Porokeratosis superficialis disseminata actinica Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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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 non-hereditary, acquired, disseminated form of porokeratosis in light-exposed areas. The single florescence is a punctually beginning, centrifugally progressive hyperkeratosis with development of a ring-shaped, parakeratotic cornea 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 laser (Co2 or Erbium-YAG laser)
  • vitamin D3 analogues
  • Fluorouracil Therapy
  • Photodynamic Therapy
  • In a smaller study (Schilling B 2018), good results were obtained with so-called boundary rays (long-wave, soft X-rays)
  • Imiquimod
  • Tretinoin
  • Light protection (e.g. Anthelios), see below light protection agent.

Progression/forecast
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Progression with increasing age, especially in the summer months. Development of actinic keratosis and carcinoma are possible.

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. Act Dermatol 14: 287-289
  2. Besenhard HM et al (1988) Disseminated superficial actinic porokeratosis (DSAP) with Bowen's disease. Dermatologist 39: 286-290
  3. Biswas A (2015) Cornoid lamellation revisited: apropos of porokeratosis with emphasis on unusual clinicopathological variants. On 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. Pediatric 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. Nayeemuddin FA et al (2002) Topical photodynamic therapy in disseminated superficial actinic porokeratosis. Clin Exp Dermatol 27: 703-706
  8. Schilling B (2018) Boundary radiation in refractory actinic porokeratosis: a well tolerated and effective therapy. Bare compass 6:32-33
  9. Schwarz T et al (1984) Disseminated superficial actinic porokeratosis. J Am Acad Dermatol 11: 724-730
  10. Sidwell RU et al (2003) Giant actinic porokeratosis of the scalp. Br J Dermatol 149: 654
  11. 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
  12. Theng CT et al (2004) Warty nodules on an annular plaque in a renal transplant recipient. Arch Dermatol 140: 121-126
  13. Thomas C et al (2003) Hypertrophic perianal porokeratosis in association with superficial actinic porokeratosis of the leg. Clin Exp Dermatol 28: 676-677
  14. Torkamani N et al (2015) Disseminated superficial actinic porokeratosis accentuated by spray tan. Int J Dermatol 54:e545-546

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020