Elastolysis mediodermal L90.9

Rare, etiologically unexplained skin disease caused by an acquired loss of elastic fibrous material in the mid-dermis (mid-dermal). Clinically, the disease is characterized by the localized or disseminated appearance of soft, skin-colored crinkly plaques. The disease may also be characterized by perifollicular protrusions.

Sharply defined plaques with fine puckering as well as peau d'orange-like perifollicular protrusions on the trunk and proximal extremities, partly also discrete, laminar erythema.

The clinical picture can be divided into 3 different appearances:

• Type 1: circumscribed roundish or oval lesions with fine surface puckering
• Type 2: Perfollicular protrusions
• Type3: reticular erythema (rare: overlap with type1/type2 is possible)

Selective loss of elastic fibers in the mid dermis, partial lymphohistiocytic infiltrates, elastophagocytosis, and multinucleated giant cells with granuloma formation. Ultrastructurally, multinucleated giant cells were found with phagocytosed elastic material.

In medio-dermal elastolysis, in contrast to anetoderma, the loss of elastic fibers is limited to the middle dermis.

Immunohistologically, a reduced expression of fibulin 4 and fibulin 5 is found (proteins which are important in the processing of elastic fibers - see also Cutis laxa).

Anetoderma (see also the differential diagnostic explanations there).

Anular elastolytic giant cell granuloma

Cutis laxa

Striae cutis distensae

No effective therapy known. Sun protection is recommended.

A 45-year-old woman noticed circumscribed, unusual, wrinkled skin areas on the arms for 2 years. later, the wrinkling spread to the trunk, back, and proximal portions of the legs. Except for occasional pruritus, skin findings were asymptomatic, and there were no previous erythema.

Medical history was completely unremarkable; she had not traveled recently. The pateinten was athletic, very active outdoors, and often exercised without sunscreen. She was not taking any medications, including oral contraceptives, and had no known allergies.

Physical examination revealed extensive, slightly bulging, skin-colored, wrinkled papules and plaques. The changes were most apparent on the abdomen, trunk, and proximal arms and legs, whereas the face and hands were spared.

Histologically, rarefaction of the elastic fiber network was demonstrated. No inflammatory changes.

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