeratoderma (ASA) is a rare skin disorder of the palms and/or soles, characterized by whitish papules with occasional pruritus or pain sensation. Herein we report a 27-year-old man with a diagnosis of ASA based on clinical and histopathological features, and describe the dermatoscopic features consistent with threefold enlarged sweat duct pores compared with a normal-looking palmar skin area. As far as we are aware, dermatoscopic features of ASA have not been reported so far.

Acquired perforating dermatosis successfully treated with photodynamic therapy.

Acquired perforating dermatosis is a rare perforating skin disorder characterized by intensely pruritic papules or nodules with central adherent plugs mainly observed on the lower extremities and transepidermal elimination of collagen bundles. Treatment of acquired perforating dermatosis is a matter of debate and conventional treatment options including topical and systemic retinoids, topical corticosteroids and keratolytics, ultraviolet B phototherapy, psoralen plus ultraviolet A (PUVA), allopurinol and cryosurgery show mixed results. Herein, we describe a 60-year-old woman with a diagnosis of acquired perforating dermatosis secondary to diabetes mellitus in whom we achieved excellent results with photodynamic treatment. As far as we are aware, this is the first case report of photodynamic treatment for acquired perforating dermatosis.

Secondary syphilis with an interstitial granuloma annulare-like histopathologic pattern.

A granulomatous tissue response may be observed in untreated, long-standing lesions of secondary syphilis. In addition to poorly defined granulomatous inflammation, leprosy-like sarcoidal and palisaded granulomatous inflammation has been documented in literature reports of lues. Herein, we report a 47-year-old man who presented with a 3-month history of a generalized non-pruritic macular and papular rash located on the trunk and extremities. Histopathologically, there was an interstitial arrangement of histiocytes with occasional multinucleated giant cells positioned among collagen bundles without associated necrosis, thereby closely mimicking interstitial granuloma annulare. A clue to the diagnosis was the presence of admixed plasma cells. To the best of our knowledge, this is the first reported case of secondary syphilis showing an interstitial granulomatous pattern mimicking interstitial granuloma annulare.

Secretan Syndrome: A Fluctuating Case of Factitious Lymphedema.

Secretan syndrome (SS) is a recurrent or chronic form of factitious lymphedema that usually affects the dorsal aspect of the hand and is accepted as a subtype of Munchausen syndrome. Secretan syndrome usually is induced by compression of the extremity by tourniquets, ligatures, cords, or similar equipment. This unconsciously motivated and consciously produced lymphedema is an expression of underlying psychiatric disease. Herein, we present a fluctuating case of SS involving the hand, with periods of severe lymphedema and those of complete amelioration, parallel to stressful and peaceful periods in the patient's personal life. We briefly review the current literature on SS to increase awareness among dermatologists, and we present information on the clinical portrait, diagnosis, and appropriate management of this peculiar and underreported disorder.

Warty and clear-cell Bowen's disease successfully treated with photodynamic treatment.

Clear cell Bowen's disease (BD) is a rare histopathological subtype of BD, characterized by epidermal atypical keratinocytes with clear-cell changes exceeding 80% of the tumor population. Warty and clear cell Bowen's disease (WCCBD) is a recently described variant of clear cell BD, with verrucous and hyperkeratotic surface changes in addition to pathological features consisting of an extremely acanthotic epidermis, hyperkeratosis and clear-cell changes. Herein we present a 72-year-old man with a 1-year history of tumoral lesion located on the left cheek with a diagnosis of WCCBD, in whom we achieved excellent result with photodynamic treatment. To the best of our knowledge, this is the second reported case of this entity.

Scleredema treated with broad-band ultraviolet A phototherapy plus colchicine.

Scleredema is an uncommon condition characterized by induration of skin. The pathogenesis is not known, although the increased expression of collagen-producing fibroblasts in the skin has been demonstrated. Scleredema has been difficult to treat with scattered reports of success with bath psoralen plus ultraviolet A (PUVA), cream PUVA, UVA1 and others. The use of low-dose broad-band UVA phototherapy was found to be effective in the treatment of morphea. Colchicine has been shown to interfere with collagen synthesis and is used successfully in scleroderma. We report the first case of scleredema that improved markedly with low-dose broad-band UVA plus colchicine treatment.

Identification of human papilloma virus type 6 in psoriasiform keratosis.

Psoriasiform keratosis is a recently reported unique clinicopathological entity characterized by the appearance of a solitary lesion mimicking seborrheic keratosis, actinic keratosis, or squamous cell carcinoma and histopathologic features closely resembling psoriasis. We report a 74-year-old woman presenting with a solitary, scaly lesion on the dorsum of the left arm of several months duration and histopathologic findings of psoriasiform acanthosis of the epidermis with thinning of the suprapapillary plates and intraepidermal spongiform neutrophilic pustules, consistent with a diagnosis of psoriasiform keratosis. On additional work-up investigation, human papilloma virus type 6 was detected in the tissue examined by polymerase chain reaction technique, an association not described before.

Expressions of p53, cyclinD1 and histopathological features in basal cell carcinomas.

BACKGROUND: We planned this study to analyze probable associations between p53, cyclinD1, Ki67 and histopathological features in basal cell carcinomas (BCC). METHODS: Histological differentiation types, histological growth patterns and tissue responses were analyzed in 50 cases of BCC. In immunohistochemical analysis, p53, cyclinD1 and Ki67 antibodies were investigated. P53 expression was evaluated based on a cut-off value of 25% positivity. CyclinD1 expression was graded from 0 to 3+ according to the percentage of positive nuclear staining. The percentage of positively staining cells for Ki67 was recorded. RESULTS: The following significant correlations were detected. Solid infiltrative type differentiation was related to the infiltrative histological growth pattern. The rates of p53 positivity and severe fibrosis in the groups of mixed and infiltrative growth patterns were higher than others. Besides, p53-positive cases showed more severe fibrosis and had a higher mean value for Ki67 index. Epidermal p53 and cyclinD1 clones in normal epidermal areas adjacent to tumors were noticed in 42% and 52% of the cases, respectively. CONCLUSIONS: P53 expression seems to be related to Ki67 index and some histopathological features of BCC, such as infiltrative histological growth pattern and probably fibrosis.

Comparison of dose-related ocular side effects during systemic isotretinoin administration.

PURPOSE: To compare the effects of high- (Group 1) and low-dose (Group 2) isotretinoin treatments for acne vulgaris on lacrimal functions and other ocular complications. METHODS: Twenty-six patients receiving high-dose (>0.5 mg/kg per day) systemic isotretinoin treatment and 25 patients treated with low-dose systemic isotretinoin (<0.5 mg/kg per day) underwent complete ophthalmologic assessment of both eyes before treatment, at days 45 and 90 of treatment, and 1 month after the completion of treatment, together with a microbiologic evaluation of conjunctival flora, tear film break-up time (BUT), and anesthetized Schirmer test of the right eye of each patient. RESULTS: When the results of the anesthetized Schirmer test for Groups 1 and 2 were compared (pretreatment, days 45 and 90 of treatment, and 1 month after treatment), there was no statistically significant difference between the groups (p > 0.05). Although BUT did not differ significantly between the two groups before treatment (p >0.05), there was a statistically significant decrease in BUT in Group 1 when compared with Group 2 at days 45 and 90 of treatment (p <0.05). One month after the completion of treatment, there was no difference in BUT between the two groups (p >0.05). No difference in Staphylococcus aureus colonization was detected between the two groups at days 45 and 90 of treatment (p >0.05). CONCLUSIONS: During systemic isotretinoin treatment, eye dryness was related to the dose used, at least during the period of treatment. Conversely, the rate of conjunctival S aureus colonization was unrelated to the dose of isotretinoin.

An unusual case of Wegener's granulomatosis with tongue involvement.

Wegener's granulomatosis is a type of vasculitis characterized by necrotizing granulomatosis respiratory tract lesions and necrotising glomerulonephritis. Nasal, lung and renal biopsies and positive antineutrophil cytoplasmic antibody (c-ANCA) analysis is helpful in diagnosis. Early diagnosis and treatment is critical in prognosis. A 42 years-old male had dyspnea, coughing, hemoptysis, fatigue, loss of appetite, night sweating and arthralgia. Violaceous palpable, purpuric lesions were detected on the sublingual region of the mouth. On chest X-ray, there was nonhomogeneous infiltration in the parenchyma of both lungs. There was alveolar density involving upper lobes of both lungs detected in thoracal computerized tomography as well as patchy densities on the right lung upper-middle lobes. A nasal mucosa biopsy showed strongly destructive vasculitis. c-ANCA test was positive. We report an unusual case with Wegener's granulomatosis, characterized by a rare presentation of tongue involvement and atypical lung radiology with alveolar opacity.