DefinitionThis section has been translated automatically.
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.
EtiopathogenesisThis section has been translated automatically.
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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ManifestationThis section has been translated automatically.
In classic reactive perforating collagenosis, the mean age of onset is 50-60 years. The average duration of disease is 8 months.
LocalizationThis section has been translated automatically.
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 featuresThis section has been translated automatically.
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.
HistologyThis section has been translated automatically.
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 diagnosisThis section has been translated automatically.
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:
- Elastosis perforans serpiginosa (anular grouped papules with adherent keratoses).
- perforating granuloma anulare (skin-colored bifurcated papules)
- calcinosis cutis (rock-hard, pointed calcifications)
- Hyperkeratosis follicularis et parafollicularis in cutem penetrans (predominantly localized on the legs)
- Ecthyma (V.a. lower leg)
TherapyThis section has been translated automatically.
A causal therapy is otherwise not known. In the acquired forms, treatment of the underlying disease.
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.
Progression/forecastThis section has been translated automatically.
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).
Note(s)This section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- Fretzin DF, Beal DW, Jao W (1980) Light and ultrastructural study of reactive perforating collagenosis. Arch Dermatol 116: 1054
- Haneke E (1991) Symptomatic reactive perforating collagenosis. Z Hautkr 66: 725-728
- Karpouzis A et al (2010) Acquired reactive perforating collagenosis: current status. J Dermatol 37:585-592
- Karpouzis A et al (2004) Acquired reactive perforating collagenosis associated with myelodysplastic syndrome evolving to acute myelogenous leukaemia. Australas J Dermatol 45:78-79
- Kruger K et al (1999) Acquired reactive perforating dermatosis. Successful treatment with allopurinol in 2 cases. Dermatologist 50: 115-120
- Lotz C et al (2014) Successful treatment of acquired reactive perforating dermatosis with allopurinol. Act Dermatol 4: 84-87
- Mehregan AM, Schwartz OD, Livingood CS (1967) Reactive perforating collagenosis. Arch Dermatol 96: 277
- Vega Díez D et al (2020) Reactive perforating collagenosis: a rare side effect associated with sorafenib. Rev Esp Enferm Dig 112:960-961.
- Weiss SC (2003) Cutaneous mucormycosis secondary to acquired reactive perforating collagenosis. Cutis 72: 119-123
Incoming links (13)Collagenoma perforans verruciformis; Collagenosis, familial, reactive, perforating; Dermatosis perforating; Elastosis perforans serpiginosa; Folliculitis perforating; Granuloma anulare perforans; Koebner phenomenon; Kyrle's disease; Prurigo simplex subacuta; Reactive perforating collagenosis; ... Show all
Outgoing links (20)Allopurinol; Calcinosis cutis (overview); Chondrodermatitis nodularis chronica helicis; Chronic prurigo; Cutaneous t-cell lymphomas; Ekthyma; Elastosis perforans serpiginosa; Granuloma anulare perforans; Koebner phenomenon; Kyrle's disease; ... Show all
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