28 SKIN DISORDERS JUNE 15, 2009 • FAMILY PRACTICE NEWS Use Skin Changes to Identify Scleroderma Mimics

BY PATRICE WENDLING upper half remains edematous and has a distinguished from systemic sclerosis by much greater circumference than does the absence of Raynaud’s phenomenon, C HICAGO — Few physicians would be the lower sclerotic portion. autoantibodies, and visceral involvement, fooled nowadays by gadolinium-induced Lipodermatosclerosis is sign of severe and—unlike systemic sclerosis—it re- nephrogenic systemic fibrosis, but there end-stage venous insufficiency, and sponds to corticosteroids, Dr. Steen said. are other diseases that can masquerade should be differentiated from scleroder- Histologically, there are marked as scleroderma. ma, cellulitis, superficial throm- eosinophilia and inflammatory infiltrates The precise diagnosis of scleroderma- bophlebitis, and erythema nodosum. The in the fascia. The extent of the histolog- like illnesses is important because even diagnosis is made from clinical observa- ic changes depends on the stage of the though many of them are called sclero- tion, but direct immunofluorescence of disease, and thus is not a consistent com- derma, they are different from systemic early and late lesions has been used to ponent of the disease. Aside from sclerosis in their treatments and out- show dermal pericapillary fibrin deposits. marked peripheral eosinophilia, other comes, Dr. Virginia Steen said at a sym- If left untreated, lipodermatosclerosis laboratory features to watch for include posium sponsored by the American Col- can progress to ulceration, atrophy REER an increased erythrocyte sedimentation lege of Rheumatology. blanche, or shortening of the Achilles E. G rate, increased gamma globulin, and an The diagnosis is most often based on tendon. Treatment involves weight loss, increased aldolase, with a normal creati- the distribution and clinical characteris- controlling the underlying disease, and ENNETH nine phosphokinase. . K R

tics of skin findings, as biopsies don’t al- emphasis on support stockings that may D Aggressive physical therapy is a key ways differentiate types of scleroderma. need to be specially made, said Dr. component of treatment, because this She recommended watching for the fol- Steen, who is professor of medicine and most scleroderma mimics discussed OURTESY lowing four conditions: and director of the rheumatology fel- C here can cause joint contractures. In Ǡ Lipodermatosclerosis is one condi- lowship program at Georgetown Uni- The sclerotic plaque on this patient’s eosinophilic fasciitis, low- to moderate- tion that physicians often fail to think of versity in Washington. lower leg is lipodermatosclerosis. dose prednisone (20-30 mg)—and, if as a scleroderma mimic. Also known as Topical steroids are useful if the skin needed, methotrexate as a steroid-spar- hypodermatitis sclerodermaformis, it is inflamed, and antibiotics are recom- is commonly associated with diabetes, ing agent—can be given, Dr. Steen said. refers to localized chronic inflammation mended for cellulitis. it can also occur after a viral illness. Ǡ Diabetic cheiroarthropathy is a syn- and fibrosis of the skin and subcuta- Ǡ Scleredema tends to target the upper Ǡ Eosinophilic fasciitis is a rare disor- drome of limited joint mobility in the neous tissues of the lower leg. In the body without affecting the lower ex- der characterized by symmetrical and hands. It is characterized by thickened, acute stage, the leg is inflamed and tremities. The skin on the neck and painful inflammation and swelling of tight, waxy hands with sclerosis of the warm, the skin is very tight, and celluli- face thicken and harden; severely af- the extremities, leading to induration palmar tendon sheaths that noticeably re- tis may be present. The ankle and toes fected patients are unable to wrinkle and the characteristic peau d’orange stricts mobility in the proximal inter- are not involved. their foreheads or open their mouths. In configuration. The palms may be in- phalangeal joints and metacarpopha- In its chronic stage, there is induration, most patients, the shawl sign is present, volved, but typically the fingers and toes langeal joints. contraction of the skin and subcuta- with skin involvement over the chest are spared. Contractures demonstrating The “prayer sign,” in which the patient neous tissues, and irregular depressions and arms, she said. the groove sign commonly evolve as a will be unable to fully oppose the palmar that can look almost identical to lower- Pathologic features include swollen result of induration. surfaces of the digits, is a clue to this di- leg scleroderma, she said. collagen with clear spaces and accumu- Eosinophilic fasciitis is slightly more agnosis when it is present in a patient with The leg eventually resembles an in- lation of hyaluronic acid and gly- common in middle-aged men, but can oc- diabetes, said Dr. Steen, who disclosed no verted champagne bottle, in which the cosaminoglycans. Although scleredema cur in women and children. It was initially relevant conflicts of interest. ■ Periocular Skin Cancers Tend Unexplained Erythema May To Be Basal Cell Carcinoma Be Associated With Cancer

BY ALICIA AULT carcinoma, trichoepithelioma, and der- BY CHARLES BANKHEAD with re-evaluation for malignancy even matofibrosarcoma protuberans. if the initial investigation had been AUSTIN, TEX. — A chart review aimed The investigators also quantified location P RAGUE — Unexplained erythema negative,” they said in a poster at the In- at quantifying the incidence and type of and pre- and postoperative defect sizes. should raise suspicion about a possible ternational Congress of Dermatology. periocular skin cancers showed that the Most often, BCCs were located on the low- underlying malignancy, according to a Few investigators have attempted to vast majority were basal cell carcinomas, er eyelid (246, or 57%). They were also review of cases as one Asian institution. examine the natural history and po- and that there was a slight predominance found on the medial canthus (28%), upper In an effort to determine the clinical tential clinical consequences of un- of the cancers in men. eyelid (10%), and lateral canthus (6%). implications of idiopathic erythema, explained erythema, the researchers The study was undertaken because there The squamous cell tumors also were Dr. Steven Thng and colleagues at the wrote. Moreover, previous studies pri- has been an increase in eyelid malignancies found most frequently on the lower eye- National Skin Center in Singapore re- marily involved white populations. that is thought to be due to a lack of pro- lid (64 or 61%), followed by the medial viewed the records of patients evalu- Most patients with idiopathic ery- tection from ultraviolet radiation, Dr. Jens canthus (17%), the upper eyelid (15%), and ated for erythema from 2001 to 2005 thema were men (73%) and idiopath- Thiele said at the annual meeting of the the lateral canthus (7%). Six of the 10 and compared those patients with pub- ic erythema tended to occur at an old- American College of Mohs Surgery. melanomas were also on the lower eyelid; lished cases series as well as with data er age (69 years) when compared with This is the largest U.S.-based study of pe- 8 of the tumors were in females. from the Singapore Cancer Registry. erythema of known cause (62 years). riocular cancers ever conducted, said Dr. For BCCs, the pre- and postoperative Dr. Thng and colleagues identified Patients with idiopathic erythema Thiele, a dermatologist in private practice sizes were smallest on the upper eyelids, 218 patients evaluated for erythema tended to experience a slow onset of in Birmingham, Ala. He and his colleagues while the largest tumors were found on during the study period—108 cases disease and an average duration of 22 reviewed charts at a single center from 553 the medial canthus. The mean number of (50%) were classified as idiopathic. days. They also experienced more consecutive Mohs surgery patients from Mohs layers needed for BCC clearance Among patients with an identified episodes of disease (average of 1.75 January 2005 to September 2008. ranged from 1.33 in the lateral canthus to cause of erythema, preexisting der- episodes), compared with patients All of the patients were white (Fitz- 1.42 in the medial canthus. matoses (30%) and drug reaction (15%) who had erythema of known cause patrick skin types I, II, and III). There SCCs had larger pre-op and postop sizes, were the most common diagnoses. (average of 1.32 episodes). were 346 men and 207 women. Interest- but the number of layers needed for clear- On follow-up, the researchers found When compared with age-stan- ingly, 61% of the tumors were in men. ance was lower. The mean number for idiopathic erythema was associated dardized cases, patients with idio- Of the tumors, there were 435 basal cell SCC clearance was 1.5 in the medial can- with visceral malignancy in 18% of pathic erythema had more than a carcinomas (BCCs), 105 squamous cell thus and 1.1 in the lateral canthus, Dr. patients and with cutaneous T-cell threefold greater risk of visceral ma- carcinomas (SCCs), 10 melanomas, and Thiele said. lymphoma in 5%. lignancy. Dr. Thng and colleagues re- one of each of the following: sebaceous Dr. Thiele reported no conflicts. ■ “We recommend close follow-up ported no disclosures. ■