**Cutaneous Assessment Tool for Myositis**

The Cutaneous Assessment Tool (CAT) is designed to catalogue and grade
the severity of the cutaneous lesions found in the adult and juvenile
idiopathic inflammatory myopathies. A wide spectrum of both classical
and more obscure cutaneous lesions is listed with definitions, followed
by a list of descriptors which will be used to grade the severity of the
lesion. Grading is based on: (a) absence or presence of a given lesion;
(b) presence of a primary finding without secondary changes; and (c)
presence of a primary lesion associated with different degrees of
secondary changes. Some lesions are graded by absence or presence, with
severity depending upon the extent and/or site of the lesion. By
choosing from the list associated with a lesion, an observer can
describe the major characteristics of the lesion in a given patient.
Scores were assigned a priori by the investigators based on expert
opinion on the relative importance of individual lesions and their
degree of activity or damage.

To complete the questionnaire, assess each lesion by checking all items
that describe it. If the lesion is absent, indicate this. Every item
should be assessed. More than one item may be chosen for each lesion
(e.g. item 2 heliotrope rash may include edema and
hypo/hyperpigmentation with erythema, or either descriptor alone).
Complete the assessment in the order specified. After completing the
questionnaire, rate the activity and damage of the skin both by 10 cm
visual analogue scales and by 5 point Likert scales (page 9).

The CAT is organized to catalogue active lesions, and irreversible
lesions or cumulative damage. Activity is associated with underlying
inflammation or vasculopathy. Active lesions include those that are
characteristic, erythematous vasculopathic, hand changes and other
(panniculitis and alopecia). Persistent manifestations can be thought of
in two ways: a) persistent lesions that have a low level of continued
activity, yet demonstrate residual changes (such as poikiloderma
vasculare atrophicans or calcinosis) and b) irreversible lesions such as
scars. Damage constitutes lesions that persistent for at least 6 months
that are which result from prior active disease or therapy. Changes
associated with damage are often post inflammatory, cumulative, or
irreversible.

**[Index of Lesions]{.underline}**

  ----------------------------------- -----------------------------------
  **[ACTIVE CUTANEOUS                 
  MANIFESTATIONS]{.underline}**       

  **Characteristic Lesions:**         **Vasculopathic Lesions:**

  [1A. Gottron's papules or Gottron's [9A. Livedo
  sign]{.underline}                   reticularis]{.underline}

  [2A. Heliotrope rash]{.underline}   [10A. Ulceration]{.underline}

  **Erythematous Lesions:**           [11A. Mucous membrane
                                      lesions]{.underline}

  [3A. Malar or facial                [12A. Periungual capillary loop
  erythema]{.underline}               changes]{.underline}

  [4A. Linear extensor                **Hand Lesions:**
  erythema]{.underline}               

  [5A. "V" sign rash]{.underline}     [13A. Mechanic's hands]{.underline}

  [6A. "Shawl" sign rash]{.underline} [14A. Cuticular
                                      overgrowth]{.underline}

  [7A. Non-sun exposed                **Other Active Lesions:**
  erythema]{.underline}               

  [8A. Erythroderma]{.underline}      [15A. Subcutaneous
                                      edema]{.underline}

                                      [16A. Panniculitis]{.underline}

                                      [17A. Alopecia]{.underline}

  **[CUTANEOUS MANIFESTATIONS OF      
  DAMAGE]{.underline}**               

  [1D -- 7D. Atrophy or               
  dyspigmentation without blanchable  
  erythema in a distribution of the   
  stated lesion]{.underline}          

  [18D. Poikiloderma vasulare         
  atrophicans]{.underline}            

  [19D. Calcinosis]{.underline}       

  [20D. Lipoatrophy]{.underline}      

  [21D. Depressed scar]{.underline}   
  ----------------------------------- -----------------------------------

[Example of how to complete the CAT]{.underline}:

A patient has some Gottron's papules which are hyperpigmented with
blanching on compressing the lesions and many Gottron's papules which
are erythematous-violaceous with secondary changes. Each of these
descriptors would be checked on the form. All other descriptors for this
lesion, which are not present in this patient, would be left blank.

1\. [Gottron\'s papules OR Gottron's sign:]{.underline} erythematous to
violaceous papules and small plaques over the extensor surfaces of large
or interphalangeal joints.

\_\_\_\_ absent

\_X\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_X\_\_ erythematous-violaceous papules, or hyperpigmented papules in a
dark-skinned person

\_\_\_\_ erythematous-violaceous papules, or hyperpigmented papules in a
dark-skinned person, accompanied by secondary changes of scale,
crusting, or erosions

\_\_\_\_ erythematous-violaceous papules, or hyperpigmented papules in a
dark-skinned person, with vesicles, bullae, ulceration, or necrosis

Each of the 21 items would be similarly assessed.

**CUTANEOUS ASSESSMENT TOOL (CAT)**

[Instructions]{.underline}:

Assess each lesion by checking all descriptors that describe it. If the
lesion is absent, indicate this. Every item should be assessed. More
than one box may be chosen for each lesion. Complete the questionnaire
in the order specified. Also rate the global activity and damage of the
skin disease using the 10 cm visual analogue scales and the 5 point
Likert scales.

**ACTIVE CUTANEOUS MANIFESTATIONS: Lesions 1A -- 7A, 8A -- 17A**

**Characteristic lesions:**

1\. [Gottron\'s papules OR Gottron's sign:]{.underline} erythematous to
violaceous papules and small plaques over the extensor surfaces of large
or interphalangeal joints.

[1A. Activity:]{.underline}

\_\_\_\_ absent

\_\_\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_\_ erythematous-violaceous papules, or hyperpigmented papules in a
dark-skinned person

\_\_\_\_ erythematous-violaceous papules, or hyperpigmented papules in a
dark-skinned person, accompanied by secondary changes of scale,
crusting, or erosions

\_\_\_\_ erythematous-violaceous papules, or hyperpigmented papules in a
dark-skinned person, with vesicles, bullae, ulceration, or necrosis

[1D. Damage:]{.underline}

\_\_\_\_\_\_ absent

\_\_\_\_ atrophy (epidermal or dermal) with or without telangiectasia in
a distribution of Gottron's papules or Gottron's sign

\_\_\_\_ hypo/hyperpigmentation without blanchable erythema in a
distribution of Gottron's papules or Gottron's sign

2\. [Heliotrope rash:]{.underline} violaceous to erythematous
periorbital skin eruption, confined to the upper eyelids, with or
without edema.

2A. Activity:

\_\_\_\_ absent

\_\_\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_\_ faint erythema or faint violaceous rash, or faint
hyperpigmentation in a dark- skinned person

\_\_\_\_ intense erythema or intense violaceous rash, or intense
hyperpigmentation in a dark-skinned person

\_\_\_\_ edema

2D. Damage:

\_\_\_\_\_ absent

\_\_\_\_\_\_atrophy (epidermal or dermal) with or without telangiectasia
in a distribution of heliotrope rash

\_\_\_\_ hypo/hyperpigmentation without blanchable erythema in a
distribution of heliotrope rash

**[Erythema:]{.underline}** erythematous discrete and confluent macular
eruption.

**[Erythematous lesions are numbered 3-7:]{.underline}**

3\. [Malar OR facial erythema:]{.underline} erythema over the face which
may be isolated malar erythema, but may include more extensive erythema
including perioral temporal, ear and frontal regions.

[3A. Activity:]{.underline}

\_\_\_\_ absent

\_\_\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

[3D. Damage:]{.underline}

\_\_\_\_ absent

\_\_\_\_ atrophy (epidermal or dermal) with or without telangiectasia in
a distribution of malar or facial erythema

\_\_\_\_ hypo/hyperpigmentation without blanchable erythema in a
distribution of malar or facial erythema

4\. [Linear extensor erythema:]{.underline} erythema specifically
located over the extensor tendon sheaths of the hands, forearms, feet
and/or forelegs, which spares the skin over the extensor joint surfaces.

[4A. Activity:]{.underline}

\_\_\_\_ absent

\_\_\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

[4D. Damage:]{.underline}

\_\_\_\_ absent

\_\_\_\_ atrophy (epidermal or dermal) with or without telangiectasia in
a distribution of linear extensor erythema

\_\_\_\_ hypo/hyperpigmentation without blanchable erythema in a
distribution of linear extensor erythema

5\. [\"V\" sign rash:]{.underline} discrete confluent and erythematous
macular eruption over the lower anterior neck and upper anterior chest.

[5A. Activity:]{.underline}

\_\_\_\_ absent

\_\_\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae, or ulceration.

[5D. Damage:]{.underline}

\_\_\_\_ absent

\_\_\_\_ atrophy (epidermal or dermal) with or without telangiectasia in
a distribution of "V" sign rash

\_\_\_\_ hypo/hyperpigmentation without blanchable erythema in a
distribution of "V" sign rash

6\. [\"Shawl\" sign rash:]{.underline} discrete confluent and
erythematous macular eruption in a shawl distribution over the upper
back, posterior neck, and shoulders, sometimes extending to the lateral
arms.

[6A. Activity:]{.underline}

\_\_\_\_ absent

\_\_\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

[6D. Damage:]{.underline}

\_\_\_\_ absent

\_\_\_\_ atrophy (epidermal or dermal) with or without telangiectasia in
a distribution of "shawl" sign rash

\_\_\_\_ hypo/hyperpigmentation without blanchable erythema in a
distribution of "shawl" sign rash

7\. [Non-sun exposed erythema:]{.underline} erythema in areas not
exposed to sun i.e. usually covered by clothing or in protected areas
such as under the chin, flexural areas, palms, soles, trunk, groin, etc;
found in addition to or exclusive of erythema described in items 3-6.

[7A. Activity:]{.underline}

\_\_\_\_ absent

\_\_\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

[7D. Damage:]{.underline}

\_\_\_\_ absent

\_\_\_\_ atrophy (epidermal or dermal) with or without telangiectasia in
a distribution of non-sun exposed erythema

\_\_\_\_ hypo/hyperpigmentation without blanchable erythema in a
distribution of non-sun exposed erythema

8A. [Erythroderma:]{.underline} extensive areas of confluent erythema,
involving both sun exposed and non-sun exposed skin; can involve the
entire

\_\_\_\_ absent

\_\_\_\_ present

**Vasculopathic Lesions: Items 9 - 12**

9A. [Livedo reticularis:]{.underline} a fixed peripheral vascular
condition characterized by a bluish-reddish netlike mottling of the skin
that is found on the trunk or the extremities. The discoloration
persists after the skin has been warmed.

\_\_\_\_ absent

\_\_\_\_ present, occupying 1-10% body surface area

\_\_\_\_ present, occupying 10-25% body surface area

\_\_\_\_ present, occupying \> 25% body surface area

10A. [Ulceration]{.underline}: injury to dermis, subcutaneous or deeper
soft tissue, secondary to vascular insufficiency, trauma, infection, or
unknown etiology. This category is for isolated ulceration. Other types
of ulceration are in other categories: i.e., Gottron\'s ulceration (#1),
ulceration with erythema (#3-6), mucous membrane ulceration (#12),
ulceration with panniculitis (#18), and ulceration with calcinosis
(#21).

\_\_\_\_ absent

\_\_\_\_ ulcer(s) with depth to dermis/subcutis (fat)

\_\_\_\_ ulcer(s) with depth to muscle, tendon, or bone

11A. [Mucous membrane lesions]{.underline}: macules, dilated gingival
capillaries, erosions/apthae, or ulceration (injury to submucosa or
deeper) involving oral, nasal, gingival, or genital regions.

\_\_\_\_ absent

\_\_\_\_ macular lesions

\_\_\_\_ gingival erythema and/or swelling involving dilated gingival
capillaries

\_\_\_\_ erosions/aphthae

\_\_\_\_ ulceration

12A. [Periungual capillary loop changes:]{.underline} dilatation of
capillaries which may be accompanied by vessel dropout.

\_\_\_\_ absent

\_\_\_\_ periungual erythema

\_\_\_\_ capillary loop changes present: detected only by otoscopy using
additional magnification aids (oil or water)

\_\_\_\_ capillary loop changes present: visible to naked eye

**Hand changes: Items 13-14**

13A. [Mechanic\'s hands:]{.underline} lesions on the palmar or lateral
aspects of the digits which can include fissuring, cracking,
hyperkeratosis, scaling, hyperpigmentation.

\_\_\_\_ absent

\_\_\_\_ superficial scale, dryness, or hyperpigmentation only

\_\_\_\_ cracking and fissuring without bleeding

\_\_\_\_ cracking and fissuring with bleeding

14A. [Cuticular overgrowth:]{.underline} enlargement and overgrowth of
the cuticle onto the nailbed.

\_\_\_\_ absent

\_\_\_\_ cuticular overgrowth present in 1 - 4 digits

\_\_\_\_ cuticular overgrowth present in ≥ 5 digits

**Other:**

15A. [Subcutaneous edema:]{.underline} swelling of skin and soft tissue
which may be localized or generalized.

\_\_\_\_ absent

\_\_\_\_ present, occupying 1-10% body surface area

\_\_\_\_ present, occupying 10-25% body surface area

\_\_\_\_ present, occupying \> 25% body surface area

16A. [Panniculitis:]{.underline} painful erythematous or violaceous
subcutaneous nodules or depressions due to inflammation in the
subcutaneous fat (biopsy documentation preferable). These lesions may
ulcerate.

\_\_\_\_ absent

\_\_\_\_ tender nodules or plaques

\_\_\_\_ tender nodules or plaques with ulceration

17A. [Alopecia: hair lo]{.underline}ss.

Diffuse: Nonscarring, non-erythematous widespread alopecia (telogen
effluvium)

Focal: Focal patchy alopecia with erythema (localized to areas of
inflammation)

\_\_\_\_ absent

\_\_\_\_ diffuse

\_\_\_\_ focal/patchy

Other active manifestation:
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**CUTANEOUS MANIFESTATIONS of damage: Lesions 1 D-- 7D, 18D -- 21D.**

Atrophy or dyspigmentation without blanchable erythema in a distribution
of lesions 1D -- 7D (see pages 3 - 6)

18D. [Poikiloderma vasculare atrophicans:]{.underline} a fine speckled
pattern of hyperpigmentated and hypopigmentated macules interspersed
with fine telangiectasia and cutaneous atrophy. Usually occurs in areas
of photosensitivity eruption. May arise de novo.

\_\_\_\_ absent

\_\_\_\_ poikiloderma occupying 1-10% body surface area

\_\_\_\_ poikiloderma occupying 10-25% body surface area

\_\_\_\_ poikiloderma occupying \> 25% body surface area

19D. [Calcinosis:]{.underline} dystrophic calcification in the
subcutaneous tissue, muscle, interfascial planes, or across joints

\_\_\_\_ absent

\_\_\_\_ superficial plaques or nodules

\_\_\_\_ calcinosis circumscripta (large tumerous deposits) or
calcinosis universalis (intermuscular fascial plane deposition)

\_\_\_\_ extensive subcutaneous exoskeleton or calcinosis with
ulceration

20D. [Lipoatrophy:]{.underline} loss of subcutaneous fat which may be
localized or more widely distributed and accompanied by hyperlipidemia.

\_\_\_\_ absent

\_\_\_\_ localized: focal areas of subcutaneous fat loss

\_\_\_\_ partial: involving face, upper limbs and shoulder, possibly
accompanied by complement abnormalities

\_\_\_\_ total: widespread accompanied by some of the following:
hepatomegaly, hirsutism, acanthosis nigricans, hyperlipidemia,
hyperinsulinemia.

21D. [Depressed scar \> 0.5 cm:]{.underline} end stage of lesions due to
vascular occlusion or vascular insufficiency manifested as depressions
due to atrophy or scarring. May be the end stage of ulceration, purpura,
vascular insufficiency seen in Raynaud\'s, panniculitis, or infection
healing with atrophy, depression and scarring (this may include scarring
alopecia).

\_\_\_\_ absent

\_\_\_\_ present

Other manifestation of damage:
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

After completing the assessment tool (items 1-21), rate the activity and
damage of the skin disease on the following 10 cm visual analogue scales
and 5 point Likert scales

+---+--------------------------------------------------------------------+
| # | **(Absent) (Maximum)**                                             |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
|   |                                                                    |
+---+--------------------------------------------------------------------+

no evidence of extreme skin

Skin disease activity disease activity

\_\_\_\_ 0 = no evidence of skin disease activity

\_\_\_\_ 1 = mild skin disease activity

\_\_\_\_ 2 = moderate skin disease activity

\_\_\_\_ 3 = severe skin disease activity

\_\_\_\_ 4 = extremely active skin disease

+---+--------------------------------------------------------------------+
| # | **(Absent) (Maximum)**                                             |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
| # |                                                                    |
|   |                                                                    |
+---+--------------------------------------------------------------------+

no evidence of extreme skin

skin disease damage disease damage

\_\_\_\_ 0 = no evidence of skin disease damage

\_\_\_\_ 1 = mild skin disease damage

\_\_\_\_ 2 = moderate skin disease damage

\_\_\_\_ 3 = severe skin disease damage

\_\_\_\_ 4 = extreme skin disease damage

**CUTANEOUS ASSESSMENT TOOL (CAT) (SCORING)**

[For each lesion, check all boxes that apply. (more than one box may be
marked per lesion)]{.underline}

**ACTIVE CUTANEOUS LESIONS: Lesions 1A -- 7A, 8A -- 17A.**

**[Characteristic lesions:]{.underline}**

1\. [Gottron\'s papules OR Gottron's sign:]{.underline} erythematous to
violaceous papules and small plaques over the extensor surfaces of large
or interphalangeal joints.

[1A. Activity::]{.underline}

\_\_\_0\_ absent

\_\_\_1\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_\_2\_ erythematous-violaceous papules, or hyperpigmented papules in
a dark-skinned person

\_\_\_3\_ erythematous-violaceous papules, or hyperpigmented papules in
a dark-skinned person, accompanied by secondary changes of scale,
crusting, or erosions

\_\_\_4 erythematous-violaceous papules, or hyperpigmented papules in a
dark-skinned person, with vesicles, bullae, ulceration, or necrosis

[1D. Damage:]{.underline}

\_ 0\_\_\_\_\_ absent

\_0 for Activity (A), 1 for Damage (D)\_\_\_ atrophy (epidermal or
dermal) with or without telangiectasia in a distribution of Gottron's
papules or Gottron's sign

\_0 for A, 1 for D\_\_\_ hypo/hyperpigmentation without blanchable
erythema in a distribution of Gottron's papules or Gottron's sign

2\. [Heliotrope rash:]{.underline} violaceous to erythematous
periorbital skin eruption, confined to the upper eyelids, with or
without edema.

2A. Activity:

\_\_0\_\_ absent

\_\_1\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_2\_\_ faint erythema or faint violaceous rash, or faint
hyperpigmentation in a dark- skinned person

\_\_3\_\_ intense erythema or intense violaceous rash, or intense
hyperpigmentation in a dark-skinned person

\_\_1\_\_ edema

2D. Damage:

\_\_0\_\_\_ absent

\_\_0 for A, 1 for D\_\_\_\_atrophy (epidermal or dermal) with or
without telangiectasia in a distribution of heliotrope rash

\_\_0 for A, 1 for D\_\_ hypo/hyperpigmentation without blanchable
erythema in a distribution of heliotrope rash

[Erythema:]{.underline} erythematous discrete and confluent macular
eruption**.**

**[Erythematous lesions:]{.underline}**

3\. [Malar OR facial erythema:]{.underline} erythema over the face which
may be isolated malar erythema, but may include more extensive erythema
including perioral, temporal, ear and frontal regions.

3A. Activity:

\_\_0\_\_ absent

\_\_1\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_2\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_3\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_4\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

3D. Damage:

\_\_0\_\_ absent

\_\_0 for A, 1 for D\_\_ atrophy (epidermal or dermal) with or without
telangiectasia in a distribution of malar or facial erythema

\_0 for A, 1 for D\_\_\_ hypo/hyperpigmentation without blanchable
erythema in a distribution of malar

4\. [Linear extensor erythema:]{.underline} erythema specifically
located over the extensor tendon sheaths of the hands, forearms, feet
and/or forelegs, which spares the skin over the extensor joint surfaces.

4A. Activity:

\_\_0\_\_ absent

\_\_1\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_2\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_3\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_4\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

4D. Damage:

\_\_0\_\_ absent

\_\_0 for A, 1 for D\_\_ atrophy (epidermal or dermal) with or without
telangiectasia in a distribution of linear extensor erythema

\_\_0 for A, 1 for D\_\_ hypo/hyperpigmentation without blanchable
erythema in a distribution of linear extensor erythema

5\. [\"V\" sign rash:]{.underline} discrete confluent and erythematous
macular eruption over the lower anterior neck and upper anterior chest.

5A. Activity:

\_\_0\_\_ absent

\_\_1\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_2\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_3\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_4\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae, or ulceration.

5D. Damage:

\_\_0\_\_ absent

\_\_0 for A, 1 for D\_\_ atrophy (epidermal or dermal) with or without
telangiectasia in a distribution of "V" sign rash

\_\_0 for A, 1 for D\_\_ hypo/hyperpigmentation without blanchable
erythema in a distribution of "V" sign rash

6\. [\"Shawl\" sign rash:]{.underline} discrete confluent and
erythematous macular eruption in a shawl distribution over the upper
back, posterior neck, and shoulders, sometimes extending to the lateral
arms.

6A. Activity:

\_\_0\_\_ absent

\_\_1\_\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_2\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_3\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_4\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

6D. Damage:

\_\_0\_\_ absent

\_\_0 for A, 1 for D\_\_ atrophy (epidermal or dermal) with or without
telangiectasia in a distribution of "shawl" sign rash

\_\_0 for A, 1 for D\_\_ hypo/hyperpigmentation without blanchable
erythema in a distribution of "shawl" sign rash

7\. [Non-sun exposed erythema:]{.underline} erythema in areas not
exposed to sun i.e. usually covered by clothing or in protected areas
such as under the chin, flexural areas, palms, soles, trunk, groin, etc;
found in addition to or exclusive of erythema described in items 3-6.

7A. Activity:

\_\_0\_\_ absent

\_\_1\_ hypo/hyperpigmentation with erythema (blanching occurs on
compression of the lesion and/or erythema is distinguishable at the
advancing border): resolution stage

\_\_2\_\_ erythema, or hyperpigmentation in a dark-skinned person,
without secondary changes (no scale, crust, erosions)

\_\_3\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by secondary changes of scale, crusting or erosions.

\_\_4\_\_ erythema, or hyperpigmentation in a dark-skinned person,
accompanied by vesicles, bullae or ulceration.

7D. Damage:

\_\_0\_\_ absent

\_\_0 for A, 1 for D\_\_ atrophy (epidermal or dermal) with or without
telangiectasia in a distribution of non-sun exposed erythema

\_\_0 for A, 1 for D\_\_ hypo/hyperpigmentation without blanchable
erythema in a distribution of non-sun exposed erythema

8A. [Erythroderma:]{.underline} extensive areas of confluent erythema,
involving both sun exposed and non-sun exposed skin; can involve the
entire

\_\_0\_\_ absent

\_\_8\_\_ present

**Vasculopathic Lesions:**

9A. [Livedo reticularis:]{.underline} a fixed peripheral vascular
condition characterized by a bluish-reddish netlike mottling of the skin
that is found on the trunk or the extremities. The discoloration
persists after the skin has been warmed.

\_\_0\_\_ absent

\_\_2\_\_ present, occupying 1-10% body surface area

\_\_3\_\_ present, occupying 10-25% body surface area

\_\_4\_\_ present, occupying \> 25% body surface area

10A. [Ulceration]{.underline}: injury to dermis or deeper, secondary to
vascular insufficiency, trauma, infection, or unknown etiology. This
category is for isolated ulceration. Other types of ulceration are in
other categories: i.e., Gottron\'s ulceration (#1), ulceration with
erythema (#3-6), mucous membrane ulceration (#12), ulceration with
panniculitis (#18), and ulceration with calcinosis (#21).

\_\_0\_\_ absent

\_\_15\_ ulcer(s) with depth to dermis/subcutis (fat)

\_\_30\_ ulcer(s) with depth to muscle, tendon, or bone

11A. [Mucous membrane lesions: m]{.underline}acules,dilated gingival
capillaries, erosions/apthae, or ulceration (injury to submucosa or
deeper) involving oral, nasal, gingival, or genital regions.

\_\_0\_\_ absent

\_\_1\_\_ macular lesions

\_\_2\_\_ gingival erythema and/or swelling involving dilated gingival
capillaries

\_\_3\_\_ erosions/aphthae

\_\_4\_\_ ulceration

12A. [Periungual capillary loop changes:]{.underline} dilatation of
beginning, middle and end of capillaries which may be accompanied by
vessel dropout.

\_\_0\_\_ absent

\_\_+1\_ periungual erythema

\_\_2\_\_ capillary loop changes present: detected only by otoscopy
using additional magnification aids (oil or water)

\_\_3\_\_ capillary loop changes present: visible to naked eye

**Hand Lesions:**

13A. [Mechanic\'s hands:]{.underline} lesions on the palmar or lateral
aspects of the digits which can include fissuring, cracking,
hyperkeratosis, scaling, hyperpigmentation.

\_\_0\_\_ absent

\_\_1\_\_ superficial scale, dryness, or hyperpigmentation only

\_\_2\_\_ cracking and fissuring without bleeding

\_\_3\_\_ cracking and fissuring with bleeding

14A. [Cuticular overgrowth:]{.underline} enlargement and overgrowth of
the cuticle onto the nailbed.

\_\_0\_\_ absent

\_\_1\_\_ cuticular overgrowth present in 1 - 4 digits

\_\_2\_\_ cuticular overgrowth present in ≥ 5 digits

**Other:**

15A. [Subcutaneous edema: swelling of skin and soft tissue which may
be]{.underline} localized or generalized.

\_\_0\_\_ absent

\_\_1\_\_ present, occupying 1-10% body surface area

\_\_2\_\_ present, occupying 10-25% body surface area

\_\_3\_\_ present, occupying \> 25% body surface area

16A. [Panniculitis:]{.underline} painful erythematous or violaceous
nodules or depressions which may or may not ulcerate, due to
inflammation in the subcutaneous fat (biopsy documentation preferable).

\_\_0\_\_ absent

\_\_4\_\_ tender nodules or plaques

\_\_8\_\_ tender nodules or plaques with ulceration

17A. [Alopecia: hair lo]{.underline}ss.

Diffuse: Nonscarring, non-erythematous widespread alopecia (telogen
effluvium)

Focal: Focal patchy alopecia with erythema (localized to areas of
inflammation)

\_\_0\_\_ absent

\_\_1\_\_ diffuse

\_\_2\_\_ focal/patchy

Other active manifestation: \_\_\_Not
scored\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**CUTANEOUS MANIFESTATIONS of damage: Lesions 1D -- 7D, 18D -- 21D**

Atrophy or dyspigmentation without blanchable erythema in a distribution
of lesions 1 -- 7 (see pages 11 - 13)

18D. [Poikiloderma vasculare atrophicans:]{.underline} a fine speckled
pattern of hyperpigmentated and hypopigmentated macules interspersed
with fine telangiectasia and cutaneous atrophy. Usually occurs in areas
of photosensitivity eruption. May arise de novo.

\_\_0\_\_ absent

\_\_1\_\_ poikiloderma occupying 1-10% body surface area

\_\_2\_\_ poikiloderma occupying 10-25% body surface area

\_\_3\_\_ poikiloderma occupying \> 25% body surface area

19D. [Calcinosis:]{.underline} dystrophic calcification in the
subcutaneous tissue, muscle, interfascial planes, or across joints

\_\_0\_\_ absent

\_\_+1\_ superficial plaques or nodules

\_\_+2\_ calcinosis circumscripta (large tumerous deposits) or
calcinosis universalis (intermuscular fascial plane deposition)

\_\_+3\_ extensive subcutaneous exoskeleton or calcinosis with
ulceration

20D. [Lipoatrophy:]{.underline} loss of subcutaneous fat which may be
localized or more widely distributed and accompanied by hyperlipidemia.

\_\_0\_\_ absent

\_\_1\_\_ localized: focal areas of subcutaneous fat loss

\_\_2\_\_ partial: involving face, upper limbs and shoulder, possibly
accompanied by complement abnormalities

\_\_3\_\_ total: widespread accompanied by some of the following:
hepatomegaly, hirsutism, acanthosis nigricans, hyperlipidemia,
hyperinsulinemia.

21D. [Depressed scar \> 0.5 cm:]{.underline} end stage of lesions due to
vascular occlusion or vascular insufficiency manifested as depressions
due to atrophy or scarring. May be the end stage of ulceration, purpura,
vascular insufficiency seen in Raynaud\'s, panniculitis, or infection
healing with atrophy, depression and scarring (this may include scarring
alopecia).

\_\_0\_\_ absent

\_\_1\_\_ present

Other chronic manifestation: \_\_Not
scored\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_