Sciresol Sciresol https://jmsh.ac.in/ Journal of Medical Sciences and Health 10.46347/jmsh.v11.i1.24.134 CASE SERIES Acquired Reactive Perforating Collagenosis - Report of Four Cases Case series of Acquired reactive perforating collagenosis Raghavan Vijayashree 1 Thajudeen Ayeesha Sithika dr.ayeesha1@gmail.com 2 Chandrasekaran Kundhavai 3 Professor & HOD, Department of Pathology, Chettinad Hospital and Research Institute Kelambakkam, Chengalpattu, Tamil Nadu India Associate Professor, Department of Pathology, Chettinad Hospital and Research Institute Kelambakkam, Chengalpattu, Tamil Nadu India Assistant Professor, Department of Pathology, Chettinad Hospital and Research Institute Kelambakkam, Chengalpattu, Tamil Nadu India 11 1 98 2025 <bold id="s-674c4eab1380">Abstract</bold>

Acquired reactive perforating collagenosis (ARPC) is a rare skin disorder occurring more common in patients with chronic kidney disease or diabetes. It is caused by the perforation of dermal connective tissue through the epidermis. We report four cases of acquired reactive perforating collagenosis in patients with chronic diabetes, confirmed by histopathology.

Keywords Perforating collagenosis Diabetes Kidney disease <bold id="s-32a445aae1a8">Background</bold>

The perforating disorders comprise a group of disorders sharing the common characteristic of trans epidermal elimination (TEE). This phenomenon is characterized by altered collagen extruding through the epidermis. 1 They are two forms, acquired and inherited. Acquired reactive perforating collagenosis (ARPC) is a rare skin disorder occurring more common in patients with chronic kidney disease or diabetes. 2 It is caused by the perforation of dermal connective tissue through the epidermis.

<bold id="strong-0eb49cc55e6b47a18a4e89744c37eddf">Case reports</bold>

Case 1: A 53-year-old male came with complaints of hyperpigmented follicular papules with central crater over the arms and legs for 3 months [Figure 1 a]. Known case of Diabetes mellitus for 19 years.

Sections from the skin showed hyperkeratosis, irregular acanthosis with crater like depression, basophilic material with altered collagen fibres in the crater exhibiting trans epidermal elimination [Figure 1 b]. The underlying papillary dermis shows moderate lymphocytic infiltrate.

<bold id="strong-efb61b816c2c4baeb4b506e72028245d">a:</bold> <bold id="strong-abf9414936a9492ba2f66c9edad9d7e6"> Scattered hyperpigmented follicular papules with central crater over the leg</bold> <bold id="strong-22dc1c6507c64f86aeb3f28b7b79501e">, </bold> <bold id="strong-ca9e463d5b5446928d8dd9d3a5ecf669">b:</bold> <bold id="strong-fef09fb7943649148be92471a73363e0"> </bold> <bold id="strong-945f57847bf74922b513a7e1189aeca5">(H&E,40X) Section shows cup like invagination of basophilic plug with altered </bold> <bold id="strong-117026b8873d4b6d9923bcc1fe63967f">collagen</bold>

Case 2: A 71-year-old male with complaints of multiple itchy hyperpigmented follicular papules with central crater over upper limbs, lower limbs and back [Figure 2a]. He is a known diabetic.

Sections from the skin showed vertically oriented shallow invaginating epidermal channel with basophilic necrotic and degenerated collagen material. Rest of the epidermis show acanthosis, spongiosis and mild exocytosis. Moderate perivascular lymphocytic infiltrate in the papillary dermis [Figure 2 b].

<bold id="strong-f7af8a15a60949199e84cbfc92918bd3">a: </bold> <bold id="strong-ac5f03e8080f4c458a5b168232ce2efc">Crater shaped nodules and plaques over arm</bold> <bold id="strong-da1a747346654edca5f87351dbb22615">, b: </bold> <bold id="strong-3febd3e5c0ec43b08016f2e30e50d052">(H&E,40X)</bold> <bold id="strong-3dd8d5488532429aa57f64666aa61089"> basophilic necrotic and degenerated collagen material</bold>

Case 3: A 62-year-old female came with complaints of multiple skin lesions for 4 months [Figure 3 a].

Sections from the skin showed hyperplastic epithelium with focal ulceration and an adjacent invagination lined by stratified squamous epithelium, containing dense basophilic plug of keratin, altered collagen bundles and inflammatory debris. Underlying dermis showed moderate chronic inflammatory infiltrate. Masson’s Trichrome stain highlighted collagen fibres in the lesion [Figure 3 b].

<bold id="strong-acdaaee3282148458255dbffae09a705"/> <bold id="strong-730850e72a7d4396bafd52badcf1ecf3">a: </bold> <bold id="strong-280629a39c854c35a16549dd4e0adf12">Cup shaped ulcers with hyperpigmented papules over arm</bold> <bold id="strong-899743c70be74cc29f3cd06d52b3963e">, b: </bold> <bold id="strong-6742f45683c140cf880c0327652e9c56">(Masson Trichrome 10X) Section highlights the collagen </bold> <bold id="strong-b99b74861bfa4246887e01fd94e950bc">fibres</bold> <bold id="strong-77fd0d34a35f42d88d97ea50541e1634"> </bold>

Case 4: A 61-year-old female came with complaints of pigmented keratotic papule in the right thigh. She is a known case of diabetes for 8 years.

Sections from the skin showed a crater filled with keratin, collagen fibres and neutrophilic exudate. The stratum corneum shows entrapped plasma, the epidermis shows transient acantholysis with neutrophilic micro abscess. The superficial dermis shows proliferating capillaries with chronic inflammatory infiltrate composed of lymphocytes and plasma cells.

<bold id="s-49e6eb33aff8">Discussion</bold>

ARPC is a rare form of perforating dermatosis. Other forms include Kyrle disease, perforating folliculitis, and elastosis perforans serpiginosa.3 ARPC is diagnosed based on the visual or histological confirmation of trans epidermal elimination of degenerated fibres. The typical eruption has a central yellow-to-greenish crust, which represents degenerated dermal collagens. 4

Overexpression of transforming growth factor-3 has been shown around the cup shaped epidermal depression, which plays a crucial role in connective tissue metabolism and is involved in wound healing. TGF Beta, matrix metalloproteinase-1 and tissue inhibitor of metalloproteinase-1 immunoreactivity was significantly increased in the lesions which play an important role in regulation of epidermal homeostasis, delay in reepithelialisation and remodelling, and alterations in extracellular matrix protein metabolism. 5 The lesions are sometimes accompanied by severe pruritus and can impair the patient’s quality of life; therefore, clinicians should be aware of these characteristic eruptions in patients with diabetes or chronic kidney disease receiving haemodialysis for better management. 6

Disclosure Funding

Nil

 Conflict of interest

Nil

References Reid J Almond L Matthewman N Stringer H Francis N Abadie M Al A case of acquired reactive perforating collagenosis Australasian Journal of Dermatology 2018 59 1 e75 e76 https://doi.org/10.1111/ajd.12618 Poliak S C Lebwohl M G Parris A Prioleau P G Reactive perforating collagenosis associated with diabetes mellitus The New England Journal of Medicine 1982 306 2 81 84 https://doi.org/10.1056/nejm198201143060206 Fei C Wang Y Gong Y Xu H Yu Q Shi Y Acquired reactive perforating collagenosis: A report of a typical case Medicine (Baltimore) 2016 95 30 1 4 https://doi.org/10.1097/md.0000000000004305 Ye B Cao Y Liu Y Successful treatment of acquired reactive perforating collagenosis with itraconazole European Journal of Medical Research 2021 26 1 1 4 https://doi.org/10.1186/s40001-021-00542-6 Herzinger T Schirren C G Sander C A Jansen T Kind P Reactive perforating collagenosis-transepidermal elimination of type IV collagen Clinical and Experimental Dermatology 1996 21 4 279 282 https://doi.org/10.1111/j.1365-2230.1996.tb00094.x Lo Y P Snehal D Deng L H Chang C H Shih C J Successful treatment of acquired reactive perforating collagenosis induced by pregnancy with allopurinol: A case report with review of literature Dermatologica Sinica 2019 37 3 162 165 https://doi.org/10.4103/ds.ds_47_18