Deep Morphea

Isabel Bielsa, MD,* and Aurelio Ariza, MD†

Deep morphea encompasses a variety of clinical entities in which inflammation and

sclerosis are found in the deep dermis, panniculus, fascia, or superficial muscle. Morphea
profunda, eosinophilic fasciitis, and disabling pansclerotic morphea of children are in-
cluded in this group, but overlapping of the extent and depth of cutaneous involvement in
these various conditions precludes their distinction on the sole basis of clinical or even
histologic examination. Furthermore, the limits between morphea profunda and generalized
morphea, which usually are classified outside this group, are not clear. Histologically, all
these disorders show similar inflammatory and sclerotic findings, the primary difference
being the depth of these changes. Associated clinical findings, including arthralgias,
arthritis, contractures, or carpal tunnel syndrome, are frequent. Although visceral compli-
cations are uncommon, pulmonary, esophageal, and even cardiac abnormalities have been
reported. Eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimenta-
tion rate may be present with disease activity. Laboratory studies may demonstrate
autoantibody production. Treatment is nonstandardized but UVA irradiation and antiinflam-
matory or immunosuppressive drugs (mainly antimalarial agents and corticosteroids) may
be beneficial.
Semin Cutan Med Surg 26:90-95 © 2007 Elsevier Inc. All rights reserved.

KEYWORDS localized scleroderma, subcutaneous morphea, morphea profunda, generalized

morphea, eosinophilic fasciitis, pansclerotic morphea

T he term deep morphea, or morphea profunda, describes

a variant of morphea (localized scleroderma) in which
inflammation and sclerosis are found in the deep dermis,
cently, Peterson and coworkers4 have proposed a morphea
classification that includes the plaque, generalized, bullous,
linear, and deep types and their corresponding subtypes (Ta-
panniculus, fascia, or superficial muscle. Deep morphea has ble 1). Essentially, plaque morphea is a superficial type often
been recognized for many years,1 but this form of localized confined to the dermis, whereas linear morphea consists of
scleroderma did not gain wide acceptance until the descrip- one or more indurate linear streaks that may involve the
tions by Person and Su in 1979 and 1981.2,3 dermis, subcutaneous tissue, muscle, and underlying bone.
Scleroderma encompasses a spectrum of disorders charac- Deep morphea lesions also involve the deep dermis, subcu-
terized by thickening of the skin and subcutaneous tissue. taneous tissue, and muscle but, in contrast to linear morphea,
Two different clinical categories generally are identified: sys- they are more diffuse and do not show a linear pattern. This
temic sclerosis, in which visceral changes are present, and review focuses on deep morphea.
localized scleroderma (morphea), in which lesions are lim-
ited to the skin. Nevertheless, as discussed herein, morphea Concept and Classification
cannot always be regarded as a purely cutaneous disease.
Several types of localized sclerosis or morphea may be In both editions of Jablonska’s classic monograph Sclero-
distinguished on the basis of clinical manifestations and lev- derma and Pseudoscleroderma (1965, 1975)1,5 subcutaneous
els of tissue involvement. However, morphea classification is morphea was described as a well-recognized form of local-
hampered by common overlaping of the various types. Re- ized scleroderma. In 1971, Winkelmann claimed that sclero-
sis in generalized morphea may be superficial, with clinical
features of lichen sclerosus and atrophicus or bullous mor-
*Department of Dermatology, Hospital Germans Trias i Pujol, Autonomous phea, dermal as in typical morphea, or deep with pannicular
University of Barcelona, Badalona, Barcelona, Spain.
or fascial involvement.6 In 1972, Fleischmajer and cowork-
†Department of Pathology, Hospital Germans Trias i Pujol, Autonomous
University of Barcelona, Badalona, Barcelona, Spain. ers7 emphasized that, in generalized morphea, as in systemic
Address reprint requests to Isabel Bielsa, C/Molas 8, Mataró-08302, Barce- scleroderma, early pathological changes occur in the deep
lona, Spain. E-mail: [email protected] dermis and panniculus.

90 1085-5629/07/$-see front matter © 2007 Elsevier Inc. All rights reserved.

doi:10.1016/j.sder.2007.02.005
Deep morphea 91

Table 1 Classification of Morphea (Localized Scleroderma) presentation in individual patients suggests that they may be
Plaque morphea closely related.2,11
Morphea en plaque
Guttate morphea
Atrophoderma of Pasini and Pierini Clinical Manifestations
Keloid morphea (nodular morphea)
(Lichen sclerosus et atrophicus) Morphea Profunda
Generalized morphea It is a generalized or, more rarely, localized sclerotic process
Bullous morphea that mainly involves the deep dermis and subcutaneous tis-
Linear morphea sue but also the fascia and superficial muscle. Clinically,
Linear morphea (linear scleroderma) plaques are mildly inflamed, hyperpigmented, symmetrical,
En coup de sabre
and somewhat ill-defined. The skin feels thickened and
Progressive hemifacial atrophy
Deep morphea
bound down to the underlying fascia and muscle. Plaques are
Morphea profunda smooth and shiny, but areas of both dermal and subcutane-
Eosinophilic fasciitis ous atrophy may be present, particularly in chronic lesions
Disabling pansclerotic morphea of children (Fig. 1).2,12-14 Coexistence with white morphea-like or lichen
Modified from Peterson LS, et al.4 sclerosus et atrophicus-like findings may happen in some
cases2 and sometimes bullae arise on the plaques.15,16 Clinical
findings may be difficult to distinguish from generalized
In 1974, Shulman8 described 2 male patients showing morphea, as shown by some cases which or with sclerosis of
what he believed to be a new sclerodermatous syndrome the panniculus and fascia.7,17-19 The limits between morphea
consisting of diffuse sclerotic fasciitis, elevated erythrocyte profunda and generalized morphea are not clear and, in fact,
sedimentation rate (ESR), hypergammaglobulinemia, and in their original description of subcutaneous morphea Person
peripheral eosinophilia. Onset was preceded by extreme and Su2 claimed that it may well be part of a spectrum in-
physical exertion in both patients, one of which improved cluding generalized morphea and eosinophilic fasciitis. The
with corticosteroid therapy. Shortly thereafter, Rodman and overlaping extent of cutaneous involvement shown by these
coworkers reported 6 patients (4 men and 2 women) with a various conditions precludes their clinical or even histologi-
similar disorder, although the dermis and panniculus also cal distinction.
showed inflammatory sclerosis with presence of eosinophils. Morphea profunda is more frequent in females than males.
They proposed the term “eosinophilic fasciitis” for this con- The onset of sclerosis is gradual and relatively rapid, usually
dition. Five of the 6 patients improved with corticosteroid occurring during a period of several months. Signs of acute
therap, and the remaining case experienced spontaneous re- inflammation, such as edema and erythema, are rarely ob-
mission.9 served. Increased physical exertion has been invoked as a
In 1979, Person and Su2 described 16 patients, 12 of them possible precipitating factor.2
women, with biopsy-proven generalized inflammatory scle- Sclerosis may involve the digits but is not associated with
rosis of the panniculus or fascia. Some of these patients ulceration, distal phalangeal resorption, or Raynaud’s phe-
showed some degree of systemic involvement and peripheral nomenon. Flexion contractures of joints are a relatively fre-
eosinophilia. They called this condition subcutaneous mor- quent finding, and carpal tunnel syndrome may be present as
phea and considered it to be part of a spectrum that included well. Contractures mainly develop in patients who are at
typical generalized morphea, eosinophilic fasciitis, and sys- prepubescence at the onset of disease.2,19 Arthralgias, arthri-
temic sclerosis. In 1981, the same authors3 increased the tis, or myalgias are commonly present. Evidence of abnormal
aforesaid group of patients to include 23 cases and described pulmonary function test results, esophageal sclerodermatous
their microscopic findings in detail. Similarly to the designa- changes, altered esophageal motility, and even renal or car-
tion lupus erythematosus profundus, they proposed the term diac disease have been documented in patients with morphea
morphea profunda instead of subcutaneous morphea, be- profunda as well as in the widespread or generalized type of
cause major pathological changes do occur in the superficial morphea.2,19,20 Peripheral eosinophilia, high serum gamma-
muscle, fascia, and deeper dermis in addition to the subcu- globulin or IgG levels, increased ESR, and serologic abnor-
taneous tissue.3 In 1980, Díaz-Perez and colleagues10 re- malities, including the presence of antinuclear antibodies
ported 14 children with an aggressive mutilating form of (ANAs), antisingle-stranded DNA (ssDNA) antibodies,21
cutaneous scleroderma that they named “disabling panscle- rheumatoid factor, and low serum complement levels, have
rotic morphea.” been noted in these patients.2,19 Specifically, the major anti-
Currently, morphea profunda, eosinophilic fasciitis, and gens recognized by antinuclear antibodies are nuclear his-
disabling pansclerotic morphea of children are clustered in tones (antihistone antibodies), especially H1, H2A, and
the same deep morphea group (Table 1).4 Although each of H2B.22-24 Antihistone antibodies and elevation of serum pro-
these conditions is prone to involve specific levels of the skin collagen type I are considered useful indicators of disease
and underlying structures (muscle and bone), they share sig- severity in patients with localized scleroderma.23,25 Recently,
nificant similarities in laboratory and histopathologic find- antiphospholipid antibodies against phosphatidylserine-pro-
ings and response to therapy. Additionally, their concomitant thrombin complex have been detected in generalized mor-
92 I. Bielsa and A. Ariza

Figure 1 (A) Smooth, shiny, hard and thickened plaques bound down to the underlying fascia and muscle on the lower
extremities. (B) Coexisting in the same patient with hyperpigmented and ill-defined plaques on the trunk.

phea with a frequency (27%) comparable with that of sys- Subsequently, the upper extremities medial aspects typically
temic lupus erythematosus (32%).26 show skin dimpling resulting from focal dermis-to-fascia
Occasionally, morphea profunda may appear as a solitary tethering caused by sclerotic bands striding the panniculus,
indurate plaque, often located on the upper trunk near the which is followed by a final stage consisting of induration and
spine, and is then known as solitary morphea profunda.27-30 tightening of the skin.36,37 Lesions of morphea on different
Generally considered being an unusual form of localized parts of the body are present in a significant proportion of
scleroderma, solitary morphea profunda frequently is asymp- cases (30%).37-39 Usually, these lesions are not synchronous
tomatic and shows no associated systemic involvement. His- with fasciitis and appear either before or after fascial inflam-
tological examination shows dense collagen sclerosis and a mation.
marked subcutaneous infiltrate of lymphocytes admixed Hypergammaglobulinemia, peripheral eosinophilia, and
with plasma cells. Deep dermis new bone formation sugges- increased ESR are features of eosinophilic fasciitis but, be-
tive of osteoma cutis has been demonstrated in some cause they may be transient, normal laboratory findings do
cases.31,32 Finally, several instances of morphea profunda or not rule out the diagnosis.37 Similarly, although eosinophilia
morphea-like reactions at previous vaccination33 or intra- may be seen on histological examination of the fascia (often
muscular vitamin K (phytonadione) injection34,35 sites have in association with peripheral eosinophilia), its presence is
been reported. not required for the diagnosis.40 Extracutaneous involvement
most commonly presents as synovitis or tenosynovitis, arthri-
Eosinophilic Fasciitis tis, contractures, or carpal tunnel syndrome. Although un-
Eosinophilic fasciitis is an uncommon disease characterized common and mild, other manifestations of visceral disease in
by a symmetrical, scleroderma-like thickening of the skin of eosinophilic fasciitis are pulmonary, muscle, esophageal, and
the limbs. Most patients start with an edematous phase that even cardiac abnormalities. Additionally, hematologic find-
may be accompanied by pitting edema of the extremities. ings other than eosinophilia, such as megakaryocytic apla-
Deep morphea 93

sia,41 aplastic anemia, thrombocytopenia,42-44 hemolytic ane-

mia,45 and lymphoproliferative or myeloproliferative
disorders, have been described.3,46,47

Disabling Pansclerotic Morphea of Children

Disabling pansclerotic morphea of children is an aggressive
mutilating variant of morphea with onset before the age of 14
years,10 although an adult onset has been described.48 This
condition involves all layers of the skin, extending through
the dermis and subcutaneous tissues to involve muscle, ten-
don, and bone. Typically, sclerotic plaques develop on the
extensor aspects of the extremities and trunk to progressively
involve the entire skin, including the neck, face, and scalp,
with sparing of the fingertips and toes and lack of Raynaud’s
phenomenon.10,49 Extensive, severe, unilateral lesions of
pansclerotic morphea also have been described.50 Painful ul- Figure 3 Sclerotic and thickened interlobular connective tissue
cerations and contracture deformities, often severe and dis- septa.
abling, occur as a result of pansclerotic changes. Soft-tissue
calcification and subsequent ulceration are recognized com-
plications posing significant management difficulties. Squa- hyalinization, particularly near the junction of the dermis
mous cell carcinoma arising in long-standing pansclerotic and subcutaneous fat, is common. The histopathological
morphea has been occasionally reported.48,51,52 changes in the deep fascia are identical to those in the over-
Frequent laboratory findings include peripheral eosino- lying panniculus and are equivalent to those found in eosin-
philia, hypergammaglobulinemia, and increased ESR. ANA ophilic fasciitis. In the latter condition, the fascia shows vary-
test results are sometimes positive. Finally, some visceral ing degrees of edema, thickening, sclerosis, and infiltration
complications such as abnormal pulmonary function test re- with lymphocytes, plasma cells, histiocytes, and eosinophils.
sults, myopathic electromyographic alterations related to the Eosinophils may be lacking, and their presence or absence
areas of sclerosis, and reduced esophageal motility have been seems to be related to the inflammatory process duration.
reported.10 Sclerotic changes may spread not only superficially into the
pannicular interlobular fibrous septa but also deeply into the
epimysium.40 Biopsy specimens of disabling pansclerotic
Histological Findings morphea show lymphocyte and plasma cell infiltration and
Similar histological findings are shown by the various clinical sclerosis in the entire dermis and panniculus.10
subtypes of deep morphea, which share homogenization of
collagen bands as their basic change. Consequently, distinc- Pathogenesis
tion among subtypes is determined by the depth of inflam-
mation and sclerosis. Biopsy specimens of subcutaneous The etiology of localized scleroderma is unknown. The in-
morphea show lymphocyte and plasma cell infiltration and flammatory stage is followed by dermal fibrosis due to exces-
collagen tissue thickening and hyalinization in the subcutis. sive accumulation of extracellular matrix components, espe-
Lymphocyte and plasma cell infiltration is perivascular in
distribution and involves fat lobules, whereas sclerosis pro-
duces thickened septa and fat lobule obliteration (Figs. 2-4).
Occasionally, there is formation of lymphocytic aggregates
reminiscent of lymphoid follicles devoid of germinal centers.
Eosinophils also may be part of the cellular infiltrate.3 Sub-
cutaneous sclerosis usually shows a more prominent inflam-
matory component than in the dermal variety.2 Deep dermis

Figure 2 Collagen tissue thickening and hyalinization involving the

reticular dermis and panniculus, with thickened septa and fat lobule Figure 4 The inflammatory infiltrate is mainly constituted by lym-
obliteration. phocytes and plasma cells.
94 I. Bielsa and A. Ariza

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