Elastoidosis cutanea nodularis et cystica L57.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 16.12.2020

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Synonym(s)

Blackheads; blackheads of the cheeks; Cheek-blackhead; Cheek comedones; élastéidose cutanée nodulaire à kystes et à comédons Favre-Racouchot; Elastoidosis cutanea nodularis; Elastoidosis cutanée nodulaire à kystes et à comédons; Elastosis nodular with cysts and comedones; Favre Racouchot disease; Favre-Racouchot disease; Favre-Racouchouchoche disease; M. Favre Racouchot; M. Favre-Racouchot; Morbsu Favre-Racouchaud; Nodular elastosis with cysts and comedones; Nodular skin astoidosis with cysts and comedones; Skin astoidosis nodular with cysts and comedones; Solar elastotic syndromes

History
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Favre and Racouchot, 1951

Definition
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Solar (possibly combined with senile) atrophy, marked, nodular actinic elastosis with grouped, non-inflammatory, black, expressible comedones and yellowish follicular cysts.

Etiopathogenesis
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Pathogenetically, the localization at chronic light-exposed, mostly hyperpigmented skin areas indicates chronic actinic damage of the cutaneous connective tissue. The resulting loss of elasticity of the connective tissue leads to horn retention in the follicles at the exposed sites (zygomatic bone), to multiple open, black stained, actinic comedones. The follicles are widened by the horn retention. In addition to open comedones, closed epithelial cysts can also be found in advanced clinical pictures. This results in a mixed picture of actinic elastosis, grouped (non-inflammatory) black comedones and deep-seated epithelial cysts.

Manifestation
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Occurs mainly in men over 50.

Localization
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Primarily zygomatic region, also traumatized body areas.

Clinical features
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Thickened, yellowish, but mostly clearly brownish, hyperpigmented, wrinkled (elastotic) skin over the zygomatic arches, in the lateral periorbital and temporal region as well as on the nose (so-called lemon skin) and neck(cutis rhomboidalis nuchae).

Single, but mostly grouped, non-inflamed comedones, with black corneal plugs in strongly dilated follicular openings (open comedones) as well as larger whitish-yellowish follicular cysts filled with crumbly fatty masses (closed comedones). In the zygomatic region mostly distinct hypertrichosis.

Rarely ectopic localisation is possible (so-called actinic comedone plaque on the forearm).

Frequently in combination with cutaneous precanceroses or carcinoma formations. Rarely the clinical picture is an indication of an underlying porphyria cutanea tarda.

Histology
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Severe laminar or nodular elastosis of the upper and middle dermis, completely replacing the normal texture of the dermis; enlarged horny follicles with hair segments, atrophy of the follicle wall and sebaceous glands. Frequent melanin deposits in the superficial horny lamellae.

Differential diagnosis
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Porphyria cutanea tarda (uro- and coproporphyrins); chronic terminal renal failure (dialysis patients). Strictly speaking, these are not differential diagnoses but an identical process initiated by an underlying disease state.

Therapy
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Avoid direct sunlight, light protection (e.g. Anthelios). Express with a limited number of comedones (after softening by applying warm and moist compresses for several hours). Express open comedones with a comedone squeezer, carefully score and express closed comedones with a lancet or cannula. In case of pronounced comedone formation or in addition to expression, peeling treatment with retinoids 0.025-0.1% tretinoin in cream, gel or solution (e.g. Airol cream, solution, Cordes VAS) or 0.5% isotretinoin (e.g. Isotrex gel). Apply thinly 1-2 times/day. Initially daily, as soon as the effect occurs, only every 2nd or 3rd day. Cave! Avoid sun exposure under retinoid therapy!

Literature
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  1. Breit S et al (2003) Favre-Racouchot-like disease after radiation therapy. J Am Acad Dermatol 49: 117-119
  2. Favre M (1932) Sur une affection kystique des appareils pilo-sébacés localisée à certaines régions de la face. Bull Soc Franc Derm Syph (Lyon) 39: 93-96
  3. Favre M, Racouchot J (1951) L'élastéidose cutanée nodulaire à kystes et à comédons. Ann Derm Syph 78: 681-702
  4. Lewis KG et al (2004) Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes. J Am Acad Dermatol 51: 1-21
  5. Lugo A et al (2005) Bullous solar elastosis. At J Dermatopathol 27: 34-35
  6. Mavilia L et al (2002) Unilateral nodular elastosis with cysts and comedones (Favre-Racouchot syndrome): report of two cases treated with a new combined therapeutic approach. Dermatology 204: 251
  7. Patterson WM et al (2004) Favre-Racouchot disease. Int J Dermatol 43: 167-169
  8. Racouchot J (1937) L'élastéidose localisée nodulaire à kystes et à comédons. Thèse de Lyon
  9. Siragusa M et al (2000) An unusual location of nodular elastosis with cysts and comedones (Favre-Racouchot's disease). Acta Derm Venereol 80: 452
  10. Stefanidou M et al (2001) Unilateral nodular elastosis with cysts and comedones (Favre-Racouchot syndrome). Dermatology 202: 270-271

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Last updated on: 16.12.2020