Parapsoriasis en plaques large-hearth-poicilodermatic L41.5

Clinically and histologically well established variant of large-heart inflammatory parapsoriasis en plaques with development of a poikilodermatic-atrophic condition.

The clinical picture is probably identical with the parakeratosis variegata described by Unna, Santi and Politzer in 1890.

S.a. Poikilodermia vascularis atrophicans.

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

In principle possible on all parts of the body except for the palms of the hands and soles of the feet. However, especially on the trunk (mammae), abdomen, glutes and extremities close to the trunk.

Anular, striated or reticular patches, lichenoid papules and plaques. These are aligned in places in the cleft lines of the skin. When 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.

• 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.

In mild cases, bland-nursing therapy.

In severe forms of UVA irradiation, PUVA therapy or helioclimatic therapy.

Six-monthly clinical and, if necessary, histological check-ups are important.

In case of histological confirmation of a cutaneous T-cell lymphoma, correspondingly adapted therapy measures

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