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Multiple erythematous linear arranged hyperkeratotic umbilicated papules on the trunk of patient 19. Note Koebner phenomenon from scratching.
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Background:
Acquired perforating dermatosis (APD) is histopathologically characterized by transepidermal elimination of materials from the upper dermis. APD can be divided into four diseases: Kyrle's disease, perforating folliculitis, elastosis perforans serpiginosa, and reactive perforating collagenosis. APD is usually associated with systemic di...
Citations
... The most common lesions in our study were excoriated and hyperkeratotic papules, also with plaques and nodules. The lower extremity was the most commonly affected area in our study (86.31%) and it was similar to the literature[4,6,7,[9][10][11]. Koebnerization such as linear localized lesions in the excoriated skin are observed in the APD and were detected in 25.26% of our patients. ...
Abstract
Introduction: Acquired perforating dermatosis (APD) is a disease group characterized by transepidermal elimination of dermal connective tissue materials such as collagen, elastic fibers, and keratin through the epidermis and observed with pruritic skin lesions.
Objectives: In this study, we aim to clarify the clinical, histopathological, and dermoscopic characteristics of APD, identify the associated systemic disease, and figure out treatment options.
Methods: This study was designed as a single-center retrospective, observational, cross-sectional study. We evaluated all accessible APD cases between January 2004 and June 2022 in a tertiary care hospital.
Results: A total of 95 patients with confirmed APD were included in the study. Sixty percent of the patients were women and 40% were men. The median age at diagnosis was 63.1 years (35-85 years). The most common site of lesions was the lower extremities which were detected in 86.31% of the patients. The concomitant systemic disease was identified in 84.21% of the patients. The most common systemic disease was type 2 diabetes mellitus (65.26%). Antihistamines and topical corticosteroids were the most commonly prescribed treatment agents.
Conclusions: Transepidermal elimination of dermal connective tissue components is a feature of APD and the disease usually presents with pruritic papules and nodules with central keratotic crust or plug. The diagnosis of APD requires a clinical examination and histological investigation. APD is usually accompanied by systemic comorbidities. There are several topical and systemic medications available for APD, however, sometimes the therapy might be challenging.
... Seven retrospective case series studies have summarized a total of 282 cases of APD. [1][2][3][4][5][6][7] The condition mainly affects patients in their 40s and 50s. Pruritus was the most common symptom, with a few complaining of pain. ...
... Koebner phenomenon was seen in 32% to 56% of patients. [2][3][4]6,7 Patients with APD often have an underlying systemic disease, particularly diabetes (type 1 or type 2) or chronic kidney disease. [1][2][3][4][5][6][7] Other underlying comorbidities include cardiovascular disease, infection (human immunodefi ciency virus, hepatitis virus, and tuberculosis), rheumatic disease, pulmonary disease, malignancy, psychiatric disease, hypothyroidism, pregnancy, and dermatoses (atopic dermatitis, psoriasis, scabies). ...
... [2][3][4]6,7 Patients with APD often have an underlying systemic disease, particularly diabetes (type 1 or type 2) or chronic kidney disease. [1][2][3][4][5][6][7] Other underlying comorbidities include cardiovascular disease, infection (human immunodefi ciency virus, hepatitis virus, and tuberculosis), rheumatic disease, pulmonary disease, malignancy, psychiatric disease, hypothyroidism, pregnancy, and dermatoses (atopic dermatitis, psoriasis, scabies). [1][2][3][4][5][6][7] Pathogenesis still unclear The pathogenesis of APD remains unknown. ...
... The most common lesions in our study were excoriated and hyperkeratotic papules, also with plaques and nodules. The lower extremity was the most commonly affected area in our study (86.31%) and it was similar to the literature[4,6,7,[9][10][11]. Koebnerization such as linear localized lesions in the excoriated skin are observed in the APD and were detected in 25.26% of our patients. ...
Introduction:
Acquired perforating dermatosis (APD) is a disease group characterized by transepidermal elimination of dermal connective tissue materials such as collagen, elastic fibers, and keratin through the epidermis and observed with pruritic skin lesions.
Objectives:
In this study, we aim to clarify the clinical, histopathological, and dermoscopic characteristics of APD, identify the associated systemic disease, and figure out treatment options.
Methods:
This study was designed as a single-center retrospective, observational, cross-sectional study. We evaluated all accessible APD cases between January 2004 and June 2022 in a tertiary care hospital.
Results:
A total of 95 patients with confirmed APD were included in the study. Sixty percent of the patients were women and 40% were men. The median age at diagnosis was 63.1 years (35-85 years). The most common site of lesions was the lower extremities which were detected in 86.31% of the patients. The concomitant systemic disease was identified in 84.21% of the patients. The most common systemic disease was type 2 diabetes mellitus (65.26%). Antihistamines and topical corticosteroids were the most commonly prescribed treatment agents.
Conclusions:
Transepidermal elimination of dermal connective tissue components is a feature of APD and the disease usually presents with pruritic papules and nodules with central keratotic crust or plug. The diagnosis of APD requires a clinical examination and histological investigation. APD is usually accompanied by systemic comorbidities. There are several topical and systemic medications available for APD, however, sometimes the therapy might be challenging.
... 4 Long-term follow-up has shown that APD is a disabling disease with severe pruritus in 83.3% and generalized ulcerating lesions that are associated with infections and scarring. 5 Management of the disease has been hindered by a lack of knowledge of the disease's etiology and the failure of supportive measures for both DM and renal disease to halt the natural progression of the disease. 4 Hence, the treatment of APD has been limited to individual case reports and small series of patients. ...
Acquired perforating dermatosis (APD) is an adult skin disease characterized by an umbilicated papulonodular rash with transepidermal elimination of dermal components such as collagen and/or elastin. It is frequently associated with multiple medications and diseases such as diabetes and chronic renal failure. It is a disabling disease with severe pruritus in 83.3% of cases and generalized ulcerating lesions that are associated with infections and scarring. Nearly 10% of renal patients are affected. Supportive measurements of disease activity and previous medications failed to halt its natural progression. In our study, we documented significant improvements in the severity of the disease as measured by the eczema area and severity index (EASI), in 32 patients with the renal disease through the use of mycophenolate mofetil (MMF), with EASI decreasing from 31 [interquartile range (IQR) = 4] to 3 (IQR = 4) by the 3 rd month. Moreover, such changes persisted for up to 2 years despite a decrease in the dose of MMF to half after 1 year. In conclusion, our study showed that MMF is a safe and effective immunosuppressive drug for short- and intermediate-term therapy of severe APD and confirmed its autoimmune etiology.
... Since they have different probabilities of local recurrence and, in some rare controversial cases, metastasis, the clinical presentation as well as the correct identification of histopathological features of different variants of FH are essential in making the correct diagnosis and in determining the prognosis [5,6]. Multiple FHs, and/or eruptive FH, have been observed in the context of immunosuppression, HIV infection, with highly active antiretroviral therapy (HAART), during pregnancy, in systemic lupus erythematous, and in some acquired perforating dermatosis (APD) as Kyrle's disease, perforating folliculitis, reactive perforating collagenosis, and elastosis perforans serpiginosa [5,7,8]. ...
... Trauma from scratching was suggested to be a major trigger of perforating disorders, inducing damage to the epidermis or dermal collagen, resulting in the transepidermal elimination of collagen or elastic fibers. The alteration in collagen or elastic fibers due to metabolic disturbances or microdeposition of various substances represents another hypothesis [7]. Some authors also support the hypothesis that ischemic epidermal alterations may result in the destruction of the epidermis [21]. ...
A 31-year-old male presented with a firm, well-demarcated, erythematous, crateriform, and ulcerated nodule in the left lumbar region, which persisted for 3 months. Clinically, a keratoacanthoma was suspected. The histological analysis was consistent with perforating fibrous histiocytoma, a rare histopathologic variant of fibrous histiocytoma. To our knowledge, this is the third case reported in the literature.
... Based upon the nature of the eliminated material and the type of epidermal disorder, acquired perforating dermatoses traditionally tend to be divided into four subtypes: Kyrle's disease (KD), perforating folliculitis (PF), elastosis perforans serpiginosa (EPS), and reactive perforating collagenosis (RPC). 1,2 Several later studies show that both collagen and elastin could penetrate the surface of RPC-like lesions. [3][4][5] There may be different results of PF, KD and RPC in the same patient depending on the sites, period, and layers of the biopsy. ...
... 6 Although APD can be associated with many systemic diseases such as chronic renal failure and diabetes mellitus, its pathogenesis has not been made clear. 2,3 The diagnosis of APD is traditionally based upon clinical umbilicated erythematous papules with firmly adherent crusts and histologically transepidermal elimination of fibers with basophilic cellular debris. The application of noninvasive techniques such as dermoscopy and reflective confocal microscopy (RCM) in APD for assisted diagnosis has not been fully evaluated. ...
Background
Acquired perforating dermatosis (APD) is a rare skin condition characterized by degenerated materials eliminated from the dermis. Several retrospective studies on APD have been reported; however, few data are available on Chinese APD and their features on dermoscopy and reflective confocal microscope (RCM) assays.
Objective
The aim of this study was to retrospectively evaluate the clinical and histopathologic data of 37 acquired perforating dermatosis cases, and assess their features on dermoscopy and RCM.
Methods
Thirty‐seven APD patients were retrospectively enrolled in our study. We characterized the clinical histopathological features, concomitant diseases, treatment responses, and the dermoscopy and RCM findings.
Results
Pruritus was the most common symptom, with the lower extremities as the most predilection sites (86.5%, n = 32; 91.9%, n = 34, respectively). Concomitant diseases were found in 34 patients (92.6%), among which diabetes mellitus was the most common, followed by thyroid nodules, allergic dermatosis, and chronic renal insufficiency. Dermoscopy and RCM assays were performed in 11 patients. The typical RCM images were hyperreflective cord‐like structures from the epidermis to dermis. Dermoscopy features of fully developed lesions showed central ulceration with peripheral hairpin‐like or loop‐like capillaries with characteristic garland arrangements.
Conclusion
APD is an uncommon skin disorder associated with various systemic conditions in Chinese individuals. Thyroid disorders are an overlooked complication and may play an important role in the development of APD. The results of this study indicate that noninvasive dermoscopy and RCM examination are helpful in the rapid diagnosis and early intervention of APD.
... Perforating dermatoses are a group of conditions characterized by transepidermal elimination (TEE) of dermal components, such as collagen, elastin, and fibrin [3]. Acquired reactive perforating collagenosis (ARPC) is a type of perforating dermatosis that usually develops in adulthood, in association with systemic diseases, especially diabetes mellitus or chronic renal failure [4,5]. Recently, several cases of BP that developed in patients with ARPC have been reported [6][7][8]. ...
A 61-year-old man presented with 6-month and 5-day histories of multiple, pruritic nodular eruptions on the trunk and extremities and bullous eruptions on the left foot, respectively. The nodular eruptions had been treated with topical corticosteroids without improvement. He had been diagnosed with diabetes mellitus at the age of 42 years and had been suffering from end-stage renal disease for 1 year. Physical examination revealed scattered violet-brown papules and nodules on the trunk and extremities, many of which had central umbilicated necrosis or keratin plugs. Additionally, two tense bullae and five erosions were noted on the dorsal aspect of the left foot. Laboratory tests showed elevated levels of serum anti-bullous pemphigoid (BP)180 antibody. Histopathological findings of a nodule and a bulla were compatible with those of acquired reactive perforating collagenosis (ARPC) and BP, respectively. The papular and nodular lesions were diagnosed as ARPC, while bullous and erosive lesions were diagnosed as localized BP. The present case, together with previously reported cases of coexisting generalized BP and ARPC, suggests that coexistence of BP, regardless of whether generalized or localized, is significantly associated with ARPC.
... 9,10 The clinical features of papules and hyperkeratotic nodules of the four subtypes are difficult to distinguish clinically. These four subtypes of APD can be distinguished based on the eliminated dermal component revealed by histopathological examination, which is collagen in ARPC, keratotic material without collagen and elastin in KD, and follicular components with or without collagen and elastin in PF. 10 Kim et al 11 reported that ARPC was the most common subtype of APD, occurring in 73.3% of 30 APD patients at Eulji General Hospital and Chung Ang University Hospital. Collagen is the eliminated dermal connective tissue component in ARPC. ...
An abundance of endocrine receptors is expressed on the skin and becomes the target of many hormones. This was associated with various skin diseases that might occur in some endocrine diseases eg, lichen amyloidosis (LA) and acquired reactive perforating collagenosis (ARPC). Here, we report a coexistent LA and ARPC in a 55-year-old woman, characterized with multiple pruritic hyperkeratotic papules and plaques on both arms accompanied by pruritic hyperkeratotic papules and nodules on both legs. She had a history of type 2 diabetes mellitus (DM) and post-thyroidectomy hypothyroidism due to papillary thyroid carcinoma. Histopathological examination revealed amyloid deposition in the papillary dermis corresponding with LA and cup-shaped epidermal depression filled with collagen corresponding with ARPC. The hyperkeratotic papules and nodules flattened in one month after application of 0.05% clobetasol propionate ointment with occlusion on both arms and 0.05% retinoic acid gel on both legs. Hyperkeratotic papules, nodules, and plaques in a patient with a history of endocrine diseases, such as type 2 DM and thyroid disorders should undergo histopathological examination to confirm the diagnosis of skin diseases eg, LA or ARPC.
... This group encompasses both primary disorders, such as reactive perforating collagenosis, elastosis perforans serpiginosa, and perforating folliculitis, and acquired forms associated with systemic diseases, to include most commonly diabetes mellitus and chronic renal failure. 7 In rare cases, calcinosis cutis has been additionally reported as a cause of acquired perforating dermatosis. 8 Multiple hypotheses regarding the pathogenesis of acquired perforating dermatoses exist, identifying possible triggers for degeneration and elimination of collagen fibers, to include trauma, microvasculopathy, and biochemical collagen alterations such as microcrystalline calcium salt deposition. ...
Iatrogenic calcinosis cutis represents a subset of calcinosis cutis resulting secondary to treatments or procedures. We present the first report of calcinosis cutis resulting from the intraosseous infusion and one of a few cases with associated transepidermal elimination. A previously healthy 2-year-old female presented with a new-onset unilateral shin rash 1 week following hospitalization for a near-drowning event. A dermatologic exam revealed multiple small, tender, firm, chalky-white papules with surrounding erythema, in addition to two erythematous macules superior and medial to the papular lesions, corresponding to prior intraosseous access sites. The rash persisted despite trials of topical mupirocin and acyclovir cream, prompting a referral to a dermatologist. An excisional biopsy was performed, revealing circumscribed dermal deposits of acellular basophilic material connected to the overlying epidermis through an invaginated keratin plug. A von Kossa silver stain highlighted the deposits, confirming the diagnosis of perforating calcinosis cutis. The lesions did not recur following the excisional biopsy. Iatrogenic calcinosis cutis may be seen as a complication of the infusion of calcium-containing fluids via intraosseous access, in addition to the more commonly observed peripheral intravenous access. Awareness of this disorder is important in order to distinguish it from an infectious mimic and guide the selection of therapy.
... 5 Se ha informado la relación con otras enfermedades sistémicas, tales como la insuficiencia renal, diabetes, trastornos hepáticos, endocrinológicos, carcinoma hepatocelular, enfermedad Hodgkin, leucemia aguda, SIDA, tuberculosis, aspergilosis pulmonar, neurodermitis, dermatitis atópica, escabiosis, e incluso durante el estado fisiológico del embarazo. 6 Las lesiones características son pápulas de 2-10 mm eritematosas o eritematovioláceas con un tapón queratósico central, relacionado histológicamente con la queratina y los desechos necróticos, estas pápulas pueden unirse para formar placas queratósicas más grandes, las lesiones pueden estar localizadas en cualquier sector de la superficie cutánea principalmente en la cara anterior o ventral de miembro inferior y cara posterior o dorsal de miembro superior y con menos frecuencia en la región cefálica, cuello y tronco; algunas son asintomáticas y otras muy pruriginosas, estas con el rascado, pueden producir el fenómeno de Köebner. 4,7 Su evolución es crónica, observándose mejoría de las lesiones cutáneas espontáneamente, pero principalmente, cuando se logra la estabilización de la enfermedad subyacente, puede dejar como secuelas cicatrices residuales. ...
