Variegated parakeratosis L41.81

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

atrophic parapsoriasis; large plaque parapsoriasis; Lichen variegatus; LPP; Paraliche (Brocq); parapsoriasis lichenoides; Parapsoriasis poikilodermatic; poicilodermal parapsoriasis; poikilodermia vascularis atrophicans (Jacobi); retiform parapsoriasis; variegated parapsoriasis

History
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Politzer, Santi and Unna, 1890; in 1902 Brocq assigned the disease to the parapsoriasis group.

Definition
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Today the name "Parakeratosis variegata" has only a historical meaning. This poikilodermatic clinical picture is assigned to the group of "large-heart parapsoriasis en plaques" (see below parapsoriasis en plaques large-heart poikilodermatic form). The special clinical aspect with the "striated or reticularly arranged, lichenoid and atropical, little scaly papules and plaques" does not justify the independence of the clinical picture. A more recent literature on this clinical picture is not available.

Occurrence/Epidemiology
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Very rare.

Etiopathogenesis
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Unsolved. Today, the clinical picture is usually regarded as a large-heart poicilodermatic variant of parapsoriasis en plaques or even as a separate entity (as an entity rather unlikely).

Manifestation
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Mostly occurring in middle age, more rarely also in adolescence or old age.

Localization
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In principle possible on all parts of the body except for the palms of the hands and soles of the feet. Especially on the trunk, abdomen, glutes and extremities close to the trunk.

Clinical features
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Anular, striated or reticularly arranged, lichenoid papules and plaques. These are aligned in places in the cleft lines of the skin. If net-like formations are formed, they appear to follow the deep vascular plexus of the skin (misinterpretation as livedo). Plaques confluent (random confluence patterns) to larger inhomogeneously patterned areas in which two-dimensional elevations alternate with atrophy. Peripheral brownish spots (pigmentation) and telangiectasia are also found. If the capillitium is affected, focal or diffuse (scarring) alopecia can occur.

Histology
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  • Partly atrophic but slightly acanthotic epithelium mostly with compact orthokeratosis or orthohyperkeratosis. Only rarely there is spot-like parakeratosis. Bulky, diffuse, less pleomorphic lymphoid cell infiltrate in a band-shaped subepithelial arrangement. The upper dermis is loosened with numerous dilated vessels. Focal epidermotropy with low spongiosis of the epidermis. Isolated cytoid corpuscles are found. Pauterian microabscesses are possible. Pigment incontinence.
  • Electron microscopy: Lutzner cells in the epidermis.

Differential diagnosis
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Therapy
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In mild cases, bland-nursing therapy. In severe forms, try UVA irradiation, PUVA therapy or helioclimatic therapy. Six-monthly clinical and, if necessary, histological check-ups are important. If histological confirmation of a cutaneous T-cell lymphoma is available, correspondingly adapted therapeutic measures should be taken.

Progression/forecast
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Chronic course over years to decades. Transition to a cutaneous T-cell lymphoma in about 10% of cases (depending on literature 5-45%).

Case report(s)
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  • In the 65-year-old patient, red, asymptomatic spots appeared for the first time on both forearms 3 years ago, which slowly spread over the months to the trunk, especially the buttocks and breasts, and to parts of the extremities near the trunk. Pat. complained of moderate itching, especially in the evening. HV appeared more pronounced after a warm shower or bath.
  • Findings: Exercise dyspnea (condition after myocardial infarction and multiple stents) anemic (hypochromic microcytic anemia, HB 7.4 g/dl). Normothyroid goiter nodosa and well controlled type II diabetes. Normal leukocyte values, no lymphocytosis, leukocyte differentiation o.B. Sonography: o.B.; no subcutaneous lymph node enlargement.
  • Skin status: Anular, partly also striated or reticularly arranged, lichenoid red papules and up to 8 cm in the diameter of the plaques. These are locally aligned in the cleft lines of the skin. If net-like formations are formed, they seem to follow the deep vascular plexus of the skin (misinterpretation as livedo). Plaques confluent (random confluence patterns) to larger inhomogeneously patterned areas in which two-dimensional elevations alternate with atrophy. At the buttocks 6 cm, large, flat ulcer embedded in lesional skin.
  • Histology: See above findings. Immunohistology: CD3+. CD4+, MiB 15% reactive. No evidence of clonal rearrangement of T cell receptor genes.
  • Therapy: Iron substitution, continuation of antidiabetic treatment. Local: Blande nursing therapy. PUVA bath therapy, 4 times/week 0.2-0.3 J/cm2, with each 3rd therapy unit increase of the dose by 0.2 J/cm2); including after 14 days significant improvement of the clinical findings, itching satisfactorily reduced. Ambulant continuation of PUVA therapy (3 times/week) until further notice.
  • Further procedure: Clinical and possibly histological control examinations are necessary. Repetition of the T-cell receptor gene rearrangement. Otherwise symptomatic (follow-up) non-aggressive treatment.

Literature
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  1. Holubar K (2003) Psoriasis and parapsoriasis: since 200 and 100 years, respectively. J Eur Acad Dermatol Venereol 17: 126-127
  2. Karenko L et al (2003) Chromosomal abnormalities in relation to clinical disease in patients with cutaneous T-cell lymphoma: a 5-year follow-up study. Br J Dermatol 148: 55-64
  3. Popp C et al (1992) Reflections on the pathogenesis of parakeratosis variegata based on morphological and molecular genetic findings. Dermatologist 43: 634-639
  4. Wolf IH et al (2009) Parapsoriasis lichenoides/parapsoriasis variegata--a new concept. J Dtsch Dermatol Ges 7:993-995

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Last updated on: 29.10.2020