Acquired reactive perforating collagenosis

Abstract

Background:Reactive perforating collagenosis (RPC) is a rare form of transepithelial elimination, in which altered collagen is extruded through the epidermis. There are 2 types of RPC, acquired RPC (ARPC) and inherited RPC, while the latter is extremely rare. Here we report on 1 case of ARPC.Methods:A 73-year-old female was presented with strongly itchy papules over her back and lower limbs for 3 months. She denied the history of oozing or vesiculation. A cutaneous examination showed diffusely distributed multiple well-defined keratotic papules, 4 to 10 mm in diameter, on the bilateral lower limbs and back as well as a few papules on her chest and forearm. Scratching scars were over the resolved lesions while Koebner phenomenon was negative. The patient had a history of type 2 diabetes for 15 years. Laboratory examinations showed elevated blood glucose level. Skin lesion biopsy showed a well-circumscribed area of necrosis filled with a keratotic plug. Parakeratotic cells and lymphocytic infiltration could be seen in the necrosed area. In dermis, sparse fiber bundles were seen perforating the epidermis. These degenerated fiber bundles were notarized as collagen fiber by elastic fiber stain, suggesting a diagnosis of RPC.Results:Then a diagnosis of ARPC was made according to the onset age and the history of diabetes mellitus. She was treated with topical application of corticosteroids twice a day and oral antihistamine once a day along with compound glycyrrhizin tablets 3 times a day. And the blood glucose was controlled in a satisfying range. Two months later, a significant improvement was seen in this patient.Conclusion:Since there is no efficient therapy to RPC, moreover, ARPC is considered to be associated with some systemic diseases, the management of the coexisting disease is quite crucial. The patient in this case received a substantial improvement due to the control of blood glucose and application of compound glycyrrhizin tablets.

Acquired reactive perforating collagenosis associated with Hodgkin disease

“…Acquired reactive perforating collagenosis (ARPC) is a perforating disorder characterized by transepidermal elimination of altered collagen . It is associated with multiple disorders, especially diabetes mellitus, but it has only been described in association with Hodgkin lymphoma (HL) (nodular sclerosis type) in three previous cases .…”

“…Reactive perforating collagenosis (RPC) is a perforating disorder, characterized by transepidermal elimination of altered collagen . The other main diseases in this group are elastosis perforans serpiginosa, Kyrle disease and perforating folliculitis . Mehregan et al .…”

“…There are two patterns of RPC: inherited (typical of childhood) and acquired (typical of adulthood), with the latter being the most common pattern . ARPC is associated with various disorders, especially diabetes mellitus, but also hyperparathyroidism, hypothyroidism, liver disorders, neurodermatitis, autoimmune diseases such as systemic lupus erythematous, vasculitis, dermatomyositis, or Mikulicz disease, and some malignancies, including lymphoma, thyroid carcinoma or breast carcinoma . The association with Hodgkin lymphoma has been reported only in three cases, all of them related to the nodular sclerosis type .…”

“…Pruritus and scratching are the common factor between all these pathologies, and control of pruritus helps clear the lesions . It is unclear if ARPC is primary in these cases, but most authors consider it a reaction to a traumatic stimulation, such as scratching, which induces transepidermal elimination of the degenerated collagen fibres . The disease classically erupts as multiple round plaques and nodules with central hyperkeratotic plugs, distributed on the extremities and back, and sometimes the face and neck .…”

“…It is unclear if ARPC is primary in these cases, but most authors consider it a reaction to a traumatic stimulation, such as scratching, which induces transepidermal elimination of the degenerated collagen fibres . The disease classically erupts as multiple round plaques and nodules with central hyperkeratotic plugs, distributed on the extremities and back, and sometimes the face and neck . The histopathology shows in the early stages thickening of the epidermis and widening of the papillary dermis with degenerated collagen bundles.…”