Three clinical patterns have been suggested based on case reviews: i) a rare, self-healing form with spontaneous resolution leaving areas of atrophy or anetoderma
; ii) a common persistent form in which lesions may never resolve; and iii) a very rare, progressive form with organ dysfunction and central nervous system involvement.
The differential diagnoses mainly include anetoderma
, mid-dermal elastolysis, focal dermal hypoplasia, lupus panniculitis, and localized scleroderma.
The clinical differential diagnosis includes aplasia cutis, anetoderma
and atrophoderma, and atrophic DFSP.
reviewed the medical records of 30 patients with anetoderma
. The results suggest that protruding type anetoderma
may represent a more advanced stage, and the metalloproteinase(MMP-) 2 and MMP-9 could be responsible for elastic fiber degradation in anetoderma
Connective tissue diseases: pseudoxanthoma elasticum, anetoderma
, and Ehlers--Danlos syndrome in pregnancy.
Atypical cutaneous manifestations, that is, clinical features not surely linked to LB, were 16 (2.27%), in details: 8 cases of morphoea, 2 Raynaud's phenomenon, 1 lichen sclerosus and atrophicus, 1 pityriasis lichenoides, 1 Gilbert's pityriasis rosea, 1 urticaria, 1 anetoderma
, and 1 erythema nodosum.
APA-associated cardiac valve disease APA-associated livedo reticularis APA-associated nephropathy APA-associated thrombocytopenia Other skin manifestions Ulcerations, pseudo-vasculitic lesions, digital gangrene, superficial phlebitis, malignant atrophic papulosis-like lesions, subungual splinter hemorrhages, and anetoderma
(a circumscribed area of loss of dermal elastic tissue) Other neurological manifestations Cognitive dysfunction, chorea, headache or migraine, multiple sclerosis, transverse myelopathy, and epilepsy Table 4.
One unusual physical consequence of underdeveloped skin is anetoderma
of prematurity, characterized by tissue paper-like depressed brown scars or outpouchings of skin.
Morphometric analysis of elastic skin fibers from patients with cutis laxa, anetoderma
, pseudoxanthoma elasticum and Buschke-Ollendorff and Williams Beuren syndromes.
The dermatologic conditions included livedo reticularis, skin ulcerations, pseudovasculitis lesions, superficial skin necrosis, digital gangrenes, superficial phlebitis, multiple subungual splinter hemorrhages, and anetoderma
No patient appeared with anetoderma
, but four developed it during follow-up.