Persistent Panniculitis in Dermatomyositis

E16 I CUTIS® WWW.MDEDGE.COM/DERMATOLOGY

CASE LETTER

To the Editor:A 62-year-old woman with a history of dermatomyosi-tis (DM) presented to dermatology clinic for evaluation of multiple subcutaneous nodules. Two years prior to the current presentation, the patient was diagnosed by her primary care physician with DM based on clinical presentation. She initially developed body aches, muscle pain, and weakness of the upper extremities, specifi-cally around the shoulders, and later the lower extremi-ties, specifically around the thighs. The initial physical examination revealed pain with movement, tenderness to palpation, and proximal extremity weakness. The patient also noted a 50-lb weight loss. Over the next year, she noted dysphagia and developed multiple sub-cutaneous nodules on the right arm, chest, and left axilla. Subsequently, she developed a violaceous, hyperpig-mented, periorbital rash and erythema of the anterior chest. She did not experience hair loss, oral ulcers, pho-tosensitivity, or joint pain.

Laboratory testing in the months following the initial presentation revealed a creatine phosphokinase level of 436 U/L (reference range, 20–200 U/L), an erythro-cyte sedimentation rate of 60 mm/h (reference range, <31 mm/h), and an aldolase level of 10.4 U/L (reference range, 1.0–8.0 U/L). Lactate dehydrogenase and thyroid function tests were within normal limits. Antinuclear antibodies, anti–double-stranded DNA, anti-Smith anti-bodies, anti-ribonucleoprotein, anti–Jo-1 antibodies, and anti–smooth muscle antibodies all were negative. Total blood complement levels were elevated, but complement C3 and C4 were within normal limits. Imaging demon-strated normal chest radiographs, and a modified barium swallow confirmed swallowing dysfunction. A right quad-ricep muscle biopsy confirmed the diagnosis of DM. A malignancy work-up including mammography, colonos-copy, and computed tomography of the chest, abdomen, and pelvis was negative aside from nodular opacities in the chest. She was treated with prednisone (60 mg, 0.9 mg/kg) daily and methotrexate (15–20 mg) weekly for several months. While the treatment attenuated the rash and improved weakness, the nodules persisted, prompt-ing a referral to dermatology.

Physical examination at the dermatology clinic dem-onstrated the persistent subcutaneous nodules were indurated and bilaterally located on the arms, axillae, chest, abdomen, buttocks, and thighs with no pain or erythema (Figure). Laboratory tests demonstrated a nor-mal creatine phosphokinase level, elevated erythrocyte sedimentation rate (70 mm/h), and elevated aldolase level (9.3 U/L). Complement levels were elevated, though complement C3 and C4 remained within normal limits.

Persistent Panniculitis in DermatomyositisKayla M. Babbush, MD; Ranon E. Mann, MD; Amanda A. Dunec, MD; Jules B. Lipoff, MD

Drs. Babbush and Mann are from the Department of Medicine, Division of Dermatology, Albert Einstein College of Medicine, Bronx, New York. Dr. Dunec is from Dermatology Consultants of Short Hills, New Jersey. Dr. Lipoff is from the Department of Dermatology, University of Pennsylvania, Philadelphia.The authors report no conflict of interest.Correspondence: Jules B. Lipoff, MD, Department of Dermatology, University of Pennsylvania, Penn Medicine University City, 3737 Market St, Ste 1100, Philadelphia, PA 19104 ([email protected]).doi:10.12788/cutis.0308

PRACTICE POINTS• Clinical panniculitis is a rare subcutaneous manifesta-

tion of dermatomyositis (DM) that dermatologists must consider when evaluating patients with this condition.

• Panniculitis can precede, occur simultaneously with, or develop up to 5 years after onset of DM.

• Many patients suffer from treatment-resistant pannic-ulitis in DM, suggesting that therapeutic management of this condition may require long-term and more aggressive treatment modalities.

Copyright Cutis 2021. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher.

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PANNICULITIS IN DERMATOMYOSITIS

VOL. 108 NO. 1 I JULY 2021 E17WWW.MDEDGE.COM/DERMATOLOGY

Histopathology of nodules from the medial right upper arm and left thigh showed lobular panniculitis with fat necrosis, calcification, and interface changes. The patient was treated for several months with daily mycophenolate mofetil (1 g increased to 3 g) and daily hydroxychloro-quine (200 mg) without any effect on the nodules.

The histologic features of panniculitis in lupus and DM are similar and include multifocal hyalinization of the subcuticular fat and diffuse lobular infiltrates of mature lymphocytes without nuclear atypia.1 Though clinical pan-niculitis is a rare finding in DM, histologic panniculitis is a relatively common finding.2 Despite the similar histo-pathology of lupus and DM, the presence of typical DM clinical and laboratory features in our patient (body aches,

muscle pain, proximal weakness, cutaneous manifestations, elevated creatine phosphokinase, normal complement C3 and C4) made a diagnosis of DM more likely.

Clinical panniculitis is a rare subcutaneous manifesta-tion of DM with around 50 cases reported in the literature (Table). A PubMed search of articles indexed for MEDLINE was conducted using the terms dermatomyositis and panniculitis through July 2019. Additionally, a full-text review and search of references within these articles was used to identify all cases of patients presenting with panniculitis in the setting of DM. Exclusion criteria were cases in which another etiology was considered likely (infectious panniculitis and lupus panniculitis) as well as those without an English translation. We identified 43 cases; the average age of the patients was 39.6 years, and 36 (83.7%) of the cases were women. Patients typically presented with persistent, indurated, painful, erythematous, nodular lesions localized to the arms, abdomen, buttocks, and thighs.

While panniculitis has been reported preceding and concurrent with a diagnosis of DM, a number of cases described presentation as late as 5 years following onset of classic DM symptoms.12,13,31 In some cases (3/43 [7.0%]), panniculitis was the only cutaneous mani-festation of DM.15,33,36 However, it occurred more com-monly with other characteristic skin findings, such as heliotrope rash or Gottron sign. Some investigators have recommended that panniculitis be included as a diag-nostic feature of DM and that DM be considered in the differential diagnosis in isolated cases of panniculitis.25,33

Though it seems panniculitis in DM may corre-late with a better prognosis, we identified underlying malignancies in 3 cases. Malignancies associated with panniculitis in DM included ovarian adenocarcinoma, nasopharyngeal carcinoma, and parotid carcinoma, indi-cating that appropriate cancer screening still is critical in the diagnostic workup.2,11,22

A majority of the reported panniculitis cases in DM have responded to treatment with prednisone; how-ever, treatment with prednisone has been more recal-citrant in other cases. Reports of successful additional therapies include methotrexate, cyclosporine, azathio-prine, hydroxychloroquine, intravenous immunoglobu-lin, mepacrine, or a combination of these entities.19,22 In most cases, improvement of the panniculitis and other DM symptoms occurred simultaneously.25 It is notewor-thy that the muscular symptoms often resolved more rapidly than cutaneous manifestations.33 Few reported cases (6 including the current case) found a persis-tent panniculitis despite improvement and remission of the myositis.3,5,10,11,30

Our patient was treated with both prednisone and methotrexate for several months, leading to remission of muscular symptoms (along with return to baseline of cre-atine phosphokinase), yet the panniculitis did not improve. The subcutaneous nodules also did not respond to treat-ment with mycophenolate mofetil and hydroxychloroquine.

A and B, Indurated subcutaneous nodules on the right axilla and chest consistent with panniculitis.

B

A


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Recent immunohistochemical studies have suggested that panniculitic lesions show better outcomes with immunosuppressive therapy when compared with other DM-related skin lesions.40 However, this was not the case for our patient, who after months of immunosuppressive therapy showed complete resolution of the periorbital and chest rashes with persistence of multiple indurated subcutaneous nodules.

Our case adds to a number of reports of DM present-ing with panniculitis. Our patient fit the classic demo-graphic of previously reported cases, as she was an adult woman without evidence of underlying malignancy; however, our case remains an example of the therapeutic challenge that exists when encountering a persistent, treatment-resistant panniculitis despite resolution of all other features of DM.

REFERENCES 1. Wick MR. Panniculitis: a summary. Semin Diagn Pathol. 2017;34:261-272. 2. Girouard SD, Velez NF, Penson RT, et al. Panniculitis associated

with dermatomyositis and recurrent ovarian cancer. Arch Dermatol. 2012;148:740-744.

3. van Dongen HM, van Vugt RM, Stoof TJ. Extensive persistent panniculitis in the context of dermatomyositis. J Clin Rheumatol. 2020;26:E187-E188.

4. Choi YJ, Yoo WH. Panniculitis, a rare presentation of onset and exacer-bation of juvenile dermatomyositis: a case report and literature review. Arch Rheumatol. 2018;33:367-371.

5. Azevedo PO, Castellen NR, Salai AF, et al. Panniculitis associated with amyopathic dermatomyositis. An Bras Dermatol. 2018;93:119-121.

6. Agulló A, Hinds B, Larrea M, et al. Livedo racemosa, reticulated ulcer-ations, panniculitis and violaceous plaques in a 46-year-old woman. Indian Dermatol Online J. 2018;9:47-49.

7. Hattori Y, Matsuyama K, Takahashi T, et al. Anti-MDA5 antibody- positive dermatomyositis presenting with cellulitis-like erythema on the mandible as an initial symptom. Case Rep Dermatol. 2018; 10:110-114.

8. Hasegawa A, Shimomura Y, Kibune N, et al. Panniculitis as the initial manifestation of dermatomyositis with anti-MDA5 antibody. Clin Exp Dermatol. 2017;42:551-553.

9. Salman A, Kasapcopur O, Ergun T, et al. Panniculitis in juvenile dermatomyositis: report of a case and review of the published work. J Dermatol. 2016;43:951-953.

10. Carroll M, Mellick N, Wagner G. Dermatomyositis panniculitis: a case report. Australas J Dermatol. 2015;56:224‐226.

11. Chairatchaneeboon M, Kulthanan K, Manapajon A. Calcific panniculitis and nasopharyngeal cancer-associated adult-onset dermatomyositis: a case report and literature review. Springerplus. 2015;4:201.

12. Otero Rivas MM, Vicente Villa A, González Lara L, et al. Panniculitis in juvenile dermatomyositis. Clin Exp Dermatol. 2015;40:574-575.

13. Yanaba K, Tanito K, Hamaguchi Y, et al. Anti‐transcription intermediary factor‐1γ/α/β antibody‐positive dermatomyositis asso-ciated with multiple panniculitis lesions. Int J Rheum Dis. 2015; 20:1831-1834.

14. Pau-Charles I, Moreno PJ, Ortiz-Ibanez K, et al. Anti-MDA5 positive clinically amyopathic dermatomyositis presenting with severe cardio-myopathy. J Eur Acad Dermatol Venereol. 2014;28:1097-1102.

15. Lamb R, Digby S, Stewart W, et al. Cutaneous ulceration: more than skin deep? Clin Exp Dermatol. 2013;38:443-445.

16. Arias M, Hernández MI, Cunha LG, et al. Panniculitis in a patient with dermatomyositis. An Bras Dermatol. 2011;86:146-148.

17. Hemmi S, Kushida R, Nishimura H, et al. Magnetic resonance imaging diagnosis of panniculitis in dermatomyositis. Muscle Nerve. 2010;41:151-153.

18. Geddes MR, Sinnreich M, Chalk C. Minocycline-induced dermatomyo-sitis. Muscle Nerve. 2010;41:547-549.

19. Abdul‐Wahab A, Holden CA, Harland C, et al Calcific panniculitis in adult‐onset dermatomyositis. Clin Exp Dermatol. 2009;34:E854-E856.

20. Carneiro S, Alvim G, Resende P, et al. Dermatomyositis with panniculi-tis. Skinmed. 2007;6:46-47.

21. Carrera E, Lobrinus JA, Spertini O, et al. Dermatomyositis, lobarpan-niculitis and inflammatory myopathy with abundant macrophages. Neuromuscul Disord. 2006;16:468-471.

22. Lin JH, Chu CY, Lin RY. Panniculitis in adult onset dermatomyositis: report of two cases and review of the literature. Dermatol Sinica. 2006;24:194-200.

23. Chen GY, Liu MF, Lee JY, et al. Combination of massive mucinosis, der-matomyositis, pyoderma gangrenosum-like ulcer, bullae and fatal intes-tinal vasculopathy in a young female. Eur J Dermatol. 2005;15:396-400.

24. Nakamori A, Yamaguchi Y, Kurimoto I, et al. Vesiculobullous dermato-myositis with panniculitis without muscle disease. J Am Acad Dermatol. 2003;49:1136-1139.

25. Solans R, Cortés J, Selva A, et al. Panniculitis: a cutaneous manifestation of dermatomyositis. J Am Acad Dermatol. 2002;46:S148-S150.

26. Chao YY, Yang LJ. Dermatomyositis presenting as panniculitis. Int J Dermatol. 2000;39:141-144.

27. Lee MW, Lim YS, Choi JH, et al. Panniculitis showing membranocystic changes in the dermatomyositis. J Dermatol. 1999;26:608‐610.

28. Ghali FE, Reed AM, Groben PA, et al. Panniculitis in juvenile dermato-myositis. Pediatr Dermatol. 1999;16:270-272.

29. Molnar K, Kemeny L, Korom I, et al. Panniculitis in dermatomyositis: report of two cases. Br J Dermatol. 1998;139:161‐163.

30. Ishikawa O, Tamura A, Ryuzaki K, et al. Membranocystic changes in the panniculitis of dermatomyositis. Br J Dermatol. 1996;134:773-776.

31. Sabroe RA, Wallington TB, Kennedy CT. Dermatomyositis treated with high-dose intravenous immunoglobulins and associated with pannicu-litis. Clin Exp Dermatol. 1995;20:164-167.

32. Neidenbach PJ, Sahn EE, Helton J. Panniculitis in juvenile dermatomyo-sitis. J Am Acad Dermatol. 1995;33:305-307.

33. Fusade T, Belanyi P, Joly P, et al. Subcutaneous changes in dermatomyo-sitis. Br J Dermatol. 1993;128:451-453.

34. Winkelmann WJ, Billick RC, Srolovitz H. Dermatomyositis presenting as panniculitis. J Am Acad Dermatol. 1990;23:127-128.

35. Commens C, O’Neill P, Walker G. Dermatomyositis associated with multifocal lipoatrophy. J Am Acad Dermatol. 1990;22:966-969.

36. Raimer SS, Solomon AR, Daniels JC. Polymyositis presenting with pan-niculitis. J Am Acad Dermatol. 1985;13(2 pt 2):366‐369.

37. Feldman D, Hochberg MC, Zizic TM, et al. Cutaneous vasculitis in adult polymyositis/dermatomyositis. J Rheumatol. 1983;10:85-89.

38. Kimura S, Fukuyama Y. Tubular cytoplasmic inclusions in a case of childhood dermatomyositis with migratory subcutaneous nodules. Eur J Pediatr. 1977;125:275-283.

39. Weber FP, Gray AMH. Chronic relapsing polydermatomyositis with predominant involvement of the subcutaneous fat. Br J Dermatol. 1924;36:544-560.

40. Santos‐Briz A, Calle A, Linos K, et al. Dermatomyositis panniculitis: a clinicopathological and immunohistochemical study of 18 cases. J Eur


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spec

ts o

f so

me

inte

rpha

lang

eal jo

ints

with

sm

all u

lcer

ated

pap

ules

; fiss

ured

er

ythe

mat

ous

and

edem

atou

s pl

aque

s on

the

finge

rtips

, spl

inte

r he

mor

rhag

es, n

ecro

tic p

laqu

es

and

pain

ful u

lcer

atio

ns o

n th

e ha

nds

and

feet

Pre

dnis

one

and

AZA

; the

n cy

clop

hosp

ham

ide

and

IVIG

Pat

ient

die

d du

e to

at

riove

ntric

ular

blo

ck-

-

1365

/F15

11 m

o pr

ior

Arm

s, le

gsN

one

Pre

dnis

olon

e w

ith re

curre

nce;

then

M

TX a

nd p

redn

isol

one

Impr

oved

--

1463

/F2

25 m

o la

ter

Arm

s, th

ighs

, bu

ttock

sP

erio

rbita

l vio

lace

ous

eryt

hem

a,

liche

noid

vio

lace

ous

papu

les

over

lyin

g th

e kn

uckl

es a

nd

dors

al h

ands

Pre

dnis

one

20 m

g da

ily, M

TX 1

5 m

g w

eekl

y, a

nd fo

lic a

cid

(AZA

dis

cont

inue

d du

e to

leuk

open

ia, a

nd m

ycop

heno

late

m

ofet

il dis

cont

inue

d du

e to

gen

eral

ized

m

orbi

llifor

m e

rupt

ion

Impr

oved

1 m

oO

varia

n ad

enoc

arci

nom

a

Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

1530

/F16

2 m

o la

ter

Upp

er a

nd

low

er lim

bsEd

emat

ous

purp

lish

eyel

ids,

pa

tche

s of

alo

peci

a w

ith

desq

uam

atio

n on

the

scal

p

Mep

redn

ison

e 60

mg

daily

Res

olve

d1

wk

-

1664

/F17

2 m

o la

ter

Elbo

ws,

wris

ts, t

oes

Eryt

hem

atou

s ra

shes

on

the

arm

s, tr

unk,

legs

, and

face

Pre

dnis

olon

e 40

mg

daily

Res

olve

d3

mo

-

1723

/F18

Con

curre

ntLe

gsEr

ythe

ma

the

over

eye

brow

s an

d ch

eeks

S

topp

ed m

inoc

yclin

e; th

en p

redn

ison

e

40 m

g da

ily a

nd M

TX 1

5 m

g w

eekl

yR

esol

ved

9 m

o-

1873

/F19

4 m

o la

ter

Thig

hs, u

pper

arm

sEr

ythe

mat

ous

erup

tion

over

the

cent

ral c

hest

, jaw

line,

eye

lids,

an

d sc

alp

Pre

dnis

olon

e 40

mg

daily

, cyc

losp

orin

e 10

0 m

g tw

ice

daily

; the

n H

CQ

200

mg

twic

e da

ily; t

hen

MTX

22.

5 m

g w

eekl

y;

then

pre

dnis

olon

e, m

epac

rine

100

mg

twic

e da

ily

Impr

oved

--

1950

/F19

2 y

late

rA

rms

and

legs

Eryt

hem

atou

s fa

cial

rash

, G

ottro

n pa

pule

s, v

ascu

litic

le

sion

s

Bef

ore

pann

icul

itis:

pre

dnis

olon

e 20

m

g da

ily, A

ZA 1

00 m

g da

ily, I

VIG

, MTX

, cy

clos

porin

e); a

fter p

anni

culit

is: m

epac

rine,

H

CQ

, pre

dnis

olon

e, IV

IG w

eekl

y fo

r 6 w

k

Impr

oved

--

2023

/F20

Late

rU

pper

limbs

Eryt

hem

atou

s vi

olac

eous

rash

on

the

face

, nec

k, c

hest

and

limbs

; he

liotro

pe ra

sh; r

ed to

vio

lace

ous

plaq

ues

on s

un-e

xpos

ed a

reas

on

the

dors

um o

f the

fing

ers

and

hand

s; p

eriu

ngua

l ery

them

a an

d te

lang

iect

asia

; diff

use

alop

ecia

--

--

2129

/F20

Con

curre

ntFi

nger

s, th

ighs

Eryt

hem

atou

s vi

olac

eous

rash

of

the

face

, nec

k, c

hest

and

low

er

extre

miti

es; p

eriu

ngua

l ery

them

a;

digi

tal p

etec

hiae

--

--

2219

/M21

15 m

o la

ter

Thig

hVi

olac

eous

rash

on

the

uppe

r ey

elid

s, s

tern

um, a

nd k

nuck

les

Bef

ore

pann

icul

itis:

pre

dnis

one

1 m

g/kg

da

ily, A

ZA 2

.5 m

g/kg

dai

ly, th

en ta

per;

af

ter p

anni

culit

is: p

redn

ison

e 1

mg/

kg

daily

, AZA

3 m

g/kg

dai

ly

Res

olve

d4

wk

2335

/F22

Con

curre

ntR

ight

arm

Eryt

hem

atou

s to

vio

lace

ous

mac

ulop

apul

es o

n th

e fo

rehe

ad,

periu

ngua

l ery

them

a

Cyc

loph

osph

amid

e 60

0 m

g m

onth

ly fo

r 6

mo,

pre

dnis

olon

e 50

mg

daily

Impr

oved

--

(co

ntin

ued

)

CO

NT

INU

ED

ON

NE

XT

PA

GE


CUTIS

Do

not c

opy



Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

860

/F10

2 y

late

rTh

ighs

, but

tock

sD

M w

ith ty

pica

l ski

n ch

ange

sB

efor

e pa

nnic

uliti

s: p

redn

ison

e

10 m

g tw

ice

daily

, IVI

G 2

g/k

g m

onth

ly,

cycl

opho

spha

mid

e 50

mg

daily

; afte

r pa

nnic

uliti

s: p

redn

ison

e 25

mg

twic

e da

ily, c

yclo

phos

pham

ide

50 m

g da

ily,

IVIG

2 g

/kg

mon

thly

Pan

nicu

litis

per

sist

ed-

-

926

/F11

26 m

o la

ter

Arm

s, le

gs, a

bdom

enG

ottro

n pa

pule

s, h

elio

trope

sB

efor

e pa

nnic

uliti

s: c

hlor

oqui

ne 2

50 m

g da

ily, a

zath

iopr

ine

100

mg

daily

, and

pr

edni

solo

ne 3

0 m

g da

ily);

afte

r pan

nicu

litis

: th

en c

olch

icin

e 0.

6 m

g da

ily

Pan

nicu

litis

per

sist

ed-

Nas

opha

ryng

eal

canc

er

1015

/F12

5 y

late

rP

roxi

mal

upp

er

and

low

er

extre

miti

es, b

ack

Cut

aneo

us le

sion

s in

the

mal

ar

regi

on a

nd o

n do

rsal

han

dsB

efor

e pa

nnic

uliti

s: IV

met

hylp

redn

isol

one

30 m

g/kg

mon

thly,

MTX

10

mg

wee

kly,

folic

ac

id, H

CQ

200

mg

daily

); af

ter p

anni

culit

is:

3 bo

li of I

V m

ethy

lpre

dnis

olon

e 0.

5 m

g/kg

, pr

edni

sone

60

mg

daily

, cyc

losp

orin

e

150

mg

twic

e da

ily

Res

olve

d4

wk

-

1140

/F13

5 y

late

rA

xilla

e, a

bdom

enEr

ythe

ma

on th

e ey

elid

s;

kera

totic

ery

them

atou

s pl

aque

s on

the

elbo

ws,

kne

es a

nd

dors

al a

spec

ts o

f the

fing

ers;

er

ythe

mat

ous

lesi

ons

on th

e ne

ck

and

ches

t

Bef

ore

pann

icul

itis:

pre

dnis

olon

e 60

mg

daily

, MTX

4 m

g w

eekl

y; a

fter p

anni

culit

is:

cycl

ospo

rine

150

mg

daily

Impr

oved

--

1255

/M14

Con

curre

ntFe

etLi

vedo

id e

ryth

emat

ovio

lace

ous

mac

ules

on

the

palm

ar a

spec

ts o

f so

me

inte

rpha

lang

eal jo

ints

with

sm

all u

lcer

ated

pap

ules

; fiss

ured

er

ythe

mat

ous

and

edem

atou

s pl

aque

s on

the

finge

rtips

, spl

inte

r he

mor

rhag

es, n

ecro

tic p

laqu

es

and

pain

ful u

lcer

atio

ns o

n th

e ha

nds

and

feet

Pre

dnis

one

and

AZA

; the

n cy

clop

hosp

ham

ide

and

IVIG

Pat

ient

die

d du

e to

at

riove

ntric

ular

blo

ck-

-

1365

/F15

11 m

o pr

ior

Arm

s, le

gsN

one

Pre

dnis

olon

e w

ith re

curre

nce;

then

M

TX a

nd p

redn

isol

one

Impr

oved

--

1463

/F2

25 m

o la

ter

Arm

s, th

ighs

, bu

ttock

sP

erio

rbita

l vio

lace

ous

eryt

hem

a,

liche

noid

vio

lace

ous

papu

les

over

lyin

g th

e kn

uckl

es a

nd

dors

al h

ands

Pre

dnis

one

20 m

g da

ily, M

TX 1

5 m

g w

eekl

y, a

nd fo

lic a

cid

(AZA

dis

cont

inue

d du

e to

leuk

open

ia, a

nd m

ycop

heno

late

m

ofet

il dis

cont

inue

d du

e to

gen

eral

ized

m

orbi

llifor

m e

rupt

ion

Impr

oved

1 m

oO

varia

n ad

enoc

arci

nom

a

Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

1530

/F16

2 m

o la

ter

Upp

er a

nd

low

er lim

bsEd

emat

ous

purp

lish

eyel

ids,

pa

tche

s of

alo

peci

a w

ith

desq

uam

atio

n on

the

scal

p

Mep

redn

ison

e 60

mg

daily

Res

olve

d1

wk

-

1664

/F17

2 m

o la

ter

Elbo

ws,

wris

ts, t

oes

Eryt

hem

atou

s ra

shes

on

the

arm

s, tr

unk,

legs

, and

face

Pre

dnis

olon

e 40

mg

daily

Res

olve

d3

mo

-

1723

/F18

Con

curre

ntLe

gsEr

ythe

ma

the

over

eye

brow

s an

d ch

eeks

S

topp

ed m

inoc

yclin

e; th

en p

redn

ison

e

40 m

g da

ily a

nd M

TX 1

5 m

g w

eekl

yR

esol

ved

9 m

o-

1873

/F19

4 m

o la

ter

Thig

hs, u

pper

arm

sEr

ythe

mat

ous

erup

tion

over

the

cent

ral c

hest

, jaw

line,

eye

lids,

an

d sc

alp

Pre

dnis

olon

e 40

mg

daily

, cyc

losp

orin

e 10

0 m

g tw

ice

daily

; the

n H

CQ

200

mg

twic

e da

ily; t

hen

MTX

22.

5 m

g w

eekl

y;

then

pre

dnis

olon

e, m

epac

rine

100

mg

twic

e da

ily

Impr

oved

--

1950

/F19

2 y

late

rA

rms

and

legs

Eryt

hem

atou

s fa

cial

rash

, G

ottro

n pa

pule

s, v

ascu

litic

le

sion

s

Bef

ore

pann

icul

itis:

pre

dnis

olon

e 20

m

g da

ily, A

ZA 1

00 m

g da

ily, I

VIG

, MTX

, cy

clos

porin

e); a

fter p

anni

culit

is: m

epac

rine,

H

CQ

, pre

dnis

olon

e, IV

IG w

eekl

y fo

r 6 w

k

Impr

oved

--

2023

/F20

Late

rU

pper

limbs

Eryt

hem

atou

s vi

olac

eous

rash

on

the

face

, nec

k, c

hest

and

limbs

; he

liotro

pe ra

sh; r

ed to

vio

lace

ous

plaq

ues

on s

un-e

xpos

ed a

reas

on

the

dors

um o

f the

fing

ers

and

hand

s; p

eriu

ngua

l ery

them

a an

d te

lang

iect

asia

; diff

use

alop

ecia

--

--

2129

/F20

Con

curre

ntFi

nger

s, th

ighs

Eryt

hem

atou

s vi

olac

eous

rash

of

the

face

, nec

k, c

hest

and

low

er

extre

miti

es; p

eriu

ngua

l ery

them

a;

digi

tal p

etec

hiae

--

--

2219

/M21

15 m

o la

ter

Thig

hVi

olac

eous

rash

on

the

uppe

r ey

elid

s, s

tern

um, a

nd k

nuck

les

Bef

ore

pann

icul

itis:

pre

dnis

one

1 m

g/kg

da

ily, A

ZA 2

.5 m

g/kg

dai

ly, th

en ta

per;

af

ter p

anni

culit

is: p

redn

ison

e 1

mg/

kg

daily

, AZA

3 m

g/kg

dai

ly

Res

olve

d4

wk

2335

/F22

Con

curre

ntR

ight

arm

Eryt

hem

atou

s to

vio

lace

ous

mac

ulop

apul

es o

n th

e fo

rehe

ad,

periu

ngua

l ery

them

a

Cyc

loph

osph

amid

e 60

0 m

g m

onth

ly fo

r 6

mo,

pre

dnis

olon

e 50

mg

daily

Impr

oved

--

CO

NT

INU

ED

ON

NE

XT

PA

GE

(co

ntin

ued

)


CUTIS

Do

not c

opy



Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

2456

/F22

2 y

late

rA

rms

Faci

al e

ryth

ema

and

edem

a,

helio

trope

sig

n, G

ottro

n si

gn,

periu

ngua

l ery

them

a

Bef

ore

pann

icul

itis:

pre

dnis

olon

e 30

mg

daily

; afte

r pan

nicu

litis

: pre

dnis

olon

e 20

mg

daily

, AZT

100

mg

daily

Impr

oved

-P

arot

id

carc

inom

a

2521

/F23

6 w

k pr

ior

Rig

ht lo

wer

ab

dom

en, l

abia

m

ajor

a, in

ner t

high

Mal

ar ra

sh, v

iola

ceou

s in

filtra

ted

plaq

ues

on th

e ea

rs, p

eriu

ngua

l te

lang

iect

asia

, hel

iotro

pe ra

sh,

Got

tron

papu

les

Ora

l pre

dnis

olon

e 25

mg

daily

, H

CQ

200

mg

daily

; the

n IV

m

ethy

lpre

dnis

olon

e 60

mg

daily

for

1 m

o; th

en m

ethy

lpre

dnis

olon

e 1

g

for 3

d, t

hen

tape

r

Pat

ient

die

d 9

mo

afte

r di

seas

e on

set,

prob

ably

du

e to

com

plic

atio

ns

from

gas

troin

test

inal

is

chem

ia/p

erfo

ratio

n

--

2660

/F24

Con

curre

ntA

rms

Hel

iotro

pe p

erio

rbita

l ede

ma;

pe

riung

ual n

ailfo

ld te

lang

iect

asia

; G

ottro

n pa

pule

s; e

ryth

ema

on th

e fa

ce, t

runk

, and

ext

rem

ities

Met

hylp

redn

isol

one

1000

mg

daily

for 3

d,

pred

niso

lone

60

mg

daily

, the

n ta

per

Res

olve

d-

-

2742

/F25

17 m

o la

ter

Arm

s, th

ighs

, ab

dom

enEy

elid

ery

them

aP

redn

ison

e 1

mg/

kg d

aily,

AZA

100

mg

daily

, HC

Q 1

00 m

g tw

ice

daily

; the

n pr

edni

sone

20

mg

daily

, MTX

7.5

mg

wee

kly;

then

pre

dnis

one

0.5

mg/

kg d

aily,

cy

clos

porin

e 10

0 m

g tw

ice

daily

Impr

oved

, rec

urre

d,

reso

lved

--

2880

/F25

10 m

o la

ter

Arm

s, th

ighs

Eyel

id e

ryth

ema,

ery

them

atou

s cu

tane

ous

lesi

ons

on th

e el

bow

s an

d kn

ees

Pre

dnis

one

1 m

g/kg

dai

ly, A

ZA 1

00 m

g da

ily; t

hen

dicl

ofen

ac 5

0 m

g tw

ice

daily

; th

en p

redn

ison

e 0.

5 m

g/kg

dai

ly; t

hen

pred

niso

ne 7

.5 m

g da

ily

Impr

oved

, rec

urre

d,

reso

lved

--

2944

/F26

2.5

mo

prio

rS

houl

ders

, bac

k,

ches

t, ab

dom

en,

butto

ck, t

high

s

Diff

use

eryt

hem

atou

s to

vi

olac

eous

sw

ellin

g of

the

face

, ne

ck, a

nd s

houl

der

Pre

dnis

olon

e 12

0 m

g on

ce d

aily,

M

TX 7

.5 m

g w

eekl

y, A

ZT 2

50 m

g w

eekl

y,

IVIG

25

g 5

times

mon

thly

Res

olve

d6

mo

-

3042

/M27

1 y

prio

rLe

ft bu

ttock

, lef

t in

guin

al a

rea

Per

iorb

ital e

ryth

ema

and

edem

a;

tela

ngie

ctat

ic e

ryth

ema

on th

e ch

eeks

, nec

k, a

nd fo

rear

ms;

er

ythe

mat

ous

papu

les

on th

e ha

nds

over

the

join

ts

Pre

dnis

olon

e an

d M

TX; t

hen

pred

niso

lone

30

mg

daily

, HC

Q 6

00 m

g da

ily, c

olch

icin

e 1.

8 m

g da

ily, p

ento

xify

lline

1200

mg

daily

Impr

oved

--

3114

/M28

4 y

late

rFo

rear

m, t

high

, flan

kH

elio

trope

rash

, Got

tron

papu

les,

po

ikilo

derm

a, d

ilate

d na

ilfold

ca

pilla

ries,

pho

tose

nsiti

ve

erup

tion

loca

lized

to th

e fa

ce a

nd

exte

nsor

asp

ects

of t

he u

pper

ex

trem

ities

Bef

ore

pann

icul

itis:

HC

Q 4

00 m

g da

ily,

pred

niso

ne 2

.5 m

g da

ily; a

fter p

anni

culit

is:

pred

niso

ne 0

.3 m

g/kg

dai

ly, th

en ta

per

Res

olve

d2

wk

-

Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

3254

/F29

Con

curre

nt

Arm

sP

erio

rbita

l ede

ma;

ery

them

a on

th

e ar

ms,

face

, eye

lids,

fing

ers,

kn

ees,

and

thor

ax; e

ryth

emat

ous

papu

les

over

the

MC

P jo

ints

Pre

dnis

olon

e 1

mg/

kg d

aily,

A

ZT 2

mg/

kg d

aily

Res

olve

d -

-

3357

/F29

Con

curre

ntB

utto

cks,

left

thig

h,

sacr

al re

gion

Faci

al ra

shM

ethy

lpre

dnis

olon

e 1

mg/

kg d

aily

for 2

wk,

th

en c

yclo

spor

ine

3 m

g/kg

dai

lyR

esol

ved

--

3465

/F30

Con

curre

ntB

utto

cks,

thig

hs,

low

er ri

ght l

egB

row

nish

ery

them

atou

s le

sion

s w

ith te

lang

iect

asia

on

the

neck

, ba

ck, b

utto

cks,

and

ext

enso

r su

rface

s of

the

fore

arm

s an

d lo

wer

legs

; slig

htly

ker

atot

ic

eryt

hem

atou

s le

sion

s on

the

elbo

ws,

poi

kilo

derm

atou

s ch

ange

s on

the

neck

, upp

er

ches

t, an

d ba

ck

Pre

dnis

olon

e 60

mg

daily

, the

n ta

per;

then

m

ethy

lpre

dnis

olon

e 10

00 m

g on

e tim

e,

pred

niso

lone

60

mg

daily

Pan

nicu

litis

per

sist

ed-

-

3542

/M31

5 y

late

rB

utto

cks

Eryt

hem

atou

s sw

olle

n sk

in o

ver

the

exte

nsor

sur

face

s of

the

hand

jo

ints

, elb

ows,

and

kne

es a

nd

over

the

med

ial a

nd la

tera

l ank

le

mal

leol

i, bu

ttock

s, e

ar tr

agi,

right

up

per e

yelid

; nai

l fol

d in

farc

ts,

ragg

ed c

utic

les,

per

iung

ual

tela

ngie

ctas

ia

IV/IM

/ora

l ste

roid

s, IV

/ora

l cy

clop

hosp

ham

ide,

cyc

losp

orin

e, A

ZA,

MTX

, HC

Q, m

epac

rine,

chl

oram

buci

l, pl

asm

a ex

chan

ge, a

ntith

ymoc

yte

glob

ulin

, IV

IG 2

mg/

kg m

onth

ly fo

r 5 m

o

Res

olve

d-

-

363/

F32C

oncu

rrent

Rig

ht in

ner a

rmFa

int e

ryth

ema

and

edem

a of

th

e ey

elid

s, fi

nger

s, k

nees

, and

ab

dom

en; e

ryth

emat

ous

papu

les

over

the

MC

P jo

ints

; dila

ted

prox

imal

nai

lfold

cap

illarie

s

Ora

l pre

dnis

one

1 m

g/kg

dai

ly; t

hen

MTX

an

d in

crea

sed

syst

emic

ste

roid

s P

anni

culit

is re

solv

ed,

othe

r cut

aneo

us

man

ifest

atio

ns d

id n

ot;

wea

knes

s re

curre

d

--

3742

/F33

10 m

o pr

ior

But

tock

s, th

ighs

, ar

ms,

abd

omen

, br

east

s

Non

eP

redn

ison

e 1

mg/

kg d

aily,

then

tape

rR

esol

ved,

recu

rred

with

ste

roid

tape

r, th

en

impr

oved

29 m

o-

3824

/F34

3 m

o pr

ior

Left

arm

Infra

orbi

tal e

ryth

ema

and

edem

a,

perio

rbita

l dis

colo

ratio

n, G

ottro

n pa

pule

s

HC

Q 2

00 m

g tw

ice

wee

kly

incr

ease

d to

da

ily d

oses

; the

n pr

edni

sone

60

mg

daily

, M

TX 7

.5 w

eekl

y, th

en ta

per

Impr

oved

--

397/

M35

1 y

late

rB

utto

cks,

thig

hsEr

ythe

mat

osqu

amou

s er

uptio

n on

the

back

s of

fing

ers,

han

ds,

elbo

ws,

and

pla

ntar

sur

face

s;

faci

al e

ryth

ema

Pre

dnis

one

2 m

g/kg

dai

ly, th

en ta

per,

th

en in

crea

seIm

prov

ed-

-

(co

ntin

ued

)

CO

NT

INU

ED

ON

NE

XT

PA

GE


CUTIS

Do

not c

opy



Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

2456

/F22

2 y

late

rA

rms

Faci

al e

ryth

ema

and

edem

a,

helio

trope

sig

n, G

ottro

n si

gn,

periu

ngua

l ery

them

a

Bef

ore

pann

icul

itis:

pre

dnis

olon

e 30

mg

daily

; afte

r pan

nicu

litis

: pre

dnis

olon

e 20

mg

daily

, AZT

100

mg

daily

Impr

oved

-P

arot

id

carc

inom

a

2521

/F23

6 w

k pr

ior

Rig

ht lo

wer

ab

dom

en, l

abia

m

ajor

a, in

ner t

high

Mal

ar ra

sh, v

iola

ceou

s in

filtra

ted

plaq

ues

on th

e ea

rs, p

eriu

ngua

l te

lang

iect

asia

, hel

iotro

pe ra

sh,

Got

tron

papu

les

Ora

l pre

dnis

olon

e 25

mg

daily

, H

CQ

200

mg

daily

; the

n IV

m

ethy

lpre

dnis

olon

e 60

mg

daily

for

1 m

o; th

en m

ethy

lpre

dnis

olon

e 1

g

for 3

d, t

hen

tape

r

Pat

ient

die

d 9

mo

afte

r di

seas

e on

set,

prob

ably

du

e to

com

plic

atio

ns

from

gas

troin

test

inal

is

chem

ia/p

erfo

ratio

n

--

2660

/F24

Con

curre

ntA

rms

Hel

iotro

pe p

erio

rbita

l ede

ma;

pe

riung

ual n

ailfo

ld te

lang

iect

asia

; G

ottro

n pa

pule

s; e

ryth

ema

on th

e fa

ce, t

runk

, and

ext

rem

ities

Met

hylp

redn

isol

one

1000

mg

daily

for 3

d,

pred

niso

lone

60

mg

daily

, the

n ta

per

Res

olve

d-

-

2742

/F25

17 m

o la

ter

Arm

s, th

ighs

, ab

dom

enEy

elid

ery

them

aP

redn

ison

e 1

mg/

kg d

aily,

AZA

100

mg

daily

, HC

Q 1

00 m

g tw

ice

daily

; the

n pr

edni

sone

20

mg

daily

, MTX

7.5

mg

wee

kly;

then

pre

dnis

one

0.5

mg/

kg d

aily,

cy

clos

porin

e 10

0 m

g tw

ice

daily

Impr

oved

, rec

urre

d,

reso

lved

--

2880

/F25

10 m

o la

ter

Arm

s, th

ighs

Eyel

id e

ryth

ema,

ery

them

atou

s cu

tane

ous

lesi

ons

on th

e el

bow

s an

d kn

ees

Pre

dnis

one

1 m

g/kg

dai

ly, A

ZA 1

00 m

g da

ily; t

hen

dicl

ofen

ac 5

0 m

g tw

ice

daily

; th

en p

redn

ison

e 0.

5 m

g/kg

dai

ly; t

hen

pred

niso

ne 7

.5 m

g da

ily

Impr

oved

, rec

urre

d,

reso

lved

--

2944

/F26

2.5

mo

prio

rS

houl

ders

, bac

k,

ches

t, ab

dom

en,

butto

ck, t

high

s

Diff

use

eryt

hem

atou

s to

vi

olac

eous

sw

ellin

g of

the

face

, ne

ck, a

nd s

houl

der

Pre

dnis

olon

e 12

0 m

g on

ce d

aily,

M

TX 7

.5 m

g w

eekl

y, A

ZT 2

50 m

g w

eekl

y,

IVIG

25

g 5

times

mon

thly

Res

olve

d6

mo

-

3042

/M27

1 y

prio

rLe

ft bu

ttock

, lef

t in

guin

al a

rea

Per

iorb

ital e

ryth

ema

and

edem

a;

tela

ngie

ctat

ic e

ryth

ema

on th

e ch

eeks

, nec

k, a

nd fo

rear

ms;

er

ythe

mat

ous

papu

les

on th

e ha

nds

over

the

join

ts

Pre

dnis

olon

e an

d M

TX; t

hen

pred

niso

lone

30

mg

daily

, HC

Q 6

00 m

g da

ily, c

olch

icin

e 1.

8 m

g da

ily, p

ento

xify

lline

1200

mg

daily

Impr

oved

--

3114

/M28

4 y

late

rFo

rear

m, t

high

, flan

kH

elio

trope

rash

, Got

tron

papu

les,

po

ikilo

derm

a, d

ilate

d na

ilfold

ca

pilla

ries,

pho

tose

nsiti

ve

erup

tion

loca

lized

to th

e fa

ce a

nd

exte

nsor

asp

ects

of t

he u

pper

ex

trem

ities

Bef

ore

pann

icul

itis:

HC

Q 4

00 m

g da

ily,

pred

niso

ne 2

.5 m

g da

ily; a

fter p

anni

culit

is:

pred

niso

ne 0

.3 m

g/kg

dai

ly, th

en ta

per

Res

olve

d2

wk

-

Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

3254

/F29

Con

curre

nt

Arm

sP

erio

rbita

l ede

ma;

ery

them

a on

th

e ar

ms,

face

, eye

lids,

fing

ers,

kn

ees,

and

thor

ax; e

ryth

emat

ous

papu

les

over

the

MC

P jo

ints

Pre

dnis

olon

e 1

mg/

kg d

aily,

A

ZT 2

mg/

kg d

aily

Res

olve

d -

-

3357

/F29

Con

curre

ntB

utto

cks,

left

thig

h,

sacr

al re

gion

Faci

al ra

shM

ethy

lpre

dnis

olon

e 1

mg/

kg d

aily

for 2

wk,

th

en c

yclo

spor

ine

3 m

g/kg

dai

lyR

esol

ved

--

3465

/F30

Con

curre

ntB

utto

cks,

thig

hs,

low

er ri

ght l

egB

row

nish

ery

them

atou

s le

sion

s w

ith te

lang

iect

asia

on

the

neck

, ba

ck, b

utto

cks,

and

ext

enso

r su

rface

s of

the

fore

arm

s an

d lo

wer

legs

; slig

htly

ker

atot

ic

eryt

hem

atou

s le

sion

s on

the

elbo

ws,

poi

kilo

derm

atou

s ch

ange

s on

the

neck

, upp

er

ches

t, an

d ba

ck

Pre

dnis

olon

e 60

mg

daily

, the

n ta

per;

then

m

ethy

lpre

dnis

olon

e 10

00 m

g on

e tim

e,

pred

niso

lone

60

mg

daily

Pan

nicu

litis

per

sist

ed-

-

3542

/M31

5 y

late

rB

utto

cks

Eryt

hem

atou

s sw

olle

n sk

in o

ver

the

exte

nsor

sur

face

s of

the

hand

jo

ints

, elb

ows,

and

kne

es a

nd

over

the

med

ial a

nd la

tera

l ank

le

mal

leol

i, bu

ttock

s, e

ar tr

agi,

right

up

per e

yelid

; nai

l fol

d in

farc

ts,

ragg

ed c

utic

les,

per

iung

ual

tela

ngie

ctas

ia

IV/IM

/ora

l ste

roid

s, IV

/ora

l cy

clop

hosp

ham

ide,

cyc

losp

orin

e, A

ZA,

MTX

, HC

Q, m

epac

rine,

chl

oram

buci

l, pl

asm

a ex

chan

ge, a

ntith

ymoc

yte

glob

ulin

, IV

IG 2

mg/

kg m

onth

ly fo

r 5 m

o

Res

olve

d-

-

363/

F32C

oncu

rrent

Rig

ht in

ner a

rmFa

int e

ryth

ema

and

edem

a of

th

e ey

elid

s, fi

nger

s, k

nees

, and

ab

dom

en; e

ryth

emat

ous

papu

les

over

the

MC

P jo

ints

; dila

ted

prox

imal

nai

lfold

cap

illarie

s

Ora

l pre

dnis

one

1 m

g/kg

dai

ly; t

hen

MTX

an

d in

crea

sed

syst

emic

ste

roid

s P

anni

culit

is re

solv

ed,

othe

r cut

aneo

us

man

ifest

atio

ns d

id n

ot;

wea

knes

s re

curre

d

--

3742

/F33

10 m

o pr

ior

But

tock

s, th

ighs

, ar

ms,

abd

omen

, br

east

s

Non

eP

redn

ison

e 1

mg/

kg d

aily,

then

tape

rR

esol

ved,

recu

rred

with

ste

roid

tape

r, th

en

impr

oved

29 m

o-

3824

/F34

3 m

o pr

ior

Left

arm

Infra

orbi

tal e

ryth

ema

and

edem

a,

perio

rbita

l dis

colo

ratio

n, G

ottro

n pa

pule

s

HC

Q 2

00 m

g tw

ice

wee

kly

incr

ease

d to

da

ily d

oses

; the

n pr

edni

sone

60

mg

daily

, M

TX 7

.5 w

eekl

y, th

en ta

per

Impr

oved

--

397/

M35

1 y

late

rB

utto

cks,

thig

hsEr

ythe

mat

osqu

amou

s er

uptio

n on

the

back

s of

fing

ers,

han

ds,

elbo

ws,

and

pla

ntar

sur

face

s;

faci

al e

ryth

ema

Pre

dnis

one

2 m

g/kg

dai

ly, th

en ta

per,

th

en in

crea

seIm

prov

ed-

-

CO

NT

INU

ED

ON

NE

XT

PA

GE

(co

ntin

ued

)


CUTIS

Do

not c

opy



Pat

ient

Ag

e, y

/se

x

Tim

e o

f p

anni

culit

is

ons

et

rela

tive

to

dia

gno

sis

o

f D

MLo

catio

n o

f p

anni

culit

is le

sio

nsO

ther

cut

aneo

us

man

ifest

atio

nsTr

eatm

ent

Trea

tmen

t re

spo

nse

Inte

rval

b

etw

een

ther

apy

initi

atio

n to

fina

l tr

eatm

ent

resp

ons

eM

alig

nanc

y

4058

/F36

4.5

mo

prio

rLe

ft th

igh,

abd

omen

, bu

ttock

sN

one

Pre

dnis

one

60 m

g da

ily, t

hen

tape

rR

esol

ved,

recu

rred

with

ste

roid

tape

r, th

en

reso

lved

-

4140

/F37

1 y

late

rLe

gsS

caly

ery

them

atou

s ra

sh o

n sh

ould

ers,

nec

k, k

nees

Pre

dnis

one

80 m

g da

ilyR

esol

ved

--

422/

M38

7 m

o la

ter

Extre

miti

es, c

hest

, gl

utea

l reg

ion

Faci

al e

ryth

ema,

hel

iotro

pe ra

sh,

edem

a ar

ound

eye

lids

Pre

dnis

olon

e 25

mg

daily

for 3

wee

ks,

then

tape

rR

esol

ved

6 w

k-

4322

/F39

Con

curre

ntA

rms,

thig

hsB

lotc

hy e

ryth

emat

ous

erup

tion

with

pru

ritus

ove

r the

bac

ks

of h

ands

(esp

ecia

lly th

e do

rsal

su

rface

s of

the

MC

P jo

ints

), fa

ce

(but

terfl

ylik

e di

strib

utio

n), a

nd

uppe

r ste

rnum

--

-

Cur

rent

ca

se62

/F

Follo

win

g ye

arA

rms,

axi

llae,

che

st,

abdo

men

, but

tock

s,

thig

hs

Viol

aceo

us, h

yper

pigm

ente

d,

perio

rbita

l ras

h an

d er

ythe

ma

of

the

ante

rior c

hest

Pre

dnis

one

0.9

mg/

kg d

aily,

MTX

15

–20

mg

wee

kly;

then

myc

ophe

nola

te

mof

etil 1

–3 g

dai

ly, H

CQ

200

mg

daily

Pan

nicu

litis

per

sist

ed-

-

Abb

revi

atio

ns: D

M, d

erm

atom

yosi

tis; F

, fem

ale;

MTX

, met

hotre

xate

; NA

, not

ava

ilabl

e; A

ZA, a

zath

iopr

ine;

MC

P, m

etac

arpo

phal

ange

al; H

CQ

, hyd

roxy

chlo

roqu

ine;

IV, i

ntra

veno

us; I

VIG

, int

rave

nous

imm

unog

lobu

lin;

M, m

ale;

IM, i

ntra

mus

cula

r.a L

itera

ture

revi

ew in

clud

ed c

ases

thro

ugh

July

201

9.

(co

ntin

ued

)


CUTIS

Do

not c

opy

Documents

Persistent Panniculitis in Dermatomyositis