TABLE 1.
Features of conditions in the clinical and/or pathological differential diagnosis of mid-dermal elastolysisa
| CONDITION | ANETODERMA | ANNULAR ELASTOLYTIC GIANT CELL GRANULOMA | CUTIS LAXA (ACQUIRED AND INHERITED) | MID-DERMAL ELASTOSIS | PSEUDOXANTHOMA ELASTICUM-LIKE PAPILLARY DERMAL ELASTOLYSIS |
|---|---|---|---|---|---|
| Clinical Features | |||||
| AGE AND/OR GENDER | Adults or children | Middle-aged women | Adults or children | Young women | Older women |
| POSSIBLY ASSOCIATED CONDITIONS | Presentb | Absent | Presentc | Presentd | Absent |
| PRECEDING INFLAMMATORY SKIN LESIONS | Presentb | Absent | Presentc | Presentd | Absent |
| MORPHOLOGY | Sac-like, circumscribed flaccid skin herniations | Red annular lesionse | Hanging skin folds and coarse wrinkles | Types I, II, and III Linear horizontal lumbar bandsf | Multiple yellow non- follicular papulesg |
| LOCATION | Trunk and proximal limbs | Sun-exposed areas (face) | Face | Trunk and proximal limbs Lumbar back | Neck, flexor forearms and axillae |
| Pathological Features | |||||
| DERMAL ELASTOLYSIS | |||||
| PAPILLARY | Present | h | Present | Absent | Presenti |
| RETICULAR | Present | h | Present | Presenti | Absent |
| DERMAL INFLAMMATION | Present/Absentj | Presentk | Present/Absentl | Present/Absentm | Absent |
| ELASTOPHAGO-CYTOSISn | Present | Present | Present | Present | Absent |
| REFERENCES | 13–16 | 17–21 | 22–25 | 1–12 | 26,27 |
Other conditions that might also be considered in either the clinical and/or pathological differential diagnosis of mid-dermal elastolysis include acrokeratoelastoidosis,24 amyloid elastolysis,28 fibroelastolytic papulosis of the neck,29 floppy eye syndrome and blepherochalasia,30 generalized elastolysis,31 granulomatous slack skin (lymphoma),32 intrinsic skin aging,33 nevus anelasticus,34 papular elastorrhexis,35 perifollicular elastolysis,36 upper dermal elastolysis,37 and white fibrous papulosis of the neck.38
Primary anetoderma has repeatedly been associated with antiphospholipid antibodies (with or without criteria of primary antiphospholipid syndrome); systemic diseases in some of these patients include autoimmune hemolysis, autoimmune thrombocytopenia, hypothyroidism, Grave’s disease, human immunodeficiency virus infection, mixed cryoglobulinemia, and systemic lupus erythematosus. Inflammatory skin lesions may precede those of anetoderma.
Cutis laxa may be inherited or acquired; the latter is often preceded by a cutaneous inflammatory condition (such as dermatitis, erythema multiforme, or urticaria) and may have associated internal organ involvement: cardiovascular, gastrointestinal, pulmonary, and/or urogenital. Marshall syndrome (postinflammatory elastolysis and cutis laxa) is a rare form of cutis laxa that has been observed in young children.
Mid-dermal elastolysis has been associated with autoimmune phenomena and autoimmune diseases (false-positive serology for Borrelia burgdorferi, elevated antinuclear antibodies and circulating immunocomplexes, Hashimoto’s thyroiditis, Grave’s disease, rheumatoid arthritis, and lupus erythematosus), dermatoses (atopic dermatitis, granuloma annulare, phototoxic dermatitis, pityriasis rosea, Sweet syndrome, and urticaria), genetic disorders (Keutel syndrome) and miscellaneous conditions (asthma, chronic hemodialysis, human immunodeficiency virus infection, mycosis fungoides, protein S deficiency, silicone mammoplasty, uterine carcinoma). In type I as well as type II, mid-dermal elastolysis preceding inflammatory skin lesions may occur.
The lesions are erythematous and annular; the borders are usually raised and red with the central portion of the lesion being atrophic and wrinkled in appearance;
In addition to our patient with linear, raised, indurated, flesh-colored horizontal bands that partially or completely extended across her lumbar back, mid-dermal elatolysis may present as plaques of fine wrinkling arranged parallel to skin cleavage lines (type I lesions), perifollicular popular protrusions with “peau d’orange” appearance (type II lesions), or persistent reticular erythema usually on the upper trunk (type III lesions).
The lesions resemble the “cobblestone” appearance of pseudoxanthoma elasticum.
There is paucity or absence of the elastic fibers in the center of the lesion.
There is a band-like loss of the elastic fibers.
In clinically inflammatory lesions, a perivascular inflammatory infiltrate may be present.
There is a granulomatous inflammatory infiltrate in the dermis consisting of histiocytes, lymphocytes, and giant cells.
Inflammatory infiltrates of neutrophils or lymphocytes may be present.
Inflammatory infiltrates of variable degree may be observed.
When present, elastophagocytosis may occur with (anetoderma, annular elastolytic giant cell granuloma and mid-dermal elastolysis) or without (cutis laxa) associated giant cells.