Location
Suwanee, GA
Start Date
7-5-2024 1:00 PM
End Date
7-5-2024 4:00 PM
Description
Background:
Reactive Perforating Collagenosis (RPC), a subtype of perforating dermatosis, is a rare skin condition in which altered collagen is eliminated through the epidermis. There are two forms of RPC: (1) the inherited form which is very rare and presents in childhood, and in this case (2) the acquired form which presents in adulthood and is associated with systemic diseases, most notably reported being diabetes mellitus and/or chronic renal failure. This case highlights the importance of having a high clinical index of suspicion in patients with systemic disease who present with diffuse pruritic keratotic papules.
Case Presentation:
A 65 year-old woman presented with an intensely pruritic rash on the arms, legs, neck, and face for several months. Patient reported scratching the affected areas but denied any trauma. Patient was seen prior to this visit by her PCP and was prescribed cephalexin with no improvement. Physical examination of the skin revealed hard white cutaneous nodules distributed on the right proximal posterior upper arm, left proximal posterior upper arm, right proximal pretibial region, left proximal pretibial region, right elbow, and left knee. Patient has a past medical history of chronic renal failure on dialysis, diabetes mellitus, hypertension, and congestive heart failure. Two shave biopsies biopsy were obtained for histopathology with hematoxylin and eosin stain, which revealed invagination of the epidermis with hyperkeratosis, prominent epidermal hyperplasia, and a central basophilic plug of keratin, collagen, and inflammatory debris. Transepidermal elimination of bright eosinophilic altered collagen fibers were seen, clenching the diagnosis of Acquired Reactive Perforating Collagenosis.
Conclusion:
ARPC is a rare and unique perforating dermatoses that is commonly associated with systemic disease, most notably chronic kidney disease and diabetes mellitus. The other primary perforating dermatoses, Perforating Folliculitis (PF), Elastosis Perforans Serpiginosa (EPS), and Kyrle’s Disease (KD are similarly characterized by elimination of altered dermal material through the epidermis. As these conditions present with similar features on clinical exam, differentiation is made by the type of epidermal damage and the features of elimination material, making histopathological examination paramount for definitive diagnosis. As there is not a definitive treatment for ARPC, management of underlying systemic diseases is crucial. To reduce severity and prolongation of symptoms, increased clinician awareness of the dermatological conditions associated with systemic diseases is needed.
Embargo Period
7-2-2024
Included in
Acquired reactive perforating collagenosis: A case report
Suwanee, GA
Background:
Reactive Perforating Collagenosis (RPC), a subtype of perforating dermatosis, is a rare skin condition in which altered collagen is eliminated through the epidermis. There are two forms of RPC: (1) the inherited form which is very rare and presents in childhood, and in this case (2) the acquired form which presents in adulthood and is associated with systemic diseases, most notably reported being diabetes mellitus and/or chronic renal failure. This case highlights the importance of having a high clinical index of suspicion in patients with systemic disease who present with diffuse pruritic keratotic papules.
Case Presentation:
A 65 year-old woman presented with an intensely pruritic rash on the arms, legs, neck, and face for several months. Patient reported scratching the affected areas but denied any trauma. Patient was seen prior to this visit by her PCP and was prescribed cephalexin with no improvement. Physical examination of the skin revealed hard white cutaneous nodules distributed on the right proximal posterior upper arm, left proximal posterior upper arm, right proximal pretibial region, left proximal pretibial region, right elbow, and left knee. Patient has a past medical history of chronic renal failure on dialysis, diabetes mellitus, hypertension, and congestive heart failure. Two shave biopsies biopsy were obtained for histopathology with hematoxylin and eosin stain, which revealed invagination of the epidermis with hyperkeratosis, prominent epidermal hyperplasia, and a central basophilic plug of keratin, collagen, and inflammatory debris. Transepidermal elimination of bright eosinophilic altered collagen fibers were seen, clenching the diagnosis of Acquired Reactive Perforating Collagenosis.
Conclusion:
ARPC is a rare and unique perforating dermatoses that is commonly associated with systemic disease, most notably chronic kidney disease and diabetes mellitus. The other primary perforating dermatoses, Perforating Folliculitis (PF), Elastosis Perforans Serpiginosa (EPS), and Kyrle’s Disease (KD are similarly characterized by elimination of altered dermal material through the epidermis. As these conditions present with similar features on clinical exam, differentiation is made by the type of epidermal damage and the features of elimination material, making histopathological examination paramount for definitive diagnosis. As there is not a definitive treatment for ARPC, management of underlying systemic diseases is crucial. To reduce severity and prolongation of symptoms, increased clinician awareness of the dermatological conditions associated with systemic diseases is needed.