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The term “puffy skin” is not infrequently noted at initial presentation of patients with systemic scleroderma. However, this is generally described as “non-pitting” oedema. The following case history challenges the latter widely held assumption, showing that its measurement is simple and may offer a more sensitive method of assessing response to treatment than the modified Rodnan skin score.1
A premenopausal computer analyst first presented in May 1998 with a two month history of finger stiffness. The next 10 months were spent investigating and treating the cause of her iron deficiency anaemia—gastric ectasia (watermelon stomach). The diagnosis of diffuse systemic scleroderma was made in March 1999. At this time she presented with persisting symptoms of skin stiffness, burning and pruritis, especially in the early morning, affecting the skin of her arms and legs, face, and upper chest. Clinical examination showed finger clawing, sclerodactyly, and scleroderma affecting her arms to the mid-upper arm, the face, neck, upper chest, thighs, and calves. Pitting oedema, noted in the forearms, upper arms, chest, and thighs, had the following characteristics: it occurred in areas of affected skin—typically it occurred in the advancing front of skin involvement, was slow to induce (of bees' wax consistency), and it affected non-dependent areas. Her skin was also erythematous in parts. Livedoid patterning over the knees was also noted. The rest of the clinical examination, including musculoskeletal cardiac, and respiratory systems, was unremarkable. A skin biopsy specimen from the upper right forearm and dorsum of the left fifth proximal phalanx showed dermal sclerosis and perivascular lymphocytic inflammation around superficial dermal vessels, consistent with scleroderma. Both skin biopsy sites healed with keloid scarring. Her antinuclear antibody titre was 1/640, speckled pattern, and extractable nuclear antigens were negative for RNP, antitopoisomerase, anticentromere, SSA, SSB, and Sm antibodies.
A simple bedside test (the skin pitting oedema time test or “SPOT” test) was devised to quantify the duration of skin pitting and see whether its measurement paralleled response to treatment. A small diameter coin was placed over an area of oedematous skin on the arm, the site being recorded for future reference. A sphygmomanometer cuff was placed over the coin and around the arm, inflated to 100 mm Hg for 60 seconds, and then released. The sphygmomanometer cuff and coin were removed, leaving the coin's impression in the skin. From this time (time 0) the impression was palpated every minute by two independent observers (patient and author) until it was no longer palpable. This time was noted and recorded as the skin oedema time. Both interobserver and intraobserver variation were assessed over three consecutive days (coefficient of variation 6.8% and 6.8% respectively). The patient's skin oedema time was compared with that from a similar area of skin on a control matched for age, sex, and menopausal status (24 minutes).
At the patient's request, treatment with clindamycin over two consecutive days was started on 3 May 1999, at which time the skin oedema time measured 40 minutes. Clindamycin was stopped owing to marked diarrhoea and abdominal tenderness, which settled over five days. The patient was readmitted for pulse methylprednisolone on 13 May, by which time her skin had become increasingly sensitive and pruritic.
Figure 1 outlines her treatment with pulse steroids and cyclophosphamide. Cyclosporin was added because of continuing disease activity. Before her first course of intravenous cyclophosphamide bilateral basal crepitations were noted. By 30 June 1999 skin hyperpigmentation and the development of mild dysphagia was noted. Concurrently, she also noted increased hair regrowth on the dorsum of her hands and lateral aspect of the calves. After her final course of cyclophosphamide her hands were no longer clawed into fixed flexion, the skin on the dorsum of the hands was again so supple that the dorsal hand veins were both visible, palpable and protruding from the skin surface; the forearm skin biopsy site was no longer keloid, though that on her finger was still slightly raised and palpable; her skin was much less irritable or pruritic. The most marked areas of skin improvement were those which were still oedematous at the start of treatment and where the skin was affected for the least time—upper arms, forearms, hand, thighs, and anterior chest. The area of skin least responsive to treatment was over the distal phalanges, which remained tethered and immobile.
This case is presented to demonstrate a number of features. The skin of early diffuse disease is often characterised by pitting oedema. The skin pitting oedema time is easily measured and its measurement is reproducible. Measured as a multigrade quantitative scale (units of one minute), it is more sensitive to change than the current four grade semiquantitative modified Rodnan skin score1. It is useful in determining responsiveness of the skin oedema to various disease modifying therapeutic regimens. Finally, finger clawing seen soon after scleroderma onset, if largely due to skin oedema, may be reversible using immunosuppressive treatment.
There are two potential limitations of the “skin pitting oedema time” test. The first relates to its duration, making it impractical to administer in an outpatient department. The second relates to the potential influence of already fibrotic skin on the skin oedema score measurement. The first limitation may be overcome by applying a less compressive force (for example, 50 mm Hg) for a shorter duration (for example, 30 seconds)—both of which independently appeared approximately to halve the skin oedema recovery time, having the patient and/or a relative complete the assessment before leaving the surgery, or devising a mechanical device to monitor skin oedema quantitatively. The role of excess collagen deposition in affecting skin oedema recovery time is uncertain.
The occurrence of pitting skin oedema in early scleroderma is a useful clinical observation, providing information about the underlying pathology and hence the speed of responsiveness of the disease to treatment. It also provides guidelines for the relative usefulness of anti-inflammatory/immunosuppressive drugs rather than the historical antifibrotic “gold standard”,d-penicillamine.