Poïkilodemy — Wikipedia

The PoealDermie is a skin syndrome associating an atrophy of the epidermis which can evolve towards sclerosis, a disorder of the coloring of the skin (dyschromia: skin too brown or depigmented) and dilatations of the surface vessels of the dermis (telangiectasia). The distribution of affected skin areas that can take the appearance of separate plates or a network ^{[ first ] , [ 2 ]} .

Poikilodermia can be acquired or congenital. Poikilodermia is constitutive of several diseases in combination with other symptoms.

Occurring from birth or very early, they associate atrophy, dyschromies and telangiectasies with or without bubbles.
Some have a photosensitivity (Bloom, Cockayne and Rothmund-Thomson) syndromes, or bubbles (Mendes Da Costa, Kindler and Weary syndromes), or Keratotic lesions (Zinsser-Cole-Engman and Weary syndromes )) ^{[ 2 ]} .

Rothmund poikilodermia [ modifier | Modifier and code ]

Dermatosis of genetic (genodermatosis) combining an early photosensitive poikilodermia, because occurring during the first or second year of life, reticulated erythema and sometimes palmoplantary hyperkeratosis. In the strict sense, it has neither bone anomalies nor cataract. Its transmission is autosomal recessive. A rigorous photoprotection is essential and long -term surveillance must be exercised because it is a syndrome predisposing to the occurrence of skin cancer. It was described in 1868 by a German ophthalmologist August von Rothmund (in) ^{[ 3 ]} .

Thomson poikilodermia [ modifier | Modifier and code ]

Still called Rothmund-Thomson syndrome, it has in addition to the previous bone anomalies and a bilateral cataract ^{[ 3 ]} .

Rothmund-Thomson syndrome [ modifier | Modifier and code ]

Assembly with the skin aspect of several young patients with Rothmund-Thomson syndrome. The child of image B clearly presents an atrophy of the skin of the face and a pigmentation disorder. There is a bone gap on the arm radiography at the lower end of the humerus.

It is a genodermatosis associating poikilodermia, small size, hair eyelashes and eyebrows scattered or absent, early cataract, skeletal anomalies, early aging and a predisposition to certain cancers ^{[ 3 ] , [ 4 ]} .

Generalized poikilodermia of petges-jacobi or scleropoikilodermia [ modifier | Modifier and code ]

This disease associates a poikilodermia has a pigmented edematous erythema, beginner in the face, which can reach almost all of the skin, often evolving in scleroderma. It was described in 1906 by Gabriel Petges, French dermatologist and in 1908 by E. Jacobi, German dermatologist ^{[ 5 ]} .

Weary acrokeratic poikilodermia [ modifier | Modifier and code ]

Here poikilodermia is associated with non -traumatic bubbles with hands and feet, Lichenoid and keratosic papules of the hands, feet, knees and elbows. Autosomal dominant transmission disease which distinguishes it from Kindler’s poikilodermia. It was described in 1971 by P. E. Weary, American dermatologist ^{[ 2 ]} .

Poïkiloderma de Kindler [ modifier | Modifier and code ]

Congenital poikilodermia transmitted to the autosomal recessive mode, it manifests itself from birth by diffuse bubbles then by the progressive appearance of mucous disorders: oral leukokeratosis, anal stenosis, esophageal or urethral ^{[ 6 ]} . The skin evolution takes place towards a generalized atrophy associated with a pseudosynechia of the fingers and a keratodermia punctuated by the plants of the feet. It was described in 1954 by Theresa Kindler, British dermatologist ^{[ 3 ]} .

Hereditary fibrotic poikilodermia with tendon contractures, myopathy and pulmonary fibrosis (Poiktmp) [ modifier | Modifier and code ]

This syndrome reported for the first time in 2006 in a South African family is linked to heterozygous changes in the FAM111B gene on chromosome 11q12. It also associates an early poikilodermia of childhood in areas exposed to the sun, tendon contractures of the lower limbs and the development of pulmonary fibrosis in the second decade of life with alteration of respiratory function, heat intolerance, a drop in sweating and hair disorders ^{[ 7 ] , [ 8 ] , [ 9 ]} .

Pigmentae pigment and creek’s neck poïkilodermia [ modifier | Modifier and code ]

Still called “” solar aging “, it is a rare dermatosis mixing erythema and pigmentation, mainly reaching the face and the lateral faces of the neck, combining telangiectasias and follicular hyperkeratosis. The differential diagnosis is difficult with an original pigmentation cosmetics, with the pigmentogenic lichen, even with the erythrosis interfolliculairis colli of leder which is much more frequent. It was described in 1923 by Achille Civatte, French dermatologist ^{[ ten ] , [ 11 ]} .