Folliculotropic mycosis fungoides: Clinical characteristics, treatments, and long-term outcomes of 53 patients in a tertiary hospital.

Folliculotropic mycosis fungoides (FMF) is characterized by a broad clinical spectrum and worse prognosis compared to classical MF. This study aimed to evaluate the clinical characteristics, treatment modalities and long-term outcome and risk factors for progression and survival of FMF patients. We conducted a single-center retrospective study and reviewed 53 patients diagnosed with FMF between 1990 to 2019 in a referral center at Ankara University, Turkey. Regarding to stage at diagnosis, 24 patients (45.3%) had advanced-stage disease (≥IIB). Follicular papules was observed in 66% and alopecia in 49.1% of the cases. Forty-three patients (81.1%) suffered from pruritus. The majority of the patients (92.4%) had at least one systemic therapy. Complete remission was achieved in 24.5% of the patients. The median time of overall survival (OS) was 50 months (range 9-324 months) and 5-year and 10-year OS was 83% and 69%, respectively. Twenty-eight (52.3%) patients progressed to more advanced stages and seven (13.2%) patients died due to MF during the follow-up period. FMF is associated with a progressive course and in most patients, skin-directed therapies were found to be inefficient to control the disease and multiple systemic therapeutic agents were required to control the disease.

Sweet's syndrome under ipilimumab therapy and a brief comparison of the cases in literature.

INTRODUCTION: Ipilimumab is an anti-cytotoxic T lymphocyte antigen 4 (CTLA-4) antibody. Ipilimumab has shown improvement in overall survival in patients with advanced melanoma. Because ipilimumab activates the immune system against the tumor, ipilimumab is associated with adverse events related to immune system activation. Immune-associated side effects are frequently seen in the gastrointestinal system and skin. Sweet's syndrome (SS) is an uncommon inflammatory disorder. Some drugs or malignancy can cause SS. Only a few case reports have been reported of ipilimumab-associated SS. CASE: A 53-year-old female with metastatic melanoma was treated with ipilimumab. After the fourth cycle, she developed painful lesions on her legs and hands. The pathologic biopsy of the lesions revealed neutrophilic dermatosis consistent with SS.Management and outcome: The patient was treated with 60 mg/day of prednisone for four days, nonsteroidal anti-inflammatory drugs and inhaler bronchodilator and steroid. She had symptomatic relief at the beginning of treatment. The prednisone doses were quickly tapered every three days. When the patient was treated with 10 mg/day of prednisone for three days, the skin nodules recurred. Prednisone 40 mg per day was re-started and then a slower taper by decreasing by 10 mg/day every week was instituted. After one-month treatment the prednisone dose was given as a 5 mg doses for one week and then stopped. No new lesions recurred after slower taper of prednisone. CONCLUSION: Herein we report a case presented with SS under ipilimumab therapy. Melanoma patients treated with ipilimumab can develop SS. The clinicians should be aware of this condition.

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.

A Novel PTCH1 Frameshift Mutation Leading to Nevoid Basal Cell Carcinoma Syndrome.

Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a rare multisystemic autosomal dominant disorder typically presenting with cutaneous basal cell carcinomas, multiple keratocysts, and skeletal anomalies. NBCCS is caused by heterozygous mutations in the PTCH1 gene in chromosome 9q22, in the PTCH2 gene in 1p34, or the SUFU gene in 10q24.32. Here, we report on an 18-month-old boy presenting with medulloblastoma, frontal bossing, and multiple skeletal anomalies and his father who has basal cell carcinomas, palmar pits, macrocephaly, bifid ribs, calcification of falx cerebri, and a history of surgery for odontogenic keratocyst. These clinical findings were compatible with the diagnosis of NBCCS, and a novel mutation, c.1249delC; p.Gln417Lysfs*15, was found in PTCH1 causing a premature stop codon.

Spitz nevus arising upon a congenital glomuvenous malformation.

There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM.

International Dermoscopy Society criteria for non-neoplastic dermatoses (general dermatology): validation for skin of color through a Delphi expert consensus.

BACKGROUND: The International Dermoscopy Society (IDS) recently released a set of five basic dermoscopic parameters (vessels, scales, follicular findings, "other structures," and specific clues) encompassing a total of 31 subitems to standardize the use of dermoscopy in non-neoplastic dermatoses, yet they have been developed taking into account Caucasian/Asian skin, with consequent possible limitations if used in dark skin. OBJECTIVES: To validate the abovementioned criteria for the use in dark-skinned patients (phototypes IV-VI) through an expert consensus. METHODS: The two-round Delphi method was adopted, with an iterative process consisting of two rounds of email questionnaires. Potential panelists were recruited via e-mail from all over the world based on their expertise on dermoscopy of non-neoplastic dermatoses in skin of color. RESULTS: Twenty-two panelists took part in the validation process. All of the five originally proposed parameters and subitems reached agreement during the first round, aside from "follicular red dots." Additionally, during round 1, five new subitems were proposed (perifollicular scales distribution, follicular openings obliteration, broken hairs, eccrine pigmentation, and eccrine ostia obliteration), along with the possibility to change the denomination of parameter 3 (from "follicular findings" to "follicular/eccrine findings") and split it into two subparameters ("follicular findings" and "eccrine findings"). All such proposals reached agreement during the second round and therefore were included in the final list, for a total of 37 items. CONCLUSIONS: Although nearly all the dermoscopic criteria originally proposed by the IDS are applicable even to darker phototypes, several additional variables need to be assessed.

CD8+ cytotoxic mycosis fungoides: a retrospective analysis of clinical features and follow-up results of 29 patients.

BACKGROUND AND OBJECTIVE: Less than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype. This study aimed to evaluate the clinical characteristics, treatment modalities, and clinical course in CD8+ MF patients. METHODS: In a retrospective analysis of 353 MF patients in a referral center at Ankara University, Turkey, 29 patients that were diagnosed with CD8+ MF were included in the study. RESULTS: CD8+ MF cases constituted 8.2% of all MF patients. The age at the time of diagnosis ranged between 6 and 81 years with a median value of 46 years. The female-to-male ratio was 1.41. Patients presented with erythematous scaly (69%), hyperpigmented (58.6%), poikilodermic (17.2%), and hypopigmented (17.2 %) patches/plaques. The most common sites of involvement were the trunk and lower extremities. The most common comorbidity was hypertension (24.1%, n: 7) with 13 patients (44.8%) having a history of at least one autoimmune disease. At the time of diagnosis, 93.2% of the patients had early-stage disease, and 6.8% of the patients had advanced stage. The mean follow-up period was 6.68 ± 6.04 years (range 1-28 years). Most of the patients were treated with skin-directed therapies. Complete remission was achieved in 17 (58.6%) patients, eight (27.6%) patients had partial remission, and four (13.8%) patients had stable disease. CONCLUSIONS: We concluded that CD8+ MF is associated with an indolent course and in most patients, skin-directed therapies were found to be efficient to control the disease.

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.