Poikiloderma vasculare atrophicans pedges-clepat with prolonged benign presentation in association with lipomatosis: case report

Abstract

 Poikiloderma vasculare atrophicans (PVA) is a rare disease, the onset is typically between 40 and 60 years of age [1], with a slight male predilection. It often develops in association with various genodermatoses, and autoimmune diseases and is considered to be an early stage of Mycosis fungoides (MF) or poikilodermic MF. [2] PVA, previously called Parapsoriasis variegata, manifests clinically by hyper-, hypopigmented lesions, arranged in a reticular pattern, telangiectasias, and atrophy, accompanied by moderate itching, in some cases is non-pruritic. The color of the rash can vary from pale pink to dark brown. The histopathological examination reveals poikilodermic changes with the absence of Pautrier microabscesses and atypical lymphoid cells. The first-line treatment is narrow-band UVB phototherapy, topical corticosteroids. In resistant cases, systemic retinoids, and interferon alfa, are indicated. Lipomatosis- a disorder, characterized by the presence of benign, painless, encapsulated, mobile tumors located on the trunk, and limbs with a size of 1->10 cm. Histological examination of lipomas shows mature, normal-appearing adipocytes. Treatment: surgical excision. The study’s aim is the presentation of the clinical-evolutive features of Poikiloderma vasculare atrophicans. Materials and methods The clinical case study of PVA concerns a 62-year-old female patient with lesions on the trunk, upper limbs, and inguinal region, and a 35-year history of the disease. The clinical examination revealed multiple red-brown plaques, arranged in a reticular pattern with circumscribed areas of atrophy and multiple telangiectasias, predominantly in the pectoral and inguinal regions. On the back: vascular pattern with areas of hyper-, hypopigmentation, and small circumscribed atrophic islands. Multiple soft, mobile, painless nodules, varying between 6-8 cm in size, located on the flexor and extensor surface of the upper limbs were noted. The general and systemic examinations determined that the patient was obese and had no lymphadenopathy. Laboratory investigations showed relative lymphocytosis; total cholesterol, and LDL cholesterol - insignificant increase. Histologic features (2004): degeneration of basal cells, dense infiltrate of lymphoid cells, dilated capillaries, a few melanophages in the upper dermis, no Pautrier’s microabscess or atypical lymphoid cells. The histopathological examination (2024) - HE staining: thinned epidermis, blurred dermal papillae, at the level of the dermo-epidermal junction, keratinocytes with vacuolar degeneration are attested. In the upper layers of the dermis, moderately diffuse lymphohistiocytic infiltrate with signs of exocytosis and single melanophages. PAS+Alcian blue staining – basement membrane not thickened, absence of mucin at the level of the dermis. The histopathological changes correspond to the picture of atrophic Poikiloderma. Treatment included NB-UVB, and topical - high-potency corticosteroids with an improvement in skin texture and reduction of pruritus intensity. Conclusions Histopathological findings confirmed insignificant progression of PVA, considering the biopsy data taken 20 years ago. In the presented case, the early onset and stagnation of the skin pathological process during 35 years is associated with a good prognosis and denotes its benign evolution, although no specific treatments were performed during these years.