Patients with extragenital lichen sclerosis and atrophoderma were included in the analysis, but those with pansclerotic morphea and eosinophilic fasciitis
(EF) or Shulman syndrome, is a scleroderma-like syndrome with an early phase characterized by erythema and edema distributed over the limbs or trunk and a later phase, dominated by collagenous thickening of the subcutaneous fascia.
Generalized morphea is induration of skin starting as four or more individual plaques larger than 3 cm, that become confluent and involve at least two of the seven anatomical sites (head, neck, right upper extremity, left upper extremity, right lower extremity, left lower extremity, anterior trunk and posterior trunk).3,4 Within this group there are distinct clinical presentations which are: disseminated plaques morphea, pansclerotic morphea and eosinophilic fasciitis
Another disease considered in the differential diagnosis was eosinophilic fasciitis
. In eosinophilic fasciitis
, diffuse thickening and contrast involvement in the deep and superficial fascia are typical.
The common causes for raised eosinophil count are parasitic conditions, allergy/atopy, eczema, urticaria, allergic rhinitis, angioneurotic oedema, reactive eosinophilia subsequent to T-cell lymphoma, B-cell lymphoma, acute lymphoblastic leukemia, eosinophilic leukemia, idiopathic hypereosinophilic syndrome, allergic drug reactions and collagen vascular diseases such as rheumatoid arthritis, eosinophilic fasciitis
or allergic angiitis.
, also called Shulman syndrome, is a very rare, localized fibrosing disorder of the fascia.
Scleroderma, eosinophilic fasciitis
, and systemic lupus erythematosus were considered.
Paraneoplastic eosinophilic fasciitis
: a case report.
Pope: Systemic sclerosis can be confused with such sclerodermalike skin disorders as eosinophilic fasciitis
(EF), morphea, scleromyxedema, and nephrogenic systemic fibrosis (NSF).
APMA can be either idiopathic or caused by a variety of conditions, such as acquired clonal cytogenetic abnormalities, drug sensitivity, toxin exposure, infectious diseases such as viral infection (1), (2), immune diseases such as lupus erythematosus (3), systemic sclerosis (4), eosinophilic fasciitis
(5), and malignancy (6), (7).
* Eosinophilic fasciitis
is a rare disorder characterized by symmetrical and painful inflammation and swelling of the extremities, leading to induration and the characteristic peau d'orange configuration.
Eosinophilic pneumonia, eosinophilic fasciitis
, hypereosinophilic syndrome, and cutaneous eosinophilic vasculitis have been described in patients with RA.