Acquired perforating dermatosis: clinicopathologic study of a 10‐year period at a tertiary teaching hospital

Abstract

Background Acquired perforating dermatosis (APD) comprises an uncommon group of skin disorders that develop in adulthood in association with systemic diseases. The aim of this study was to characterize clinicopathologic features and treatment outcomes in a series of patients diagnosed with APD. Methods Retrospective study of all patients diagnosed with an APD over a 10‐year period (2009–2018) at a tertiary teaching hospital in Lisbon, Portugal. Results Fifty‐seven patients with APD were identified. Thirty‐five patients presented lesions in multiple anatomic areas (61.4%), and the lower limbs were the most common location. Forty‐six patients reported pruritus (80.7%), which was classified as severe in 21 of them (36.8%). An underlying systemic disease was identified in 53 patients (93.0%). Diabetes mellitus (DM) and chronic kidney disease (CKD) were the most common associated systemic diseases, but psychiatric disorders, malignancies, and chronic infections were present in a significant number of patients. The combination of topical steroids with antihistamines was the most prescribed initial treatment, but only 37.8% of the patients had a complete response. Acitretin, systemic steroids, and phototherapy were the treatments associated with the best outcome. Conclusion Acquired perforating dermatosis can be associated with many systemic disorders that have pruritus as a common factor. Chronic viral infections and an occult malignancy should be sought, particularly in the absence of DM and CKD. The management of APD is challenging and is best achieved with the control of the underlying systemic diseases.

Evaluation of clinicopathological and treatment characteristics of 80 patients with acquired perforating dermatosis

“…In the largest series with 57 patients, the female to male ratio was reported approximately equally. 4 In our study, the lesions were frequently located on lower extremity similar to the literature. 1,2,4 But unusual presentations such as face and scalp were also observed.…”

“…Pruritus is the main symptom reported in this disease and it had been published that 61% to 100% of the patients had pruritus. [2][3][4][5] However, it has been reported in some publications that pain may also be seen, and, there was no patient with pain in our study. 3 Koebner positivity suggests that trauma may play a role in the etiology and pathogenesis of the disease.…”

“…Approximately 60% of patients with DM had been on insulin treatment, which was over the frequency reported in the literature. 4 Morton et al 16 reported a higher number of patients on insulin therapy, but this result may have been caused by the patient population, because all patients were suffering CKD.…”

“…1 It's often accompanied by systemic diseases such as diabetes mellitus (DM), chronic kidney disease (CKD), however it can be also seen with many different diseases. [2][3][4][5] The etiology and pathogenesis of the disease is not known precisely. Since there are a few studies with a limited number of patients in the literature, our study aimed to evaluate clinicopathological and treatment characteristics of 80 patients diagnosed with APD.…”

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“…1 Among systemic diseases accompanied by ARPC, hypertension is the most common, followed by diabetes, and kidney failure. 2 At present, some studies have found that there is overexpression of transforming growth factor (TGF)-β3 in many ARPC patients 3 suggesting that this factor may be related to the incidence of ARPC. Advanced glycosylation end product receptor (RAGE) is a multi-ligand transmembrane receptor that plays an important role in the inflammatory response.…”