BackgroundAnemia is a common complication of epidermolysis bullosa (EB). To date, no extensive data on the prevalence of anemia in EB patients have been well characterized worldwide.ObjectiveTo determine and to characterize the prevalence of anemia in the Australian EB population by conducting a retrospective cross-sectional study.MethodsAll (n = 368) EB patients registered in the Australasian Epidermolysis Bullosa Registry (AEBR) from 2006 to 2012 were reviewed for pathological evidence of anemia. Patients with EB without anemia and those without hematological parameters were excluded from the study. Patients’ particulars were separated into pediatric (< 18 years old) and adult (≥ 18 years old) male and female subgroups.ResultsOne-hundred sixty-nine out of 368 EB patients had eligible blood results to be analyzed, as milder forms of EB did not routinely have laboratory testing; 27.8% (n = 47/169) of EB patients were anemic at any time point in their lifetime. All generalized severe junctional EB (JEB-GS) cases (100%, n = 4/4); 68.0% (n = 17/25) of recessive dystrophic EB (RDEB); and 37.5% (n = 6/16) of generalized intermediate JEB (JEB-I) patients were anemic.LimitationsAs EB is an orphan disease, the limited sample size may have affected the significance of the study result.ConclusionThe high prevalence of anemia seen in RDEB and JEB generalized severe (JEB-GS) patients in our cohort is similar to those reported in case series.
We describe three cases of hair loss in a female pattern hair loss (FPHL) distribution with histologic features of lichen planopilaris (LPP). All patients had a history of diffuse, gradual hair loss in a Christmas tree pattern that clinically presented as FPHL on gross and dermoscopic examination. Notably, there were no characteristic clinical signs of LPP and no histologic features of FPHL. These cases are most consistent with cicatricial pattern hair loss (CPHL). This relatively new entity is similar to fibrosing alopecia in a pattern distribution (FAPD) in that they are both scarring alopecias confined to a FPHL distribution, but CPHL lacks the clinical signs of perifollicular erythema and perifollicular keratosis seen in FAPD. These three cases may present an early, subtle form of CPHL and will be of interest to clinicians and histopathologists alike.
We report five cases of Rickettsia australis infection from southern coastal New South Wales, Australia. All patients presented with a cutaneous eruption of erythematous papules and pustules and systemic features of malaise, headache, lymphadenopathy and myalgia. Acute kidney injury (AKI) was present in two of five cases and one of five cases had acute delirium. Improvement was only seen after treatment with doxycycline 100 mg b.i.d. Positive serology for R. australis was present in four of five cases and a positive polymerase chain reaction (PCR) was seen in one of five cases. Histology showed varying features, from neutrophilic vasculitis to Sweet's syndrome and lymphocytic vasculitis. Recent significant advances in the diagnosis of R. australis infection include an eschar swab or biopsy PCR and isolation of specific Rickettsia on serology. These investigations should be considered in the presence of any of the following features: eschar at site of a tick bite or lymphadenopathy and fever with an eruption of erythematous papules and pustules.
We report a case of nonuremic calcific arteriolopathy (NUCA) in an 82-year-old Caucasian woman from rural Australia. The patient had no history of kidney disease or dialysis. NUCA is rare disease suspected on cutaneous and clinical features and diagnosed by characteristic findings on skin biopsy and vasculature imaging. Calcification induced microvascular occlusion in the absence of renal failure may not be immediately apparent. Clinical suspicion and appropriate investigations are essential for making a diagnosis. A diagnosis of NUCA may be missed given the rarity of the disease, and dermatologists and patients alike would benefit from a greater awareness of this disease.
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