Dermatoscopic findings and dermatopathological correlates in clinical variants of actinic keratosis, Bowen's disease, keratoacanthoma, and squamous cell carcinoma.

Non-melanoma skin cancer (NMSC), predominantly squamous cell carcinoma (SCC) and basal cell carcinoma, is increasing worldwide. Dermatoscopy, which is one of the non-invasive diagnostic techniques, is important for early diagnosis of NMSC. In this study we aimed to determine dermatoscopic features of keratinocyte derived tumors including actinic keratosis (AK), Bowen's disease (BD), keratoacanthoma (KA), and SCC and correlate the dermatoscopic findings with pathology. A total of 242 lesions from 169 patients were included in the study and dermatoscopic and dermatopathological findings of the lesions were retrospectively studied. Revised pattern analysis was used for the dermatoscopic evaluation. Among 242 lesions, 145 were clinically flat (86 AK, 30 BD, and 29 SCC). Presence of vessels, ulceration, fiber sign, keratin mass, and blood spots decreased the probability of a lesion being AK. When the differential diagnosis was considered between KA and SCC vs AK and BD; vessel presence, ulceration, fiber sign, blood spots, white structureless, keratin, and centred vessels favored the diagnosis of KA and SCC. Our results may contribute to the determination of the lesions to be biopsied in patients with multiple AK on chronically sun damaged skin. In non-pigmented lesions when a final diagnosis cannot be established, knowledge of dermatopathologic and dermatoscopic correlation may significantly assist interpretation of dermatoscopic patterns and clues.

Assessment of Life Quality Index and Treatment Efficacy in Psoriasis Vulgaris Patients Receiving Biologic Therapies in a Turkish Population.

The introduction of biologic therapy has resulted in a major change in treatment efficacies, especially in conventional treatment-resistant psoriasis patients. This study is to assess the efficacy of biologic agents in conventional treatment-resistant patients regarding Psoriasis Area and Severity Index (PASI) and Dermatology Life Quality Index (DLQI) after therapy. Patients were monitored prospectively for 24 weeks after the initiation of etanercept, adalimumab, or ustekinumab therapy. PASI 75/90/100 responses and the number of patients with 0/1 DLQI score were compared. In the patients who used etanercept, adalimumab, and ustekinumab therapies, PASI 75 responses were found as 61.5%, 57.9%, and 84.6%, respectively, in the 12th week, while they were found as 72.7%, 76.9%, and 90.9% in the 24th week, and no statistically significant difference was found between the three groups (P > 0.05). The percentage of patients who had a DLQI score of 0 and 1 were 30.8%, 42.1%, and 38.5% in the 12th week (P = 0.92) and 36.4%, 61.5%, and 45.5% in the 24th week (P > 0.45) for etanercept, adalimumab, and ustekinumab therapies, respectively. As a result, no significant differences were found between biologic agents concerning improvement in both clinical response (PASI and VYA) and quality of life (DLQI and PDI).

Dermatopathological characteristics of dermatofibromas from dermatoscopic clues.

BACKGROUND: Several types of dermatofibroma (DF) have been identified dermatopathologically and with dermatoscopic correlation, the dermatopathology has been predictable in limited studies so far. We identify DFs with specific dermatoscopic structures and determine the respective dermatopathological correlates. METHODS: Dermatoscopic and dermatopathologic correlation of 403 DFs were assessed. A pattern analysis algorithm was used for the dermatoscopic evaluation, each lesion being evaluated according to 10 dermatoscopic patterns with further subdivision of the atypical subtype according and their dermatoscopic similarities to other defined skin lesions. RESULTS: Dermatoscopic pattern analysis showed typical patterns in 83.4% of DFs with atypical patterns in 16.6%. We classified the atypical DFs according to their dermatoscopic similarity to certain lesions: Melanoma-like 2.7%, basal cell carcinoma-like 0.7%, keratoacanthoma-like 0.5%, seborrheic keratosis-like 2.7%; nevus-like 1.5%; nevus sebaceous-like 0.9%; xanthogranuloma-like 0.5%; pyogenic granuloma-like 0.5%; and spitzoid 6.5%. Dermatopathologically 12 subtypes of DFs were identified. It was found that the most common pattern found in fibrocollagenous DFs was the typical pattern with peripheral reticular lines and central white structureless area (32.9%) whereas non-fibrocollagenous types were more likely to have atypical patterns (65.1%). CONCLUSIONS: Our study resulted in new conclusions while supporting some previous findings. The fact that the pathogenesis of DF involves a reactive inflammatory process with fibrosis is likely to have diverse effects on dermatoscopic morphology. If atypical dermatoscopic patterns are identified in suspected DF, excisional biopsy may be prudent.

Leprosy: A great imitator.

In recent years, advances in medical diagnosis and treatment have greatly attracted our attention, whereas some rare diseases, such as leprosy, have not found a place in the medical education curriculum; their existence may even be forgotten. Although the prevalence and incidence rates for leprosy have been significantly reduced as a result of the control strategies of the World Health Organization, new cases still appear. A total of 214,783 new cases were reported from 143 countries during 2016, corresponding to the global new-case detection rate of 2.9 per 100,000 population. Leprosy proves to be a very interesting model due to its immunologic properties. It joins with syphilis, mycosis fungoides, cutaneous tuberculosis, and sarcoidosis as one of the great imitators. The diagnosis of leprosy can be simple and practical, but considering the diagnosis of leprosy in the differential diagnosis is the first requisite again.

Hidradenitis suppurativa: Clinical characteristics and determinants of treatment efficacy.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disorder that causes a significant decline in quality of life. There are numerous treatment options; however, real-life data on the efficacy of these treatments is limited. This study was performed in two centers to describe clinical characteristics and assess treatment outcome in a cohort of 139 patients with HS. Data on demographic and clinical characteristics, Hurley stage and comorbidities were collected from patient charts and evaluated retrospectively. Treatment response was measured with HS clinical response index (HISCR). Mean body mass index was 27.8±4.88. Inflammatory comorbidities were present in 23%. Among first-line drugs systemic doxycycline resulted in 60% HISCR followed by rifampicin-clindamycin combination (46.4%). Isotretinoin had the lowest HISCR (30.7%) in this group. For second-line therapies, all acitretin treated patients achieved response and patients treated with tumor necrosis factor alpha (TNF-α) inhibitors had the highest HISCR. Currently recommended first-line therapies have moderate efficacy in HS. Acitretin appears to be a reasonable alternative for the highly effective TNF-α inhibitors in patients with severe and resistant HS. Overall, these results support that excessive inflammatory response play an important role in pathogenesis of HS.

Epidemiology of Pemphigus in Turkey: One-year Prospective Study of 220 Cases.

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation). The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.

Basosquamous carcinoma: Dermoscopic clues to diagnosis.

Basosquamous carcinoma (BSC) is a rare skin cancer which has areas of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) and a transition zone between them. However, dermoscopic features of BSC are not well described in the published work, except one study. The aim of the present study was to better identify and clarify the dermoscopic findings of BSC in the largest group of patients in the published work and to describe its dermoscopic features according to histopathologically BCC-dominant, SCC-dominant and intermediate categories. Dermoscopic features of 36 histopathologically proven BSC and their dermatopathological correlates were retrospectively analyzed. Dermoscopic features were evaluated by pattern analysis. Keratin mass (91.7%) was the most common dermoscopic feature. Surface scaling (77.8%), ulceration (69.4%), white structureless areas (69.4%), white clods (66.7%) and blood spots on keratin mass (66.7%) were the other frequent findings. Polymorphous vascular pattern consisting of various combinations of branched, serpentine, straight, coiled or looped vessels were detected in 61% of the lesions. BSC has BCC-dominant vascular features together with otherwise SCC-dominant morphology, the common pattern seen in BSC lesions being BCC-dominant polymorphous or monomorphous vasculature, together with dermoscopic findings of keratinization. White circles, known to be a valuable clue to SCC and keratoacanthoma, were present at the same magnitude in BSC in our study. The observed histological correlation of eosinophilic keratin overlying the epithelium which lined follicular infundibulae in these tumors, provides a plausible new perspective on dermoscopic white circles.

Long-term results of rituximab-intravenous immunoglobulin combination therapy in patients with epidermolysis bullosa acquisita resistant to conventional therapy.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare subepidermal bullous disease. Long-term remission in this disease is difficult using current treatments, unlike that in patients with other autoimmune bullous diseases. OBJECTIVE: We retrospectively evaluated the effectiveness and side effects of rituximab-intravenous immunoglobulin (IVIg) combination treatment in five patients with EBA resistant to conventional treatment. PATIENTS AND METHODS: Rituximab (375 mg/m2) was administered for four consecutive weeks to four patients, and their treatment continued with IVIg at a dose of 2 g/kg/month. One patient received two cycles of rituximab for three consecutive weeks, IVIg in the fourth week, followed by monthly IVIg administrations as in the other patients. RESULTS: The total number of IVIg therapy cycles ranged from 10 to 26 (mean 19.4). Mean skin involvement, mucosal involvement, and disease severity scores decreased after a mean follow-up of 22.6 months (range, 10-28 months). In an analysis performed during months 24-28, the number of CD19-positive B cells was found to be below the normal reference range in four patients. LIMITATIONS: This was a retrospective study with a limited number of patients. CONCLUSION: Rituximab-IVIg combination treatment seems to be effective and safe for treating patients with EBA resistant to conventional treatments.

Dermoscopic changes in melanocytic nevi in patients receiving immunosuppressive and biologic treatments: results of a prospective case-control study.

BACKGROUND: The immune system restrains benign melanocytic lesions, however the relationship between immunosuppression and changes in nevi is not known. OBJECTIVES: We sought to investigate the development of new nevi in patients using immunosuppressive agents, to evaluate any size or dermoscopic changes in existent nevi, and to evaluate any risk of developing melanoma. METHODS: There were 266 melanocytic lesions in 103 patients undergoing immunosuppressive therapy and 180 melanocytic lesions matched for age, sex, race, and Fitzpatrick skin type in 60 healthy control subjects. RESULTS: Nevus counts increased from baseline in the treatment group (P < .001) as did nevus size (P = .046) but the increase compared with the control group only remained statistically significant for nevus numbers (P = .001). There was a statistically significant appearance of dermoscopic changes in the nevi of immunosuppressed patients compared with healthy control subjects (P < .001). Ten lesions were excised including 6 because of significant dermoscopic change during treatment and all were benign. LIMITATIONS: Follow-up duration was short and the number of patients was small. CONCLUSION: Immunosuppressive therapy was associated with increased nevus counts and changed dermoscopic appearance but as none of the changed and subsequently excised nevi were malignant, continued monitoring for invasive features is a reasonable alternative to excision.

Dermatoscopic findings of atrophic dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally aggressive mesenchymal tumor with a high local recurrence rate. Atrophic DFSP is a rare variant of DFSP characterized by a non-protuberant lesion. We report on a 23-year-old female, who presented with an atrophic, asymptomatic macule on the right side of her back 2 cm in diameter. Dermatoscopic examination revealed homogenous pigment network on a purplish erythematous background. The histopathological finding of the incisional biopsy material was consistent with DFSP. To our knowledge, this is the second case of atrophic DFSP discussing the dermatoscopic features of this relatively rare condition.

Comparison of dermatoscopic diagnostic algorithms based on calculation: The ABCD rule of dermatoscopy, the seven-point checklist, the three-point checklist and the CASH algorithm in dermatoscopic evaluation of melanocytic lesions.

Dermatoscopic analysis of melanocytic lesions using the CASH algorithm has rarely been described in the literature. The purpose of this study was to compare the sensitivity, specificity, and diagnostic accuracy rates of the ABCD rule of dermatoscopy, the seven-point checklist, the three-point checklist, and the CASH algorithm in the diagnosis and dermatoscopic evaluation of melanocytic lesions on the hairy skin. One hundred and fifteen melanocytic lesions of 115 patients were examined retrospectively using dermatoscopic images and compared with the histopathologic diagnosis. Four dermatoscopic algorithms were carried out for all lesions. The ABCD rule of dermatoscopy showed sensitivity of 91.6%, specificity of 60.4%, and diagnostic accuracy of 66.9%. The seven-point checklist showed sensitivity, specificity, and diagnostic accuracy of 87.5, 65.9, and 70.4%, respectively; the three-point checklist 79.1, 62.6, 66%; and the CASH algorithm 91.6, 64.8, and 70.4%, respectively. To our knowledge, this is the first study that compares the sensitivity, specificity and diagnostic accuracy of the ABCD rule of dermatoscopy, the three-point checklist, the seven-point checklist, and the CASH algorithm for the diagnosis of melanocytic lesions on the hairy skin. In our study, the ABCD rule of dermatoscopy and the CASH algorithm showed the highest sensitivity for the diagnosis of melanoma.

Infantile lepromatous leprosy with vulvar localization.

Reports of leprosy during the first year of life are uncommon. In the early diagnosis, the spectrum for most of the children is reported to be largely confined to tuberculoid (TT), borderline tuberculoid (BT), mid-borderline (BB), and indeterminate forms of leprosy. We report a one-year-old female infant presenting numerous erythematous papules on her labium majus and pale flat macules on her chin and right cheek for about four months. A biopsy revealed changes typical for lepromatous leprosy (LL). Infantile leprosy and the route of transmission are discussed here.

Post-renal transplantation Leser-Trélat sign associated with carcinoma of the gallbladder: a rare association.

The sign of Leser-Trélat represents the sudden appearance of multiple seborrheic keratoses in association with an underlying malignancy. The most common associated neoplasms belong to the gastrointestinal tract, mainly the stomach and colon. In the literature, there is only one case of gallbladder carcinoma associated with the Leser-Trélat sign. Thus, the hallmark of our patient is post-renal transplant malignancy-associated Leser-Trélat, which has not been reported before. Here, we report on a 57-year-old man who presented with a sudden increase in the number and size of seborrheic keratoses, particularly on sun-exposed areas 24 years after renal transplantation. The search for an underlying malignancy showed the presence of an adenocarcinoma of the gallbladder which had metastasized to the liver.

Mycosis fungoides and Sezary syndrome: therapeutic approach and outcome in 113 patients.

BACKGROUND: Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common forms of cutaneous T-cell lymphoma (CTCL). Various topical and systemic therapeutic alternatives are available, but there is no standard or definite curative treatment regimen. When making a decision about the appropriate treatment modality, the age and compliance of the patient, stage of the disease, treatment accessibility, and previous treatment history should be considered. AIM: To determine the therapeutic response of patients with MF and SS to different treatment modalities. Patients were evaluated with respect to their clinical and demographic features. METHODS: One hundred and thirteen patients diagnosed clinically and dermatopathologically with MF and SS between March 1984 and June 2001 were included in the study. RESULTS: Of the 113 patients studied, 110 had a diagnosis of MF and three had a diagnosis of SS; 101 patients (89.4%) were diagnosed with early stage (IA, IB, IIA) and 12 (10.6%) with late stage (IIB, III, IVA, IVB) disease. The age at diagnosis varied between 12 and 81 years (mean, 45.6+/-15.8 years). Fifty-five (48.7%) patients were male and 58 (51.3%) were female. The duration of the skin lesions varied between 1.5 months and 32 years (mean, 6.1 years). Psoralen plus UVA (PUVA) was the most commonly used initial treatment modality in early stage disease (91%), with a complete remission (CR) rate of 80.4%. With PUVA+interferon-alpha (INF-alpha) treatment, CR was 57% in the early stages and 33.3% in the late stages. For late stage disease, systemic therapies, such as pentostatin, gemcitabine, and fludarabine, alone or in combination with INF-alpha, were preferred. Of the 113 patients, eight (7% of the total and 57.1% of the advanced stage cases) died of MF; 21.4% of the late stage patients showed partial remission and 14.2% showed CR. None of the patients diagnosed with early stage disease died of MF, but two (1.9%) progressed to late stage disease. CONCLUSIONS: PUVA and PUVA+INF-alpha are effective treatment modalities, especially for early stage MF. Once the disease has progressed, both MF and SS are very resistant to treatment regimens, including chemotherapeutic agents. It is important to diagnose and treat these diseases, especially MF, in the early stages for lasting remission.

Juvenile non-hyaline fibromatosis: juvenile hyaline fibromatosis without prominent hyaline changes.

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease of the connective tissue. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, osteolytic bone lesions and flexion contractures of the large joints. Here, we report a 14-year-old girl with characteristic clinical features of JHF with early fatal outcome. Dermatopathologic examination of the early lesions however constantly lacked the so-called hyalin changes in multiple skin biopsies. According to our experience; dermatopathological features of this entity is not often and always consists of classical hyalinisation. Only larger lesions with long duration should expected to be exhibiting those features. Therefore we suggest that; JHF may often present itself as Juvenile Non-Hyaline Fibromatosis: JHF without prominent hyaline changes. And thus this fact should not change the actual diagnosis and prognostic implications.

Treatment of Basal Cell Carcinoma Located in the Head and Neck Region with Intralesional Interferon alpha-2a : Evaluation of Long-Term Follow-Up Results.

OBJECTIVE: Intralesional injections of interferon have been reported to provide successful results in the treatment of basal cell carcinoma. However, there are only a few reports describing the long-term efficacy of this therapy. The aim of our study was to evaluate the efficacy and long-term results of interferon alpha-2a (IFNalpha-2a) in the treatment of basal cell carcinoma. METHODS: Twenty dermatopathologically proven basal cell carcinoma lesions were treated with intralesional IFNalpha-2a injections three times weekly for 3 weeks. The dose per injection was 1.5 x 10(6)IU if the lesion was <2cm in diameter and 3.0 x 10(6) IU if it was >/=2cm. Eight weeks after the last injection, the lesion sites were rebiopsied and all cases were reevaluated both clinically and dermatopathologically. Patients with complete cure were followed up for 7 years to determine the long-term results. RESULTS: Eleven lesions (55%) showed complete clinical and dermatopathological remission, six lesions (30%) showed partial remission, and two lesions (10%) showed no response. One lesion (5%) increased in size during the treatment. No serious adverse effects were observed. During the follow-up period there was only one recurrence, at the fifth year. CONCLUSION: Treatment with intralesional IFNalpha-2a was shown to be an effective therapeutic option for basal cell carcinoma, with low recurrence rates in long-term follow-up.

Juvenile colloid milium associated with conjunctival and gingival involvement.

Juvenile colloid milium is an uncommon cutaneous disease characterized by translucent papules distributed on sun-exposed areas with early onset. Association of juvenile colloid milium with conjunctival and gingival deposits is uncommon and interesting. We report a case of juvenile colloid milium associated with conjunctival and gingivai deposits of an amyloid-like homogeneous eosinophilic material. It seems that all 3 of these in our patient may be different expressions of the same pathologic disease.