Criteria for inclusion:

1. Precise identification of the contributing authors is necessary for the validation of the PMS-IMS (data will be treated personally by the staff of the On-line Archives of Rheumatology in accordance with the current Italian law and will therefore not be given to third parties or used for commercial purposes).
   Therefore the following Author's identification data must be submitted:

   first Author's name

   institution

   medical speciality

   academic position

   address

   phone & fax number

   e-mail

   Other Author's name

2. Histological confirmation of the clinical diagnosis.

3. Availability of a clinical picture or a pathology slide or a picture of a slide which will be e-mailed or scanned as high quality jpg images and sent by e-mail as an attachment to the report of a case. The clinical pictures should possibly be clear enough for a clinical or pathological re-evaluation.

4. Serum protein electrophoresis at baseline and serial determinations are required (also if negative for monoclonal gammopathy).

5. All the submitted cases will be evaluated by a scientific committee before being included in the study.

Case-report Submission Form

Epidemiologic and base-line clinical data:

sex
date of birth
approximate date of onset
approximate date of correct diagnosis
approximate date of starting treatment
duration of treatment and follow-up
date of last visit (specify if still in follow-up or lost to follow-up; if death specify date)

Evaluation of the cutaneous involvement before starting treatment

dp:	discrete papules		ue:	underlying edema
cp:	coalescent papules forming plaques		h:	hair loss
n:	nodules		e:	erythema
ui:	underlying induration		i:	itching

specify one or more letters to describe the clinical manifestations for each involved area

Evaluation of the cutaneous involvement
face	scalp
decreased oral aperture
neck: anterior surface	neck: posterior surface
thorax: anterior area	dorsal area
upper abdomen	lumbar area
peri-umbilical
lower abdomen	buttocks
shoulder
arms: extensor surface	arms: flessor surface
forearms: extensor surface	forearms: flessor surface
hands: dorsal surface	hands: palmar surface
sclerodactyly
thigh: extensor surface	thigh: flessor surface
leg: extensor surface	leg: flessor surface
feet: dorsal surface	feet: plantar surface

Histologic findings:

distribution of the areas mucin deposition and sclerosis within the dermis and the subcutaneous tissue
inflammatory infiltrate
eosinophils
mast cells
staining with Alcian blue, PAS, Toluidine blue

Direct immunofluorescence findings:

IgG / IgM / IgA / complement deposition in / outside areas of mucin deposition

Systemic / visceral involvement:

1) myositis: (specify proximal / truncal muscle involved)

muscle tenderness
muscle weakness
confirmed by muscle enzyme determination and/or EMG and/or muscle biopsy

2) neurologic involvement (of otherwise unexplained origin):

carpal tunnel syndrome
changes in the level of consciousness (clouded sensorium, disorganized thinking, hallucinations)
acute psychosis
depression
seizures
paresthesia and paralysis
dysarthria
transient ischemic attack-like manifestations
coma

3) cardiovascular involvement:

hypertension
coronary artery disease

4) rheumatologic involvement:

joint involvement (arthralgia, seronegative migratory arthritis, erosive arthropathy)

Evaluation of joint involvement:
specify a number (degree of tenderness) and a letter (degree of swelling) for each involved joint.

tenderness and/or painful motion
(0) none
(1) mild (positive response on questioning)
(2) moderate (spontaneous response elicited)
(3) severe (withdrawal by patient on examination)

swelling
(a) none
(b) mild (detectable synovial thickening without loss of bony contour; detectable by bulge sign)
(c) moderate (loss of distinctiveness of bony contours; palpable effusion but not tightly distending joint)
(d) severe (bulging synovial proliferation; tightly distending effusion)

other rheumatic manifestations:

Raynaud's phenomenon
hand stiffness
sicca syndrome
associated rheumatic diseases (dermato/polymyositis, systemic sclerosis)

5) renal involvement:

hypertension
acute glomerulo-nephritis (proteinuria, abnormal urinary sediment)
renal insufficiency
end-stage renal failure
scleroderma renal crisis

diagnostic examinations: ecographic, radiographic, angiographic, scintigraphic evaluation, renal biopsy

6) gastrointestinal involvement:

esophageal involvement: dysfagia and/or esophageal aperistalsis (confirmed by radiologic, scintigraphic, manometric studies)
hepatic involvement (confirmed by hepatic enzyme determination / echography / biopsy)
other signs of gastrointestinal involvement

7) pulmonary involvement:

dispnea
restrictive pulmonary defect (specify tests performed and results)
obstructive pulmonary defect (specify tests performed and results)

8) others:

multiple myeloma
amyloidosis
neoplastic disorders
endocrine abnormalities
corneal deposits
laringeal involvement

Laboratory evaluation:

total serum proteins, immunoelectrophoresis
monoclonal gammopathy (IgG or IgA class,   or k light chains)
Bence Jones
bone marrow examination
erythrocyte sedimentation rate
thyroid function (TSH, T3, T4)
hepatic enzymes (SGOT, SGPT, alkaline phosphatase)
muscle enzyme (CPK, LDH, aldolase)
rheumatoid factor
ANA

Treatment protocol:

melphalan (low-dose continuous / high dose pulsed)
steroids alone
steroids in combination (methotrexate, chlorambucyl, cyclophosphamide)
plasmapheresis (alone or with immune-suppressive therapy)
photopheresis
retinoids
IV-Ig
other treatment modalities

with details of treatment dose and duration, clinical response (of both cutaneous and extracutaneous manifestations and paraprotein level), adverse effects, and follow-up.