Year : 2009 | Volume
: 52 | Issue : 1 | Page : 106--107
Reactive perforating collagenosis
Mukesh Kumar Yadav1, BC Sangal1, Puneet Bhargav2, P Rishi Jai3, Mukul Goyal3,
1 Department of Pathology, SMS Medical College, Jaipur, India
2 Department of Dermatology, SMS Medical College, Jaipur, India
3 Department of Medicine, SMS Medical College, Jaipur, India
Mukesh Kumar Yadav
D-17/B Meera Marg, Bani Park, Jaipur - 302016
Reactive perforating collagenosis is a rare cutaneous disorder of unknown etiology. We hereby describe a case of acquired reactive perforating collagenosis in a patient of diabetes and chronic renal failure.
|How to cite this article:|
Yadav MK, Sangal B C, Bhargav P, Jai P R, Goyal M. Reactive perforating collagenosis.Indian J Pathol Microbiol 2009;52:106-107
|How to cite this URL:|
Yadav MK, Sangal B C, Bhargav P, Jai P R, Goyal M. Reactive perforating collagenosis. Indian J Pathol Microbiol [serial online] 2009 [cited 2021 Sep 23 ];52:106-107
Available from: https://www.ijpmonline.org/text.asp?2009/52/1/106/44987
Reactive perforating collagenosis (RPC) is a rare disease of elimination of altered collagen through the epidermis.  The disease exists in childhood form with autosomal recessive mode of inheritance  and an adult form acquired in association with diseases such as diabetes mellitus (DM),  chronic renal failure (CRF),  hypothyroidism,  lymphoma,  hyperparathyroidism,  neurodermatitis,  AIDS,  pulmonary fibrosis, scabies  and herpes zoster infection.  We hereby describe a case of acquired reactive perforating collagenosis (ARPC) occurring in a patient of DM and CRF.
A 40-year-old female presented with complaints of severe pruritus and papulonodular eruptions all over the body since 2 months. The disease was initially localized on the dorsum of feet and hands and had gradually progressed to her present state in which extensive papulonodular lesions were present all over body with severe pruritus. The lesions appeared with itching, gradually enlarged in size and subsided by themselves in 6-8 weeks and by that time new lesions began to appear. She was a known case of DM since 20 years and was taking insulin for blood sugar control. She was also diagnosed as a case of CRF 5 years back, for which she was presently on conservative treatment.
On examination, there was pallor and pedal edema. There were multiple umbilicated papulonodular lesions with central keratotic plug spread over both extensor and flexor surface of body including face and sparing palm and sole. Lesions were seen in different stages of development and a Kobner's phenomenon was demonstrable. Residual scars and areas of hyperpigmentation were also visible. Rest of clinical examination was within normal limits. A clinical diagnosis of ARPC was made. Investigations revealed the following: Hemoglobin - 6.4 gm/dl, total leucocyte count - 5700 cu/mm with 64% polys., 20% lymphos., ESR - 70 mm - 1 st hour, Blood urea - 115 mg/dl, serum creatinine - 5.4 mg/dl, blood uric acid level -7.5 mg/dl, blood sugar (fasting) - 180 mg/dl and chest X-ray showed cardiomegaly. All other hemorheological parameters were within normal limits. Punch biopsy was taken from forearm lesion and on staining with hematoxylin and eosin stains, showed cup-shaped area of depression of epidermis filled with plug of necrotic material, parakeratotic keratinocytes, inflammatory cells and fine fibers of collagen [Figure 1]. Greater part of collagen was in the lower part of plug and surrounding epidermis showed slight acanthosis. On Masson's trichrome staining, perforating bundles of collagen are seen extending to the surface [Figure 2]. Staining for elastin fibers was negative.
In view of available investigations, the diagnosis of ARPC with DM with CRF was made. and treatment with prednisolone, isotretoin and antihistaminic was started. When reviewed after 1 month of therapy, there was symptomatic improvement in skin lesions.
RPC, first described by Mehregan et al . in 1967, is a type of essential perforating disorders.  The pathogenesis of RPC is unknown. As originally postulated by Mehregan et al ., "mild superficial trauma in genetically susceptible persons leads to necrobiosis of collagen in dermal papillae, which is subsequently eliminated from the dermis by means of transepithelial elimination," still seems to be correct. In RPC, the defect occurs in papillary dermis where histochemically altered but ultra structurally intact-type IV collagen is present,  which is surrounded and engulfed by focal epidermal proliferation. A central crater containing inflammatory cells, keratinous material and altered collagen then develops and the altered collagen is subsequently expelled by transepithelial migration.  Lesions of RPC are intensely itchy, shows Kobner's phenomena and mainly involve the extensor surface. Progressive itching leads to development of a skin-colored papule, which becomes umbilicated in 3-5 weeks and undergoes spontaneous regression in next 6-8 weeks, leaving a residual scar. At any given time, lesions may be seen in various stages of development.
This patient was a known case of DM and CRF, when presented to our department had intensely itchy papulonodular lesions, which had appeared over a period of 2 months. These were the lesions of ARPC. In our patient, the disease responded to treatment, itching decreased and new lesions did not appear during treatment.
RPC remains a rare but well recognized skin condition for dermatologists and pathologists.
|1||Mehregan AH, Schwartz OD, Livingood CS. Reactive perforating collagenosis. Arch Dermatol 1967;96:277-82.|
|2||Kanan MW. Familial reactive perforating collagenosis and intolerance to cold. Br J Dermatol 1974;91:405-14.|
|3||Faver IR, Daoud MS, Su WP. Acquired reactive perforating collagenosis. J Am Acad Dermatol 1994;30:575-80.|
|4||Pedragosa R, Knobel HJ, Huguet P, Oristrell J, Valdιs M, Bosch JA. Reactive perforating collagenosis in hodgkin's disease. Am J Dermatopathol 1987;9:41-4.|
|5||Bank DE, Cohen PR, Kohn SR. Reactive perforating collagenosis in a setting of double disaster: Acquired immunodeficiency syndrome and end stage renal disease. J Am Acad Dermatol 1989;21:371-4.|
|6||Brinkmeier T, Herbst RA, Frosch PJ. Reactive perforating collagenosis associated with scabies in a diabetic. J Eur Acad Dermatol Venerol 2004;18:588-90.|
|7||Bang SW, Kim YK, Wang KV. Acquired perforating collagenosis, unilateral umbilicated papule along the lesion of herpes zoster. J Am Acad Dermatol 1997;36:778-9.|
|8||Herzinger T, Schirren CG, Sander CA, Jansen T, Kind P. Reactive perforating collagenosis - transepidermal elimination of type 4 collagen. Clin Exp Dermatol 1996;21:279-82.|