Inflammatory Diseases of the Skin
Moderator: Dr. Lorenzo Cerroni
Case 12 -
Inflammatory Morphea Profunda
Carlo Tomasini, MD
A 56–year-old woman was admitted to our department with a six-month history of multiple
erythemato-violaceous and brownish plaques symmetrically distributed on the upper and lower extremities,
and on the trunk. The skin appeared appeared atrophic and there was a remarkable induration of the
All laboratory investigations including serology for Borrelia Burgdorfery, ANA and ENA, were within
normal range. No peripheral eosinophilia was present. Chest X-ray demonstrated a moderate lung
fibrosis, confirmed by CT scan. Involvement of other internal organs was excluded.
A deep incisional biopsy including the subcutaneous fat was performed on a lesion of the thigh.
Case 12 - Slide 1
Histopathology showed marked thickening of the septa of the subcutaneous fat with deposits of mucin in
the deep portion of the dermis with a slight superficial and deep perivascular infiltrate of
lymphocytes. The epidermis was unaffected. The changes in the hypodermis were characterized by
thickened, edematous and fibrotic septa and a moderately dense inflammatory infiltrate consisting mainly
of neutrophils with variable amounts of eosinophils, lymphocytes, and plasmacells. Mucin deposits were
also copiuos. Small nodular aggregates of lymphocytes resembling lymphoid follicles were also seen at
the periphery of the fat lobules. The lining of the vessels was focally thickened with plump endothelial
cells. No fibrin in the vessel walls or thrombi were seen. Direct immunofluorescence was negative for
Ig deposits at the dermo-epidermal junction or within vessels.
The diagnosis of inflammatory morphea profunda was made on the basis of clinical and histopathologic
findings and the patient was treated with prednisolone at 1mg/kg/die with a subjective improvement of
symptoms and a partial disappearing of cutaneous lesions after two months' therapy with progressive
tapering of the steroid.
The term "scleroderma" is applied to a spectrum of disorders characterized by thickening of the skin
and/or subcutaneous tissues . Two different clinico-laboratoristic subsets can usually be
identified: systemic scleroderma, in which vesceral involvement is present, and localized scleroderma,
or morphea, in which the lesions are limited to the skin. Transition from morphea to systemic
sclerodermia may occur. According with a recent classification, morphea may appear in 5 main forms :
plaque, generalized, bullous, linear and deep, or subcutaneous . All the
subtypes of morphea have a common histopathologic denominator, i.e, the sclerosis of the connective
tissue, in company with a variable inflammatory infiltrate.
The cause and pathogenesis of morphea are unknown. Some cases are claimed to be a consequence of
infection by Borrelia burgdorferi, but that has yet to be proven.
In morphea profunda, patients have multiple lesions on the upper and lower extremities, rarely on the
torso. A deep, bound-down induration of the skin is most characteristic, with often a cobblestone or
dimpling appearance of the overlying skin surface. The induration may cause a decreased range of motion
and, in severe cases, joint contractures. Occasionally, systemic involvement is present with mild
pulmonary and esophageal impairment.
Histopathologically, morphea profunda is fundamentally a septal panniculitis characterized by
variable thickening and hyalinization of collagen bundles in the deep dermis, the subcutaneous septa,
and sometimes fascia. A moderate to marked lymphocyte-predominant infiltrate is present interstitially
and around small vessels in the panniculus. Aggregates of lymphocytes and plasmacells are also seen at
the periphery of the lobules. In the early, evolving or acute inflammatory stages of morphea, septa
are widened by copious amounts of mucin with subtle, if any, thickening in bundles of collagen, and
eosinophils and neutrophils may predominate overwhelmingly within the inflammatory infiltate. In foci,
however, crowded, thickened collagen bundles are detectable  .
In the case described herein the presence of copious amounts of mucin throughout the deep dermis and
subcutaneous septa and nodular aggregates of lymphocytes at the periphery of the fat lobules could
evocate a diagnosis of lupus profundus. This
condition may present alone or in association with other clinico-pathologic expressions of lupus
erythematosus. Histopathologically, in lupus profundus inflammatory changes involve mainly the lobules
(lobular panniculitis), with a prominent lymphocytic infiltrate sometimes forming lymphoid follicles in
concert with plasma cells e mucin deposits. In time, hyaline sclerosis may replace necrotic lobules and
extend into the septa.
- Doyle JA, Connolly SM, Winkelmann RK. Cutaneous and subcutaneous inflammatory sclerosis syndrome. Arch Dermatol 1982; 118: 886-90
- Peterson LS, Nelson AM, Su WPD. Classification of morphea (linear scleroderma). Mayo Clin Proc 1995; 70: 1068-76.
- Su WPD, Person JR. Morphea profunda. A new concept and a histopathologic study of 23 cases. Am J Dermatopathol 1981; 3: 251-60.