Acquired Reactive Perforating Collagenosis
Acquired reactive perforating collagenosis (ARPC) is a rare dermatological condition characterized by the development of hyperkeratotic, umbilicated papules or nodules, often accompanied by excoriations and crusts. The condition is typically associated with systemic diseases, particularly diabetes mellitus and chronic renal failure, but can also be linked to other underlying conditions or triggers. This article provides a comprehensive overview of ARPC, focusing on its clinical presentation, diagnostic methods, histopathological features, and treatment options, based on a detailed case study.
Clinical Presentation
The patient in this case was a 50-year-old man who presented with a 1-month history of pruritic papules and nodules on his trunk and extremities. The patient had a 20-year history of alcoholism and a 10-year history of Meniere’s disease. Two months prior to the onset of the skin lesions, he had been treated with intravenous sodium aescinate for Meniere’s disease. The patient denied any other chronic diseases or significant family medical history.
On physical examination, the patient exhibited red or brown papules and nodules, ranging in diameter from 3 to 5 mm, located on his limbs, shoulders, and dorsum. The lesions were characterized by central umbilicated necrosis or keratin plugs and were accompanied by the Kobner Phenomenon, which refers to the development of new lesions at sites of skin trauma. The clinical presentation was consistent with the typical features of ARPC, which often includes hyperkeratotic, umbilicated, millimetric or larger papules or nodules.
Diagnostic Evaluation
The diagnostic evaluation of ARPC involves a combination of clinical examination, dermoscopy, and histopathological analysis. In this case, the patient underwent ultrasonic examination, peripheral blood cell count, and biochemical examination, all of which were essentially normal. Dermoscopic examination revealed a red-brown structureless area covered with crusts and scales centrally, surrounded by a white rim, and a reddish inflammatory circle with looped and dotted vessels peripherally in polarization mode. These dermoscopic findings are consistent with the typical features of ARPC, as described in the literature.
Histopathological examination is crucial for the definitive diagnosis of ARPC. In this case, histopathology showed neutrophils and degenerated keratin components in the central goblet necrotic epidermis, degenerated collagen fibers beneath the necrotic epidermis, and a sheet of lymphocytes and scattered eosinophils around blood vessels in the dermis. Masson staining and Verhoeff-van Gieson staining confirmed the penetration of collagen fibers and fragmented elastic fibers in the necrotic epidermis. These histopathological findings are characteristic of ARPC and help to differentiate it from other perforating dermatoses.
Pathophysiology
The exact pathophysiology of ARPC is not fully understood, but it is believed to involve a reactive process in which collagen fibers are extruded through the epidermis. This process is thought to be triggered by various factors, including systemic diseases, medications, and local trauma. In this case, the patient’s history of alcoholism and Meniere’s disease, as well as the recent treatment with intravenous sodium aescinate, may have contributed to the development of ARPC.
The histopathological features of ARPC typically include a dome-shaped lesion with a central crater and epidermal ulceration, consisting of keratin, polymorphonuclear cell debris, and fibers (mainly collagen fibers) which are extruded from the bottom of the crater. In this case, both collagen fibers and fragmented elastic fibers were confirmed in the necrotic region, which is consistent with the typical pathological features of ARPC.
Treatment and Management
The management of ARPC aims to control systemic disease and alleviate pruritus, but there is currently no consensus on the optimal treatment approach. Various therapeutic options have been reported to be effective, including emollients, antihistamines, keratolytics, doxycycline, retinoids, steroids, allopurinol, ultraviolet B phototherapy, and transcutaneous electrical nerve stimulation.
In this case, the patient was treated with oral antihistamines, topical steroids, and narrow-band ultraviolet B phototherapy for 5 weeks. The patient reported clearance of the lesions for 4 months, but experienced a recurrence after alcohol intake, as reported during a telephone follow-up. This highlights the importance of addressing underlying systemic conditions and triggers in the management of ARPC.
Dermoscopic Features
Dermoscopy is a valuable tool in the diagnosis of ARPC, as it can reveal characteristic features that facilitate early diagnosis. The common dermoscopic manifestations of ARPC include three key features, which were all observed in this case. Firstly, the center of the lesion is a yellow-brown structureless area, consistent with surface crust. Secondly, this area is surrounded by a white rim of varying thickness. Thirdly, there is an outer pink inflammatory circle with commonly short looped vessels centrally and dotted vessels peripherally. The coexistence of these three features is highly suggestive of ARPC and can aid in the early diagnosis of the condition.
Histochemical Staining
Histochemical staining is an essential component of the histopathological evaluation of ARPC. In this case, Masson staining and Verhoeff-van Gieson staining were used to confirm the presence of collagen fibers and fragmented elastic fibers in the necrotic epidermis. Masson staining is a trichrome stain that highlights collagen fibers, while Verhoeff-van Gieson staining is used to visualize elastic fibers. These staining techniques provide valuable information about the composition of the necrotic material and help to confirm the diagnosis of ARPC.
Conclusion
Acquired reactive perforating collagenosis is a rare dermatological condition that can be challenging to diagnose due to its varied clinical presentation and the need for histopathological confirmation. The case presented here illustrates the typical clinical features, dermoscopic changes, and histopathological findings of ARPC. Dermoscopy and histochemical staining are valuable diagnostic tools that can facilitate the early diagnosis of ARPC. The management of ARPC involves addressing underlying systemic conditions and triggers, as well as using a combination of therapeutic options to control symptoms and prevent recurrence.
In summary, ARPC is a complex condition that requires a multidisciplinary approach for accurate diagnosis and effective management. The case highlights the importance of a thorough clinical evaluation, the use of diagnostic tools such as dermoscopy and histochemical staining, and the need for a tailored treatment plan to address the underlying causes and symptoms of the condition.
doi.org/10.1097/CM9.0000000000000906
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