Porokeratosis superficialis disseminata actinica Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 08.06.2021

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Synonym(s)

Actinic porokeratosis; disseminated porokeratosis; Disseminated superficial actinic porokeratosis; Disseminated superficial porokeratosis; Porokeratosis disseminated superficial actinic

History
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Chernosky and Freeman, 1967

Definition
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Not rare, hereditary or also non-hereditary, acquired, disseminated form of porokeratosis in light-exposed areas. The single-cell lesion is a skin-coloured, punctate, centrifugally progressing, hard, hyperkeratotic papule with formation of an annular, parakeratotic horny ridge and central atrophy.

Etiopathogenesis
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  • In some patients an irregular autosomal dominant inheritance can be demonstrated.
  • Several cytogenetic aberrations have been detected on the following gene loci: 1p31.3-p31.1 - 15q25.1-q26.1 and others
  • However, most patients deny a familial occurrence.
  • Excessive UV exposure can be detected in almost all cases. A combination with immunosuppression (HIV infection; immunosuppressive therapies) is not uncommon.
  • Disseminated superficial eosinophilic porokeratosis can be drug-induced (thiazides) or occur spontaneously.

Manifestation
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Mostly occurring in fair-skinned adults with increased sun exposure, mostly 40th-50th LJ, women seem to be preferentially affected.

Localization
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Back of the hand, forearm extension sides, lower leg extension sides, no mucosal involvement.

Clinical features
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Round to oval, 0.2-0.5-1.5 cm large, skin-coloured or brownish-red, slightly sunken horn papules or plaques with sharply defined, hyperkeratotic rim wall.

Histology
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Differential diagnosis
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Therapy
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  • Physical procedures such as curettage or ablative lasers (Co2 or erbium YAG laser)
  • Vitamin D3 analogues
  • Fluorouracil therapy
  • Photodynamic therapy
  • A smaller study (Schilling B 2018) reported good results with so-called borderline radiation (long-wave, soft X-rays).
  • Imiquimod
  • Tretinoin
  • Another theapy option is topical diclofenac in combination with etretinate (Shimizu S et al. 2018).
  • Light protection (e.g. Anthelios), see also below Light protection products.

Progression/forecast
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Progression with increasing age, especially in the summer months. The risk of malignant transformation to squamous cell carcinoma or basal cell carcinoma is reported to be 7.5% to 10% (Shimizu S et al 2018).

Notice. Provocation by UV rays and drugs (thiazides)!

Note(s)
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Superficial eosinophilic porokeratosis: As a special form, "Superficial eosinophilic porokeratosis" is described with eosinophilic spongiosis and intraepidermal microabscesses.

Eruptive pruriginous papular porokeratosis: A further, but very rare variant (up to now <15 cases have been described) is the "Eruptive pruriginous papular porokeratosis" which is characterized by a sudden itching. The plumelike parakeratosis zones are related to the follicle. Eosinophilic granulocytes are also an essential part of the inflammatory infiltrate in this form.

Literature
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  1. Altmeyer P et al (1988) Disseminated superficial eosinophilic porokeratosis. Akt Dermatol 14: 287-289
  2. Besenhard HM et al (1988) Disseminated superficial actinic porokeratosis (DSAP) with Bowen's disease. Dermatol 39: 286-290
  3. Biswas A (2015) Cornoid lamellation revisited: apropos of porokeratosis with emphasis on unusual clinicopathological variants. Am J Dermatopathol 37:145-155
  4. Boente Mdel C et al (2003) Linear porokeratosis associated with disseminated superficial actinic porokeratosis: a new example of type II segmental involvement. Pediatr Dermatol 20: 514-518
  5. Chernosky ME, Freeman RG (1967) Disseminated superficial actinic porokeratosis. Arch Dermatol 96: 611-624
  6. Chernosky ME, Anderson DE (1969) Disseminated superficial actinic porokeratosis: clinical studies and experimental production of lesions. Arch Derm 99: 401-407
  7. Le C, Bedocs PM (2021). Disseminated superficial actinic porokeratosis. 2020 Aug 10. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing PMID: 29083728.
  8. Nayeemuddin FA et al (2002) Topical photodynamic therapy in disseminated superficial actinic porokeratosis. Clin Exp Dermatol 27: 703-706
  9. Schilling B (2018) Marginal radiation in refractory actinic porokeratosis: a tolerable and effective therapy. Karger Compass 6:32-33
  10. Schwarz T et al (1984) Disseminated superficial actinic porokeratosis. J Am Acad Dermatol 11: 724-730.
  11. Shimizu S et al (2018) Inflammatory disseminated superficial porokeratosis successfully controlled with a combination of topical diclofenac gel and systemic etretinate. J Eur Acad Dermatol Venereol 32:e201-e202.

  12. Sidwell RU et al (2003) Giant actinic porokeratosis of the scalp. Br J Dermatol 149: 654
  13. Takahashi H et al (2015) Disseminated superficial actinic porokeratosis in a psoriasis patient with a long-term sun-bathing habit. J Dermatol 42:532-533
  14. Theng CT et al (2004) Warty nodules on an annular plaque in a renal transplant recipient. Arch Dermatol 140: 121-126
  15. Thomas C et al (2003) Hypertrophic perianal porokeratosis in association with superficial actinic porokeratosis of the leg. Clin Exp Dermatol 28: 676-677
  16. Torkamani N et al (2015) Disseminated superficial actinic porokeratosis accentuated by spray tan. Int J Dermatol 54:e545-546

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Last updated on: 08.06.2021