Reactive Perforating Collagenosis Research Articles

A case report on acquired reactive perforating collagenosis.

Acquired reactive perforating collagenosis (ARPC) is a rare perforating skin disease with unclear pathogenesis, often leading to misdiagnosis. Utilizing noninvasive skin microscopy improves diagnostic accuracy while reducing misdiagnosis rates. Given its association with systemic diseases, comprehensive examinations are crucial for early detection of related diseases such as tumors. Clinically, it still lacks standardized guidelines for the treatment. Clinical treatment is mostly based on symptomatic treatment. Oral administration of pregabalin capsules can significantly relieve itching symptoms, and narrow-wave ultraviolet irradiation can accelerate the recovery of skin lesions. Dermoscopy and skin biopsy was used to confirm this case was ARPC. Treatment was based on oral administration of 20 mg prednisone, 1 tablet of loratadine, 1 tablet of pregabalin in the morning and evening, and external application of halomethasone ointment. Itching symptoms were significantly relieved. This case report demonstrates that clinical dermoscopy can improve the diagnosis rate of ARPC, and pregabalin capsules can significantly relieve itching symptoms.

BG09 A case of giant variant acquired reactive perforating collagenosis in a 94-year-old man with poorly controlled diabetes

BG09 A case of giant variant acquired reactive perforating collagenosis in a 94-year-old man with poorly controlled diabetes

Acquired reactive perforating collagenosis in a patient with diabetes.

Acquired reactive perforating collagenosis in a patient with diabetes.

Acquired reactive perforating collagenosis and eosinophilic granulomatosis with polyangiitis developing simultaneously in a diabetic patient

Acquired reactive perforating collagenosis and eosinophilic granulomatosis with polyangiitis developing simultaneously in a diabetic patient

Successful Use of Dupilumab in the Treatment of Acquired Perforating Dermatosis Associated with Atopic Dermatitis

Acquired reactive perforating collagenosis is a rare cutaneous disorder characterised by the extrusion of abnormal connective tissue trough epidermidis and/or follicular units. Reactive perforating collagenosis is often associated with systemic diseases in which pruritus is a common symptom (e.g., diabetes and chronic kidney disease). Less commonly, it has been associated with chronic inflammatory dermatoses, including atopic dermatitis, as in this case. In this report, we describe the exceptional case of a 35‐year‐old man affected by acquired reactive perforating collagenosis associated with atopic dermatitis who was resistant to conventional topical and systemic treatment and experienced complete resolution of clinical signs and symptoms after 12 weeks of treatment with dupilumab. In our patient, the severe pruritus induced by atopic dermatitis likely contributed to the development of acquired perforating collagenosis lesions, which are thought to be a reactive response to chronic scratching and repetitive injury to the skin. Chronic pruritus in atopic dermatitis is known to be driven by type 2 cytokines, including IL‐4 and IL‐13, and dupilumab, a monoclonal antibody inhibiting IL‐4 and IL‐13 signalling, has been shown to be effective in the treatment of moderate to severe atopic dermatitis as well as other type 2‐driven pruritic dermatological conditions. This case supports the potential use of dupilumab for the treatment of reactive perforating dermatosis.

A case of acquired reactive perforating collagenosis after COVID-19 infection

A case of acquired reactive perforating collagenosis after COVID-19 infection

Systematic review and meta-analysis Literature-based Clinical Retrospective Analysis of Acquired Reactive Perforating Collagenosis

Objective: Acquired reactive perforating collagenosis(ARPC) is a rare dermatosis that usually associated with systemic disease. The associations, characteristics and treatment rigemen of ARPC are not well understood.This study was performed to retrospectively evaluate the diagnosis and treatment statuses of acquired reactive perforating collagenosis (ARPC). Methods: A systematic review of the PubMed, Embase, and Scopus databases was independently performed by two reviewers. Data were extracted and qualitatively analyzed. The quality of the articles was assessed by using the Newcastle–Ottawa scale. The data are described in the manuscript. The study was registered with PROSPERO, CRD 42023448576. Results: In total, 189 patients (98 women, 91 men) in 124 studies were analyzed. Their mean age was 57.31 ± 15.21 years. A total of 88.89% (n = 168) of the patients had one or more systemic diseases, of which 57.67% (n = 109) had diabetes mellitus, 29.10% (n = 55) had chronic renal failure, 25.93% (n = 49) had hypertension, and 13.76% had malignancy (n = 26). The most frequently affected area was the lower extremities (72.49%, n = 137), followed by the trunk (70.37%, n = 133) and the upper extremities (63.49%, n = 120). One hundred sixty (84.66%) patients had pruritus, and 32.80% (n = 62) of them were positive for the Koebner phenomenon. Oral antihistamines and topical steroids were the most common treatments in this study, followed by narrow-band ultraviolet B therapy and allopurinol. Management of concomitant systemic diseases also resulted in improvement of ARPC. Conclusion: In this review, ARPC was frequently associated with systemic diseases, especially diabetes mellitus, renal failure, hypertension, and malignancy. The lower extremities and trunk were the most frequently affected areas, and pruritus usually appeared. Antihistamines and topical steroids were the most commonly used treatments, and we recommend narrow-band ultraviolet B therapy as the first-line treatment. Management of systemic diseases and relief of pruritus should be considered simultaneously.

Dupilumab improve acquired reactive perforating collagenosis characterized by type 2 inflammation.

Acquired reactive perforating collagenosis (ARPC) is a clinically challenging disease with an unclear pathogenesis. To evaluate the efficacy and safety of dupilumab for the treatment of ARPC, and analyze the expression of type 2 inflammation-related molecules in ARPC lesions. This retrospective cohort study included 20 patients with ARPC; 10 received dupilumab and 10 received conventional therapy. The efficacy and safety of dupilumab were evaluated at 12 weeks. Immunohistochemical and immunofluorescence analyses of T- and B-cell markers, and type 2 inflammation-related cytokines, were performed on skin samples from ARPC patients, atopic dermatitis (AD) patients, and healthy controls. Significantly more patients showed improvements in the Investigator Global Assessment score (100% vs. 0%; p < 0.0001) and itching (90%/8.33%, P =.001) in the dupilumab group compared to the conventional group at 12 weeks. There were no adverse effects in the dupilumab group. The ARPC lesions showed enhanced dermal infiltration of CD3+ T-cells, with a predominance of Th2 cells, similar to AD lesions. IL-4 and IL-13 were co-localized with GATA3 in ARPC lesions. Dupilumab improved ARPC charaterized with type 2 inflammation.

Familial Reactive Perforating Collagenosis: Clinical, Dermoscopy, and Histopathological Features in Two Siblings

Transepidermal elimination disorders include reactive perforating collagenosis (RPC), perforating folliculitis, elastosis perforans serpiginosa, and Kyrle’s disease. RPC is a rare transepidermal elimination disorder wherein altered collagen is extruded through the epidermis. It has two types: rare inherited form and relatively common acquired form. Most inherited cases present during infancy or early childhood with papules on extensor surface of hands, elbows, knees preceded by superficial trauma. We report two female siblings of 11 and 10 years with multiple, symmetrically distributed, hyperpigmented papules with central keratotic plug, predominantly over extensor aspects of extremities with no aggravating and relieving factors. Clinical diagnosis of familial RPC was confirmed by consistent dermoscopic and histopathological findings. Both children were started on oral isotretinoin 10 mg/day with topical 12% ammonium lactate.

Підхід до діагнозу перфоруючого серпігінуючого еластозу

The goal is to demonstrate a clinical case of serpiginous perforating elastosis for better awareness of doctors.The hallmark of the perforating diseases is the transepidermal elimination of degraded dermal componenrs. The material extruded may represent abnormally constituded collagen, elastic fibers or other matrix components. Four classical forms of primary perforating dermatosis are described: reactive perforating collagenosis, Kyrle’s disease, perforating folliculitis and elastosis perforans serpiginosa.

Reactive perforating collagenosis and systemic lupus erythematosus: A rare case report

Transepidermal clearance of altered collagen and excessive excretion of keratin are characteristics of a rare cutaneous disorder known as reactive perforating collagenosis (RPC). There are different forms of RPC; however, the acquired form is the most prevalent and inherited. Reactive perforating collagenosis is rarely described in autoimmune rheumatic diseases; instead, it is typically linked to systemic conditions such as renal failure or hepatic disease. A 31-year-old Saudi female patient who was initially diagnosed with undifferentiated connective tissue disease. She developed RPC with a severe diffuse itchy skin rash with numerous papules and nodules with central hyperkeratotic plugs over the lower limb, upper limb, and face. The patient tested positive for antinuclear antibody; however, a year later, patient developed Raynaud's phenomenon, oral and nasal ulcers, malar rash, fatigue, and lupus rash around her eyes, and systemic lupus erythematosus was diagnosed clinically. The patient was treated for reactive perforating collagenosis with systemic antihistamines (diphenhydramine 50 mg orally twice daily), topical steroid cream (betamethasone dipropionate cream), and oral isotretinoin (20 mg daily). The patient was advised to undergo phototherapy. A year later, she presented with symptoms of systemic lupus erythematosus and started taking oral hydroxychloroquine 200 mg twice daily for systemic lupus erythematosus. The patient is listed on follow-up. Variable skin rash can mimic systemic lupus erythematosus and vasculitis. Therefore, reactive perforating collagenosis is a skin condition that requires high clinical suspension for diagnosis, and it might be challenging to determine whether it is an association or a complication. Furthermore, the timing of the skin biopsy may be crucial for the diagnosis of reactive perforating collagenosis.

Acquired reactive perforating collagenosis, a rare entity at uncommon site.

Acquired reactive perforating collagenosis (ARPC), rare disorder characterized by transepidermal elimination (TEE) of collagen fibers, is seen in adult diabetics. Genetic predisposition, familial aggregation, trauma, bites and scratching are implicated. Diabetics develop microvascular diseases leading to intense pruritus causing repeated micro trauma leading to necrosis of connective tissue of dermis, causing TEE. Isolated papules, plaques and nodules with central keratotic plugs, are mostly seen on extensor surfaces of limbs but trunk and face may be involved. Histopathology shows extrusion of abnormal collagen fibers through epidermis. Multiple treatment modalities show variable response. A 52 year old diabetic female had multiple, itchy, well defined, erythematous papules and plaques with central adherent crusting on lower back since 1 month. Histopathology showed cup shaped epidermal depression filled with plug of altered collagen, acanthotic epidermis with hyperkeratosis and parakeratosis. Underlying epidermis was thin with fine slits through which vertically oriented basophilic collagen fibers were extruded.

Perforating folliculitis in Down’s syndrome − a rare case report

Perforating folliculitis is a primary perforating disorder, characterized by the phenomenon of transepidermal elimination. It is associated with various disorders such as psoriasis, juvenile acanthosis nigricans, diabetic mellitus, renal failure, cardiac failure, and malignant neoplasia. Other primary perforating dermatoses are Kyrle’s disease, reactive perforating collagenosis, and elastosis perforans serpiginosa. Among the association of perforating dermatoses with genetic diseases like Down’s syndrome, elastosis perforans serpiginosa and very rarely reactive perforating collagenosis have been reported. We report the first case of an 11-year-old boy who is a known case of Down syndrome presented with perforating folliculitis.

Dupilumab may be an alternative option in the treatment of acquired reactive perforating collagenosis combined with AD.

The management of acquired reactive perforating collagenosis (ARPC) is challenging. Here, we shared two cases of ARPC combined with elderly atopic dermatitis (AD) that did not respond well to conventional treatment but responded well to the monotherapy of dupilumab, which suggests that dupilumab may be an alternative option for the treatment.

Acquired perforating dermatoses show increased levels of cutaneous advanced glycation end-products.

Acquired perforating dermatoses (APDs) are characterized by transepidermal elimination of skin materials. Altered glycation of dermal components may be involved in pathogenesis. To assess whether patients affected by APDs have increased levels of cutaneous advanced glycation end-products (AGEs). A cross-sectional controlled study involving a total of 109 patients was conducted, enrolling 29 patients consecutively diagnosed with primary APDs [reactive perforating collagenosis (RPC), elastosis perforans serpiginosa (EPS), perforating folliculitis (PF) and Kyrle disease (KD)], 40 age- and sex-matched healthy controls (HCs) and 40 patients with mild atopic dermatitis (AD). The levels of cutaneous AGEs were measured using a validated fluorescence technique. The median skin autofluorescence value in patients with APDs was significantly higher [2.7 arbitrary units (AU), interquartile range (IQR) 1.9-3.9 AU] compared with HCs (1.8 AU, IQR 1.6-2.3 AU; P < 0.001) and patients with AD (2.1 AU, IQR 1.9-2.3 AU; P = 0.01). Median values were 3.5 AU (IQR 2.7-4.6 AU) for RPC, 1.83.5 AU (1.4-2.4 AU) for EPS, 3.1 AU (2.4-4.4 AU) for PF and 2.6 AU (2.3-3.1 AU) for KD. Our results may suggest a possible physiopathological role of AGEs in the transepidermal elimination mechanisms involved in certain APDs.

Successful treatment of acquired reactive perforating collagenosis with itraconazole

BackgroundAcquired reactive perforating collagenosis (ARPC) is a rare form of transepithelial elimination in which altered collagen is extruded through the epidermis.Case presentationA 23-year-old male presented with cup-like ulcerated lesions on his limbs since 3 months. A series of serological and immunological tests showed no abnormalities. A diagnosis of ARPC was based on skin biopsy findings. The patient was cured using treatment with itraconazole for 8 weeks, in the absence of a fungal infection.ConclusionsThe anti-inflammatory and anti-angiogenic effects of itraconazole can have good therapeutic benefits for ARPC.

425 A superfacial ulcer on the scrotum

A 54-year-old man presented to the dermatology clinic with an itchy red papule on the right scrotum for 2 years, which gradually expanded to a yellow central ulcerated plaque and the crust was not easy to be removed. Topical mupirocin and corticosteroids were not effective. Fluorescent microscopy for fungus was negative, as well as antibodies to syphilis and HIV. There was no similar history in his family, and he had hypertension for 14 years, type 2 diabetes for a year with normal renal function. Biopsy revealed epidermal necrosis and crust with transepidermal penetration and elimination of collagen. Therefore, the diagnosis was acquired reactive penetrating collagenosis (ARPC). Follow-up for 3 months after resection without recurrence. Reactive perforating collagenosis is a rare skin disease that includes both inherited and acquired forms. ARPC is associated with diabetes, chronic renal failure, hyperuricemia and hypertension. It usually occurs on the trunk and limbs, characterized by multiple umbilicated hyperkeratotic papules or nodules with Koebner's phenomenon clinically, but no scrotal lesions have been reported.

Trauma-induced acquired reactive perforating collagenosis

Trauma-induced acquired reactive perforating collagenosis

Clinicopathological Differences between Acquired Reactive Perforating Collagenosis and Prurigo Nodularis

Background: Acquired reactive perforating collagenosis (ARPC) is a skin disease characterized by multiple itchy nodules, which is notably similar to the skin eruptions of prurigo nodularis (PN). The aim of this study was to prove the clinicopathological differences between ARPC and PN. Methods: We examined 22 patients with ARPC (6 males and 16 females) and 38 patients with PN (27 males and 11 females), diagnosed clinically and histologically. Transepidermal elimination of collagen which is characteristic histological findings for ARPC was found in all ARPC patients, but not in PN patients even in a serial section. Clinical findings, laboratory data, histological findings, immunohistochemical results, and efficacy of the therapies were compared between the two groups. Results: On evaluation of laboratory data, thymus and activation-regulated chemokine (TARC) levels and peripheral eosinophil counts were lower in ARPC than in PN. Histologically, we observed greater infiltration of neutrophils, histiocytes, and lymphocytes and more extensive capillaries in ARPC than in PN. Upon immunohistochemical analysis, CD163+ macrophages were found to be more abundant in the upper-to-deep dermis of ARPC, and both CD4+ T cells and CD8+ T cells were significantly increased in the mid-to-deep dermis of ARPC. The efficacies of topical steroid and antihistamines were improved by the addition of emollients in ARPC. Conclusion: This is the first report to show the histological differences in cell infiltration between the lesions of ARPC and PN. It is likely that inflammation mediated by neutrophils and M2 macrophages may be involved in the formation of ARPC lesions, as against PN lesions mediated by epidermal changes such as spongiosis. It is suggested that the addition of emollients can increase the efficacy of treatments for ARPC. However, further studies are necessary to define the precise pathomechanisms of ARPC and the effects of emollients on ARPC.

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

Acquired perforating dermatosis (APD) is a group of a rare dermatological disorder characterized by elimination of dermal connective tissue through epidermis. We aimed to evaluate the characteristics of patients diagnosed with APD and to determine the differences in comorbidities according to subtypes of APD. A retrospective, observational, cross-sectional study was designed. Patients diagnosed with APD between January 2008 and January 2019 were reviewed. Eighty patients were included in the study. 61.2% (n = 49) of the patients were female and 38.8% (n = 31) were male with a mean age of 58.4 ± 12.5 years. 82.5% (n = 66) of the patients were diagnosed with reactive perforating collagenosis (RPC) and 17.5% (n = 14) of perforating folliculitis (PF). The most common concomitant disease was diabetes mellitus (82.5%). 5.0% of the patients had malignancy. The comorbidity rate in RPC group was higher than PF (P < .05). Topical steroid was the most frequently (90.0%) used treatment. Complete response was obtained 55.0% of patients. Exitus was observed in 23.8% (n = 19) of patients in a mean 17.6 ± 25.7 months follow-up period. APD may be associated with many diseases. Comorbidities are more frequent in RPC group. This situation warns us to evaluate patients with RPC in more detail for underlying diseases. High mortality rate related to the underlying systemic diseases suggests being careful in terms of mortality in patients diagnosed with APD.