|Year : 1993 | Volume
| Issue : 1 | Page : 17-18
Familial giant reactive perforating collagenosis
Dilip Kachhawa, G Kalla, Bhushan Bharat Dingra
A 21-year old man presented with slowly growing, multiple, recurrent, discrete, asymmetrical hyperkeratotic and hyperpigmented papules of size varying between 10-15mm over face and upper limbs; usually after trauma. Histopathology confirmed the diagnosis of reactive perforating collagenosis. This case is reported because of unusual bigger size of the lesions.
|How to cite this article:|
Kachhawa D, Kalla G, Dingra BB. Familial giant reactive perforating collagenosis.Indian J Dermatol Venereol Leprol 1993;59:17-18
|How to cite this URL:|
Kachhawa D, Kalla G, Dingra BB. Familial giant reactive perforating collagenosis. Indian J Dermatol Venereol Leprol [serial online] 1993 [cited 2019 Dec 16 ];59:17-18
Available from: http://www.ijdvl.com/text.asp?1993/59/1/17/3869
Reactive perforating collagenosis (RPC) is a form of transepidermal elimination in which altered collagen is extruded from the epidermis. The disease usually starts as papules in early childhood. The papules develop umbilications with keratinous plugs. We report a case of RPC with a positive family history. The size of umbilicated papules was abnormally bigger, therefore we have named it giant RPC.
A 21- year-old man presented with slowly growing, multiple, recurrent, discrete, asymmetric, hyperkeratotic and hyperpigmented papules of size varying between 10-15 mm, over the face and upper limbs. The eruption started at the age of 15 years. Initially a skin-coloured papule appeared on dorsum of right finger which soon developed central umbilication filled with a keratinous plug which was initially adherent but soon became loose and finally fell off. Involution of lesion occurred with hyperpigmentation and epidermal atrophy.
Different stages of evolution and regression were seen at the same time, the lesions were showing Koebner's phenomenon. These lesions occurred at sites of acne, minor trauma because of shaving, and sometimes
Family history of such lesions was present in younger brother of 9 years who developed these lesions since early childhood. No history of diabetes, hepatic, renal or other systemic disorders was present.
Routine investigations were normal. Biopsy of the initial non- umbilicated papule showed an area of necrobiotic, deeply basophilic collagen in the papillary dermis. The old umbilicated lesion in which the plug was eliminated showed a central area of depression, an accumulation of parakeratotic keratin, basophilic collagen, and numerous pyknotic nuclei of inflammatory cells. The epidermis at the base of the plug was atrophic and showed perforation through which basophilic bundles of collagen extending upwards were extruded from the underlying dermis. This was confirmed by staining with von Gieson method. The periphery of the cup-shaped lesion showed epidermal hyperplasia. The elastic fibres were normal and seen only in the dermis.
According to Mehregan et al  RPC is an abnormal response to superficial trauma in which collagen causes irritation and perforation of the epidermis with transepidermal elimination.
Formation of giant lesions in RPC may be associated with increasing age as the younger brother of our patient had smaller lesions. Familial occurrence of RPC has been recorded only in some cases. ,,,
|1||Mehregan AH, Schwartz OD,Livinggood CS. Reactive perforating collagenosis. Arch Dermatol 1967; 96: 277-82.|
|2||WeinerAL. Reactive perforating collagenosis. Arch Dermatol 1970; 102: 540-4.|
|3||Kanan MW. Familial reactive perforating collgenosis, intolerance to cold. Br J Dermatol 1974; 91: 405-14.|
|4||Pasricha JS, Girgla HS, Kandhari KC. Reactive perforating collagenosis. Dermatologica 1971; 143: 353-6.|
|5||Kalla G, Malhotra Y K, Vyas MCR, Kothari A, Suhail A. Reactive perforating collagenosis. Ind J Dermatol Venereol Leprol 1989; 55: 258-9.|