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Background: Unilesional folliculotropic mycosis fungoides (UFMF) has been rarely reported. Objective: The aim of this study was to describe our experience with UFMF. Methods: Data were collected on all patients with clinicopathological UFMF who attended the Department of Dermatology of a tertiary university-affiliated medical centre in 1996-2013 and were followed prospectively. Results: The sample included seven patients (five male, two female) of mean age 38 years at diagnosis; two were aged <18 years. The lesion presented as a solitary patch/plaque with follicular accentuation in five patients, an infiltrated plaque devoid of hair in one and with follicular nodules in one. Four patients had alopecia, and one, secondary anetoderma. The lesion was located on a limb in four patients, the trunk in two, and the face in one. In all cases, the atypical folliculotropic lymphocytes expressed mainly surface CD4+. Monoclonality was detected in three of the six patients analysed. Treatment consisted of localized electron beam in five patients, all of whom had a complete response (CR), and excision in one patient. The remaining patient, a 9-year-old boy, was treated with topical psoralen and UVA with CR. The duration of follow-up was 0.5-10 years (mean 4). There were no recurrences in six patients and local recurrence in one. Conclusion: UFMF presents at a young age, usually with early disease clinical morphology. The treatment goal should be cure. Our experience indicates an excellent prognosis of early UFMF with no multifocal/internal spread. [ABSTRACT FROM AUTHOR]

Background Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature. Objectives To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group. Methods We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007. Results Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16·6 years (range 16–17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission. Conclusions In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group. [ABSTRACT FROM AUTHOR]

Wells' syndrome is a multifaceted dermatosis with a wide morphological spectrum, ranging from characteristic cellulitis-like erythema and wheals to an unusual presentation of vesicles and bullae. We describe a patient in whom Wells' syndrome presented as an insect-bite-like eruption and was associated with underlying mantle-cell lymphoma. We recommend meticulous investigation of patients diagnosed with Wells' syndrome manifesting as an insect-bite-like eruption. [ABSTRACT FROM AUTHOR]

Skin ulcers are commonly described in the juvenile form of dermatomyositis; however, in the adult form they are rarely described. We report on a patient with adult dermatomyositis, deep cutaneous ulcers in the skin folds and livedo reticularis eruption. [ABSTRACT FROM AUTHOR]

We report the case of a 65-year-old women with history of noninsulin-dependent diabetes mellitus and hyperparaproteinemia kappa (IgM) who was admitted to the Dermatology Department of Rabin Medical Center because of painful erythema of the left leg associated with high fever (39 °C), which developed approximately 2 weeks before admission, and purpuric lesions over the lower extremities, which developed approximately 3 days before admission. Biopsy obtained from one of the purpuric lesions revealed changes characteristic of leukocytoclastic vasculitis, and DIF from the purpura revealed perivascular precipitates of C3; antistreptolysin antibody titer was 1 : 400. HBV, HCV serology, C3, C4, ANA and cryoglobulins were within normal limits. To the best of our knowledge, this is the first case report indicating an association between erysipelas and leukocytoclastic vasculitis. [ABSTRACT FROM AUTHOR]

Background: The literature on paediatric mycosis fungoides (MF) and especially its folliculotropic variant (FMF) is sparse. Objectives: To describe the clinical manifestations, treatments, outcomes and long‐term course of paediatric MF, including FMF. Methods: A retrospective analysis was conducted of all consecutive MF patients diagnosed at ≤18 years attending two medical centres in 1995–2015. Results: The cohort included 71 patients, all but two of whom had early‐stage disease: hypopigmented (55%), folliculotropic (42%) and classical MF (39%), alone or in combination. The head and neck area were involved in 43% of patients with early‐stage FMF compared to 12% of the non‐FMF group (P = 0.004). There was no difference in the involvement of other body areas between the groups. Pruritus, although mild, was more often recorded among patients with early‐stage FMF compared to non‐FMF (58% vs. 29%, respectively, P = 0.02). Complete response (CR) was achieved in 60 of the 69 patients with early‐stage MF (87%) after an average of 1.8 treatment modalities. NBUVB was the most administered treatment to non‐FMF patients with CR rates of 63% vs. 29% of FMF patients (P = 0.04). Systemic/bath PUVA and UVA+NBUVB were the most administered treatments to FMF patients with CR rates of 60% vs. 81% for non‐FMF patients (P = 0.17). During a mean follow‐up of 9.2 years (range 1–24), stage progression was observed in four (6%) of the patients with early‐stage disease, two of whom (all FMF) to advanced stage. Conclusions: Paediatric MF presents as an early‐stage disease with over‐representation of hypopigmented and FMF variants. NBUVB and UVA‐based therapies yield good response rates in non‐FMF and FMF patients, respectively. Disease course is indolent, and even on relatively long follow‐up, it has a very low progression rate from early to advanced‐stage disease, occurring in patients with FMF. We propose a treatment algorithm for paediatric MF. [ABSTRACT FROM AUTHOR]

Background Lentigines are a common pigmentary disorder in adults and in patients treated by psoralen and ultraviolet A (PUVA) radiation. Their appearance following treatment with narrow-band ultraviolet B (NB-UVB) radiation has been reported in only two patients. Objective To describe the clinical and histological features of NB-UVB-induced lentigines their relation to dosimetry and the course of the eruption in patients with mycosis fungoides (MF). Methods The files of all patients with MF treated in our department in 2003-2010 were searched to identify those in whom lentigines appeared following monotherapy with NB-UVB radiation. Results Of the 73 patients with early stage MF identified, 10 met the study criteria. Lentigines were detected in skin previously involved by MF in seven patients, and in both involved and uninvolved skin in three patients. They appeared during therapy in three patients, after a mean of 56 exposures (range 50-61), and several months (mean 7.8) following completion of treatment in seven patients, after a mean of 69 exposures (range 32-157). Histopathological study of lesions from five patients revealed basal hyperpigmentation relative to adjacent normal-looking skin. Two lesions had a slight increased number of normal-looking melanocytes on immunohistochemical staining with melanoma cocktail. One lesion had elongated rete ridges. The lesions persisted throughout follow-up (mean 26.7 months) in 8 patients. Conclusions Patients with MF treated with NB-UVB may acquire lentigines. As opposed to PUVA-induced lentigines which are a known common side-effect of long-term treatment, NB-UVB-induced lentigines are uncommon but appear earlier, even after a few months of treatment. [ABSTRACT FROM AUTHOR]

Background Idiopathic guttate hypomelanosis (IGH) is a common pigmentary disorder, the aetiology and pathogenesis of which are largely unknown. The appearance of IGH-like lesions during phototherapy has been reported previously in only one patient. Objective To describe the clinical and histological features of phototherapy-induced IGH-like lesions, their relation to ultraviolet dosimetry and the course of this eruption in patients with mycosis fungoides (MF). Methods The files of all patients with MF who underwent phototherapy in our centre from 1992 to 2008 were searched to identify those in whom IGH-like lesions appeared during treatment. Results Among 87 patients with early-stage MF who underwent phototherapy, seven acquired IGH-like lesions during monotherapy with narrow-band ultraviolet B (NB-UVB; four patients) or psoralen and ultraviolet A (PUVA; three patients). All but one had a light complexion. The lesions appeared in areas exposed to ultraviolet light, and not exclusively on the skin previously involved by the disease. The mean number of exposures until appearance of the lesions was 92 for NB-UVB and 137 for PUVA. Biopsy study showed a decreased number of melanocytes. Phototherapy was discontinued in four patients, of whom three showed a partial or complete disappearance of the IGH-lesions. The other three patients are still receiving phototherapy, with no change in their IGH-like lesions. Conclusions Phototherapy may induce an eruption bearing similar clinical and histopathological features to IGH. The eruption is rare, appears to emerge only after prolonged therapy and seems to be reversible upon discontinuation of phototherapy. IGH-like eruption should be added to the list of side-effects of phototherapy. [ABSTRACT FROM AUTHOR]

We describe four patients with mycosis fungoides (MF) in whom depigmentation, and also leucotrichia in one, occurred following the resolution of the eruption during phototherapy (psoralen plus ultraviolet A treatment in three patients, climatotherapy in one). In all cases, the depigmentation was localized to the area of the pre-existing MF lesions. There was no clinically obvious phototoxicity. Biopsy study including S100 staining in all cases, and electron microscopy in one case, demonstrated the total absence of melanocytes, with no evidence of MF. It is suggested that the phototherapy may have activated a cell-mediated immunity leading to destruction of the melanocytes. We recommend that vitiligo-like leucoderma be added to the list of untoward effects of phototherapy in MF. [ABSTRACT FROM AUTHOR]

Summary: Background: The molecular basis of unilesional mycosis fungoides (MF), characterized by a solitary lesion that is clinicopathologically indistinguishable from multifocal patch or plaque MF (early MF), is unknown. Objectives: To investigate the microRNA profile in unilesional MF distinguishing it from early MF. Methods: Biopsy samples of unilesional MF and early MF were evaluated with the Affymetrix microRNA array, with further comparison with inflammatory dermatosis, using quantitative polymerase chain reaction. NanoString technology was applied to analyse the gene expression of T helper (Th)1 immune markers, and immunohistochemistry was used to evaluate CXCR3 and GATA‐binding protein 3 (GATA3) markers for Th1 and Th2 cells, respectively. Results: Unilesional MF had a significantly higher level of expression of all members of the microRNA miR‐17~92 cluster than early MF. Specifically, unilesional MF had a higher miR‐17 level than early MF and inflammatory dermatoses. There was downregulation of the expression of phosphatase and tensin homolog (PTEN) and CREB1, known targets of miR‐17~92 members; higher gene expression of interleukin‐2 and interferon‐γ; and a statistically lower average percentage of GATA3+ dermal cells (6·7% vs. 42·3%), were detected in unilesional MF compared with early MF. High immunoreactivity of CXCR3 was noted in both unilesional and early MF. Conclusions: Unilesional MF exhibits a microRNA profile distinct from that of conventional early MF, with a higher level of miR‐17~92 members along with Th1 skewing. These findings suggest a robust reactive T‐cell immune response in unilesional MF and might account for the localized nature of this disease. What's already known about this topic? Unilesional mycosis fungoides (MF) is characterized by a solitary lesion that is clinicopathologically indistinguishable from multifocal early MF.The molecular basis and the cytokine profile of unilesional MF are unknown. What does this study add? Compared with early MF lesions, unilesional MF is typified by high expression of the microRNA miR‐17˜92 cluster, high T helper (Th)1 cytokine profile and low Th2 dermal lymphocytes. What is the translational message? Whether induction of miR‐17˜92 might serve as a target for treatment of MF to promote anticancer response needs further studies. Linked Comment: Querfeld. Br J Dermatol 2019; 180:984–985. Respond to this article [ABSTRACT FROM AUTHOR]

Background Primary cutaneous marginal zone B-cell lymphoma ( PCMZL) has rarely been reported in patients younger than 20 years. Objectives To report our experience with PCMZL in the paediatric/adolescent age group. Methods Medical records of patients diagnosed with PCMZL before age 20 years and managed at two cutaneous lymphoma clinics in the U.S.A. and Israel from 1992 to 2015 were reviewed. Results The study group included 11 patients (six girls; median age 16 years, range 6-19·5); 10 had generalized/multifocal (T3) and one had regional/localized (T2) disease. Lesions were located on the limbs in all patients and the trunk in six; two had facial lesions. Staging in all but one was based on whole-body computed tomography or positron emission tomography. Initial management in most patients included nonradiation modalities: one patient with localized disease received intralesional steroids; six patients with multifocal disease received the following: topical/intralesional steroids ( n = 3); excision ( n = 2); 'watch and wait' ( n = 1). No extracutaneous progression was noted during a median follow-up of 5·5 years (mean 7·5, range 0·5-14). At present, five patients are in complete remission. Conclusions Based on our data (largest series in the literature with the longest follow-up), the clinicopathological presentation and course of PCMZL in the paediatric/adolescent age group are similar to those in adults. Given the indolent course and the long life expectancy of these young patients, the cumulative risk of imaging studies and the age-related potential toxicity of treatment, especially radiation, should be taken into consideration. [ABSTRACT FROM AUTHOR]

Paediatric mycosis fungoides - characteristics, management and outcomes with particular focus on the folliculotropic variant In the version of this article initially published,1 there was an error in the labels of the columns included in Figure 3. Reference 1 Reiter O, Amitay-Laish I, Oren-Shabtai M, Feinmesser M, Ben-Amitai D, Hodak E. Paediatric mycosis fungoides - characteristics, management and outcomes with particular focus on the folliculotropic variant. [Extracted from the article]

The article presents a case report of a 22-year-old married woman who complained of abdominal pain, nausea and vomiting for two weeks. She was treated with carbamazepine for seizures and anxiety attacks. Sonography showed bilateral solid ovarian masses and ascites were found at the abdominal cavity. Bilateral ovarian luteinized thecoma with sclerosing peritonitis was also found after surgery. She was given tamoxifen which proved to be successful in relieving her from bowel obstruction symptoms.

The significance of tumor cell contamination in marrow and peripheral blood stem cell (PBSC) collections of patients with solid tumors remains controversial. Various methods have been developed to purge tumor cells from autologous stem cell products, including CD34+ selection. PBSC harvests from patients with Ewing family of tumors (EFT) were analyzed for contaminating tumor cells prior and after CD34+ selection using reverse transcription-polymerase chain reaction (RT-PCR) and flow cytometry (FC) analyzes. The expression of CD34 was studied by RT-PCR and FC in 14 primary tumors and 13 PBSC harvests, respectively. Tumor cells were identified in the harvests by both methods. In two patients, contaminating tumor cells were evident by RT-PCR only after positive selection. FC analysis confirmed a higher level of tumor cells in the CD34+ fraction. In an attempt to explore this finding, expression of CD34 was detected in 93% of primary tumors and 67% of contaminated harvests. As CD34 is expressed on EFT cells, these cells may be enriched following CD34+ selection of harvests, although the total number of tumor cells is reduced. Other methods of purging, rather than CD34+ selection, should be explored in patients with EFT undergoing autologous stem cell transplantation.Bone Marrow Transplantation (2007) 39, 589–594. doi:10.1038/sj.bmt.1705640; published online 19 March 2007 [ABSTRACT FROM AUTHOR]

Background The coexistence of mycosis fungoides, a peripheral T-cell lymphoma, and B-cell malignancies or Hodgkin's lymphoma in the same patient is unusual. Most descriptions are isolated case reports and case series are strikingly sparse. Objectives To detect cases of mycosis fungoides associated with B-cell malignancies or Hodgkin's lymphoma and to analyse the characteristics of and the interplay between the lymphoproliferative neoplasms. Method Patients with mycosis fungoides who had B-cell malignancies or Hodgkin's lymphoma were selected from among 398 patients either treated or followed up in two tertiary medical centres during a 7-year period. Results Eleven patients with mycosis fungoides and B-cell malignancy were detected (seven of non-Hodgkin's lymphoma, three of chronic lymphocytic leukaemia, one of multiple myeloma). No case of Hodgkin's lymphoma was found. In seven patients the mycosis fungoides preceded the B-cell malignancy whereas in four it was the B-cell malignancy which occurred first. The time elapsed between onset of the two malignancies ranged from 4 to 22 years (average: 12 years). Patients who had mycosis fungoides as the first neoplasm presented with earlier stages of mycosis fungoides (four of seven: IA, three of seven: IB) than those who had mycosis fungoides as their second neoplasm (of four, one: IB, one: folliculotropic, two: IIB). Among the four patients in whom the appearance of mycosis fungoides followed the B-cell malignancy, three had been treated with multiagent chemotherapy. Two patients who presented with early-stage mycosis fungoides (IA) as the first lymphoma developed mycosis fungoides tumours after becoming immunosuppressed. In two patients infiltrates composed of both malignant T- and B-cell populations were found in a single biopsy. One showed two distinct populations of the malignant cells in the skin tumour, thus constituting a classical composite lymphoma of mycosis fungoides and chronic lymphocytic leukaemia, while in the other patient the two malignant populations of marginal B-cell lymphoma and mycosis fungoides (as evidenced by both phenotypic and genotypic findings) were intermingled. Conclusions This case series indicates that while the coexistence of Hodgkin's lymphoma and mycosis fungoides is extremely rare, the association of mycosis fungoides and B-cell malignancies is not as rare as reflected in the literature, with non-Hodgkin's lymphoma constituting the most common associated B-cell malignancy. In this series as well as in the cases reported in the literature mycosis fungoides usually preceded the development of B-cell malignancies, which may be in accordance with previous reports of an increased risk of developing a second haematological neoplasm. The importance of a competent immune system for patients with mycosis fungoides is well demonstrated in these cases. It is suggested that for greater precision the criteria for diagnosis of composite lymphoma of the skin should include both phenotypic and genotypic features. [ABSTRACT FROM AUTHOR]

The surgical treatment of primary cutaneous malignant melanoma has been an issue of debate throughout the past decades. It is generally accepted that the excised tumor-free surgical margins around the lesion should be proportional to the lesion thickness. However, the issue of adequate thickness of tumor-free margins to be resected in the depth of the lesion has not been addressed as yet. In order to assess the influence of thickness of the tumor-free margins resected under malignant melanoma on patients outcome, we have reviewed 48 patients treated for primary cutaneous malignant melanoma in the Rabin Medical Center in the years 1987–1988. Biopsy specimens and clinical charts were evaluated, the tumor-free margin in depth of the excision was measured and compared with clinical outcome. The study population comprised 21 men and 27 women with an average age of 60.2±15.2 years. Most of the primary melanomas were of intermediate thickness with a mean Breslow thickness of 1.6 mm. During a median follow-up time of 112.8 months, ten (21%) patients developed relapse of their malignancy and 13 patients (27.08%) died. In univariate analysis, the thickness of the tumor-free margin excised under the melanoma was one of seven clinical and pathological variables that showed significant predictive value for patient survival. The 10-year survival rate in patients with tumor-free margins thinner than 3 mm was 50%, compared with 82% in patients with thicker tumor-free margins (P=0.01). In multivariate analysis, these margins remained an independent significant prognostic factor, as were four other variables. Evidence indicating a tendency for local and distant melanoma recurrence in thin excisions with small margin/tumor thickness ratio was detected. The recurrence rate in the group with margin/tumor thickness ratio smaller than 1 was nearly five times higher than the rate in patients with higher ratios (66% vs 14%, P=0.003). In univariate and multivariate analysis of 10 years survival, this ratio also proved to be an independent prognostic factor. In this study we have shown that the tumor-free margins resected under the lesion play an important role in the comprehensive surgical treatment of primary cutaneous melanoma and in determining the clinical outcome of the patients. [ABSTRACT FROM AUTHOR]

A 2-y-old girl with severe edema, oliguria and hypoalbuminemia caused by protein-losing gastritis was diagnosed with cytomegalovirus-associated Ménétrier's disease. After almost two weeks, during which the patient required repeated albumin transfusions, she was treated with intravenous ganciclovir. Within five days her condition had improved, and no additional albumin replacement was needed. Complete recovery was observed after several weeks. Conclusion: In patients with severe Ménétrier's disease, a course of ganciclovir treatment may be of benefit and should be considered. [ABSTRACT FROM AUTHOR]

Idiopathic palmoplantar eccrine hidradenitis (IPPH) is a recently described disorder characterized by painful erythematous plantar nodules and in three cases, showed a typical neutrophilic infiltrate around and within the eccrine sweat apparatus. Five cases of IPPH on the soles of the feet in healthy children are reported. The disorder presented after intense physical activity in four cases. The course was benign and self-limiting. Complete bed rest for several days without any medical therapy led to alleviation of the pain and disappearance of all the lesions. Conclusion. Idiopathic palmoplantar eccrine hidradenitis may be more common than reported. Paediatricians should be aware of it in order to avoid unnecessary diagnostic tests and treatments. [ABSTRACT FROM AUTHOR]