Price, Harper

Abstract Sweet's syndrome (SS) is a neutrophilic dermatosis characterised by the acute onset of erythematous papules or plaques and a constellation of symptoms including fever, leucocytosis, and histopathology demonstrating nodular, pustular, or diffuse infiltrate of neutrophils with marked papillary oedema. SS can be a manifestation of inflammatory bowel disease and often coincides with periods of disease flares. Only a few cases of SS associated with ulcerative colitis are reported in the literature, and no cases have involved pulmonary manifestations in the paediatric population. We present a case of a 14-year-old girl presenting with new onset ulcerative colitis requiring emergent colectomy with subsequent development of pulmonary SS. Treatment consisted of intravenous and oral corticosteroids and dapsone. SS should be considered in the differential diagnosis of prolonged fever with cutaneous involvement and systemic symptoms. Special consideration should be given to paediatric patients with extracutaneous manifestations, particularly pulmonary involvement.

Abstract Background Facial angiofibromas (FAs) are a major feature of tuberous sclerosis complex (TSC). Topical rapamycin can successfully treat FAs. A new stabilized cream formulation that protects rapamycin from oxidation has been developed in 0.5% and 1% concentrations. Objectives To assess the efficacy and safety of a novel, stabilized topical rapamycin cream formulation. Methods This multicentre double-blind randomized placebo-controlled dose–response phase II/III study with a parallel design included participants aged 6–65 years with FAs of mild or moderate severity according to the Investigator’s Global Assessment (IGA) scale. Participants were randomized to one of three treatment arms: topical rapamycin 0.5%, topical rapamycin 1% or placebo. Treatment was applied once daily for 26 weeks. Safety and efficacy measures were assessed at days 14, 56, 98, 140 and 182. The primary endpoint was the percentage of participants achieving IGA scores of ‘clear’ or ‘almost clear’ after 26 weeks of treatment. Secondary measures included Facial Angiofibroma Severity Index (FASI) and participant- and clinician-reported percentage-based improvement. Safety measures included the incidence of treatment-emergent adverse events and blood rapamycin concentration changes over time. Results Participants (n = 107) were randomized to receive either rapamycin 1% (n = 33), rapamycin 0.5% (n = 36) or placebo (n = 38). All treated participants were included in the final analysis. The percentage of participants with a two-grade IGA improvement was greater in the rapamycin 0.5% treatment group (11%) and rapamycin 1% group (9%) than in the placebo group (5%). However, this was not statistically significant [rapamycin 0.5%: odds ratio (OR) 1.71, 95% confidence interval (CI) 0.36–8.18 (P = 0.50); rapamycin 1%: OR 1.68, 95% CI 0.33–8.40 (P = 0.53)]. There was a statistically significant difference in the proportion of participants treated with rapamycin cream that achieved at least a one-grade improvement in IGA [rapamycin 0.5%: 56% (OR 4.73, 95% CI 1.59–14.10; P = 0.005); rapamycin 1%: 61% (OR 5.14, 95% CI 1.70–15.57; P = 0.004); placebo: 24%]. Skin adverse reactions were more common in patients following rapamycin application (64%) vs. placebo (29%). Conclusions Both rapamycin cream formulations (0.5% and 1%) were well tolerated, and either strength could lead to clinical benefit in the treatment of FA.

AbstractAlopecia is a common sequela in children undergoing chemotherapy, radiation, and hematopoietic stem cell transplantation. In most cases, this is a transient state in which full hair regrowth eventually occurs, but permanent or persistent alopecia, defined as the presence of incomplete hair regrowth more than 6 months after cessation of treatment, is possible and can be psychologically distressing. We sought to characterize the risk factors that can lead to permanent alopecia following the aforementioned treatments in pediatric populations, as well as diagnostic and treatment options that may be considered, as part of a scoping review of the literature. A general algorithm for approaching these patients was developed based on our findings.

AbstractEccrine angiomatous hamartoma (EAH) is a rare benign vascular hamartoma characterized histologically by an increased size and number of mature eccrine glands associated with multiple foci of dilated capillaries in the dermis and subcutis. EAH typically presents in children as discrete, solitary nodules, or plaques most commonly located on the extremities. Some cases of EAH have an agminated distribution involving classic locations, or present as solitary lesions in less common locations such as the face, scalp, or trunk. We report the case of congenital EAH in a child with atypical morphological features and pattern of distribution further expanding on the range of presentations classically described.

AbstractTrichothiodystrophy is a rare autosomal recessive disorder resulting in a broad range of systemic abnormalities. Polarizing microscopy of the hair reveals the pathognomic “tiger tail” of alternating light and dark bands, but the need for a microscope prevents rapid bedside diagnosis. We describe a new technique for the bedside diagnosis of trichothiodystrophy using a handheld polarizing dermatoscope, precluding the need for microscopic examination.

AbstractPrurigo pigmentosa (PP) is a rare inflammatory dermatosis of unknown cause characterized by a predominantly truncal eruption of pruritic erythematous papules in a reticular pattern, resolving with hyperpigmentation. PP is twice as common in girls and women, and the mean age at onset is 25 years. Diagnosis of PP is challenging and is aided by characteristic histopathologic findings. We report a case of PP in a 17‐year‐old white boy. The eruption arose during strict adherence to a ketogenic diet.

AbstractWe report the case of a 2.5‐year‐old girl with linear morphea initially diagnosed as an acquired port‐wine stain (PWS). She underwent three treatments to the right face using the pulsed dye laser (PDL) before sclerotic changes were observed and the correct diagnosis was confirmed with histopathology. Treatment using the PDL reduced the skin erythema but did not prevent subsequent sclerosis. The sclerosis became most prominent superior to the patient's right ear in an area not treated using the laser. A review of the English‐language medical literature identified no cases of morphea triggered using a PDL, but there were several reports of early morphea misdiagnosed as an acquired PWS. Briefly, we review those cases, as well as morphea subtypes, and comment on how the pathophysiology of morphea may lend itself to an early underrecognized inflammatory presentation, delaying diagnosis.