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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., (© 2022 European Academy of Dermatology and Venereology.)

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., (© 2014 European Academy of Dermatology and Venereology.)

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., (© 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology.)

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.

One of the common lesions in Crohn's colitis is erythema nodosum, a septal panniculitis that may appear before diagnosis or in conjunction with flare up. We report a case of Crohn's colitis where the presenting sign was neutrophilic lobular panniculitis with few granulomas. The possibility that this case represents a forme fruste of metastatic Crohn's is suggested. As skin biopsies are not usually performed by gastroenterologists in erythema nodosum-like skin lesions in Crohn's patients it may well be that the incidence of lobular panniculitis is higher than reported in the literature.

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.