Collagenosis reactive perforating L87.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.09.2022

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Acquired reactive perforating collagenosis; Acquired reactive perforating dermatosis; Collagenoma perforans verruciformis; Collagenosis familial reactive perforating; Familial reactive perforating collagenosis; Perforating collagenosis; Reactive perforating collagenosis; Reactive perforating dermatosis

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Rare chronic skin disease characterized by transepidermal elimination of collagen and/or elastin through the skin. In the acquired collagenosis form, which occurs in adults, it is often associated with diabetes mellitus and/or chronic end-stage renal disease.

Basically, 2 clinical syndromes are to be distinguished:

  • The much more frequent acquired reactive collagenosisof the adult, which occurs mainly in diabetics and patients with renal insufficiency (incidences of up to 11% have been described). Acquired reactive collagenosis has also been described in patients with acute myeloid leukemia and after therapy with sorafenib.
  • The very rare familial (primarily described as reactive perforating collagenosis) focal connective tissue degeneration with transepithelial shedding of collagen fibers occurs predominantly in children. It is assumed that this genodermatosis is identical with the"hyperkeratosis follicularis et parafollicularis in cutem penetrans" described by Kyrle.

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1) Genodermatosis with unexplained etiopathogenesis.

2) Acquired in underlying diseases such as chronic terminal renal failure or diabetes mellitus.

3) Drug induced (e.g. by sorafenib - Vega Díez D et al. 2020).

The clinical picture has also been described in scabies and zoster, although the triggering of the disease in these cases is more likely to be reactive as a "Köbner phenomenon" with a corresponding disposition, or as an isotopic reaction.

Remark: probably as a result of mechanical or inflammatory irritation in receptive skin, there is focal damage to the collagenous connective tissue of the skin (scratching can induce RPK experimentally - Köbner phenomenon), which is finally discharged transepidermally.

Proteolytic enzymes such as matrix metalloproteases or serine proteases and modifications of sugar side chains on extracellular matrix proteins such as collagen I and III play an important role.

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In classic reactive perforating collagenosis, the mean age of onset is 50-60 years. The average duration of disease is 8 months.

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Mainly extensor sides of the extremities and the trunk. The face, palms of hands and soles of feet remain free of symptoms, as do the mucous membranes.

Clinical features
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There is moderate to distinct itching. Clinically there are mostly symmetrically distributed, few to numerous, disseminated or linearly grouped ( Koebner phenomenon), red, firm papules or plaques with central ulceration and hard, firmly adhering keratotic clot, initially about 0.2 cm in size, then slowly increasing to 1.0 -2.0 cm in size. Typical is the detection of a central crusty defect formation already in initial papules. After its detachment, an ulcerated crater is found, which heals spontaneously after 2-4 weeks with scarring due to hyperpigmentation.

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Hyperplastic epidermis with central bowl-shaped exulceration. In the material stored here, there are horny masses, granulation tissue with triggered, basophilic, collagenous fiber bundles (TOE stain). In the underlying dermis, a nonspecific mixed inflammatory infiltrate is seen.

Differential diagnosis
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Prurigo simplex subacuta (most important differential diagnosis); no causative underlying disease. Eminently severe itching of the scratched papules.

Prurigo nodularis (see below Prurigo chronic).

Rare differential diagnoses:

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A causal therapy is otherwise not known. In the acquired forms, treatment of the underlying disease.

Trial with PUVA therapy or external retinoid.

Symptomatic is the use of highly potent corticoid externa (also on large areas), which has been proven to be effective according to own experience and the experience of some authors.

Alternative: Good experience has been made with systemically administered allopurinol (100 mg/day/p.o.). The mechanism of action is unclear. It is possible that an inhibition of the xanthine oxidase and an antioxidative effect may cause an inhibition of the cross-linking of collagen fibres.

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In the acquired form of perforating collagenosis, a relapsing course is known. There is a not inconsiderable tendency to spontaneous healing. It is important to treat the underlying disease (e.g. precise adjustment of diabetes mellitus).

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Perforations of the skin (perforating dermatoses) are found as a secondary phenomenon in a larger number of diseases.

For example: calcinosis cutis, foreign body granulomas, gout, chondrodermatitis nodularis chronica helicis, granuloma anulare, sarcoidosis, necrobiosis lipoidica, malignant melanoma, T-cell lymphoma, Paget's disease, Kyrle 's disease and others.

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  1. Fretzin DF, Beal DW, Jao W (1980) Light and ultrastructural study of reactive perforating collagenosis. Arch Dermatol 116: 1054
  2. Haneke E (1991) Symptomatic reactive perforating collagenosis. Z Hautkr 66: 725-728
  3. Karpouzis A et al (2010) Acquired reactive perforating collagenosis: current status. J Dermatol 37:585-592
  4. Karpouzis A et al (2004) Acquired reactive perforating collagenosis associated with myelodysplastic syndrome evolving to acute myelogenous leukaemia. Australas J Dermatol 45:78-79
  5. Kruger K et al (1999) Acquired reactive perforating dermatosis. Successful treatment with allopurinol in 2 cases. Dermatologist 50: 115-120
  6. Lotz C et al (2014) Successful treatment of acquired reactive perforating dermatosis with allopurinol. Act Dermatol 4: 84-87
  7. Mehregan AM, Schwartz OD, Livingood CS (1967) Reactive perforating collagenosis. Arch Dermatol 96: 277
  8. Vega Díez D et al (2020) Reactive perforating collagenosis: a rare side effect associated with sorafenib. Rev Esp Enferm Dig 112:960-961.
  9. Weiss SC (2003) Cutaneous mucormycosis secondary to acquired reactive perforating collagenosis. Cutis 72: 119-123


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