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PubMed2003

Ulcers caused by bullous morphea treated with tissue-engineered skin.

Bullous morphea is an uncommon form of localized scleroderma. The exact pathogenesis is unknown and treatment of the accompanying ulcers is problematic. We report a patient with bullous morphea with long-standing ulcers whom we successfully treated with the tissue-engineered skin Apligraf (Organogenesis Inc., Canton, MA). The patient experienced rapid improvement in granulation tissue and the ulcers healed 4 months after a single application. The rationale for the use of Apligraf is based on experience with patients with venous ulcers who have surrounding peri-ulcer fibrosis. This condition, termed lipodermatosclerosis, has been reported as a poor prognostic factor for healing, yet many ulcers associated with lipodermatosclerosis may respond to treatment with tissue-engineered skin. Taken in concert, these results suggest a role for tissue- engineered skin in the treatment of chronic wounds with surrounding fibrosis.

Collagen↗

PubMed2001

Morphoea in three siblings.

Atrophoderma of Pasini and Pierini (APP) is an uncommon form of localized morphoea that occurs as superficial, hyperpigmented plaques distributed mainly on the trunk and proximal part of the limbs. There is little information about the influence of genetic and environmental factors on disease susceptibility and expression for localized scleroderma, although APP familial cases have been reported. We report three siblings without a family history of autoimmune disease presenting cutaneous lesions suggesting morphoea (APP variant).

Adult↗

PubMed2003

Influence of 5-aminolevulinic acid and red light on collagen metabolism of human dermal fibroblasts.

Patients with localized scleroderma receiving topical photodynamic therapy with 5-aminolevulinic acid show a reduction in skin tightness, suggesting that this therapy reduces skin sclerosis. To investigate potential mechanisms, the effects of 5-aminolevulinic acid and light on collagen metabolism were studied in vitro. Normal and scleroderma fibroblasts were treated with sublethal doses of 5-aminolevulinic acid and red light and transferred to three-dimensional collagen lattices. Cell supernatants were taken 6-72 h after photodynamic therapy to determine protein levels of the matrix metalloproteinases 1, 2, and 3, and of their inhibitors, tissue inhibitor of metalloproteinase 1 and 2 by enzyme-linked immunosorbent assay. Cellular mRNA expression of these proteins and of collagen type I and III was measured by quantitative real-time polymerase chain reaction. A significant, time-dependent induction of matrix metalloproteinase 1 (up to 2.4-fold after 48 h) and matrix metalloproteinase 3 (up to 4.3-fold after 48 h) protein levels was seen after 5-aminolevulinic acid-photodynamic therapy. Irradiation with ultraviolet A light, used as a positive control, showed a similar induction of matrix metalloproteinase 1 (2.3-fold after 48 h). The mRNA levels of matrix metalloproteinase 1 and matrix metalloproteinase 3 were significantly increased 12 h after irradiation, whereas collagen type I mRNA was strongly decreased already 6 h following irradiation. Collagen type III, tissue inhibitor of metalloproteinase 1, and matrix metalloproteinase 2 did not change after photodynamic therapy. Addition of nontoxic concentrations of sodium azide, a singlet-oxygen quencher, significantly inhibited induction of matrix metalloproteinase 1 by 5-aminolevulinic acid and light. These data show that 5-aminolevulinic acid and light induce matrix metalloproteinase 1 and matrix metalloproteinase 3 expression in normal and scleroderma fibroblasts in a singlet oxygen-dependent way while reducing collagen type I mRNA expression. Induction of collagen-degrading enzymes together with reduction of collagen production might be responsible for the anti-sclerotic effects of 5-aminolevulinic acid-photodynamic therapy observed in vivo.

Aminolevulinic Acid↗

PubMedDate not supplied

Effect of penicillin G on corium thickness in linear morphea of childhood: An analysis using ultrasound technique.

Linear morphea is the most common form of localized scleroderma in children. The pathogenesis of this disorder is unknown. We report a child with a 6-year history of linear morphea in whom intravenous administration of 5 MU aqueous penicillin G three times a day for 10 consecutive days caused reduction in the thickness of the corium as demonstrated by ultrasound measurements.

Child↗

PubMed2002

Non-oncologic indications for ALA-PDT.

Topical photodynamic therapy with 5-aminolevulinic acid (ALA-PDT) is a well-established treatment regimen for superficial epithelial skin tumours, but it is evident that inflammatory diseases of the skin and virus-induced lesions can also profit from PDT. Depending on the light dose applied, either cytotoxic effects resulting in tumour destruction or immunomodulatory effects resulting in improvement of inflammatory conditions occur. Patients with localized scleroderma that had been unresponsive to various treatments, including PUVA or bath-PUVA therapy, respond very well to topical ALA-PDT performed repeatedly. In contrast to PUVA therapy, no carcinogenic potential is being discussed for PDT. Also, HPV-induced skin lesions might provide a possible indication for topical ALA-PDT. The rapidly proliferating cells in viral acanthomas accumulate ALA-induced protoporphyrin IX (PPIX) selectively when compared to the surrounding non-infected cells. The efficacy of topical ALA-PDT in the treatment of recalcitrant foot and hand warts has been shown in a placebo-controlled, randomized, double-blind trial. Furthermore, case reports describe a good response of other virus-induced diseases, for example condylomata acuminata and epidermodysplasia verruciformis, to topical PDT with ALA. However, controlled clinical trials are still needed to demonstrate more fully the effectiveness of PDT for inflammatory skin diseases.

Aminolevulinic Acid↗

PubMed1999

Morphea limited to the superficial reticular dermis: an underrecognized histologic phenomenon.

Morphea (localized scleroderma) is a disease of unknown etiology, presenting as circumscribed areas of indurated skin. Histologically, most cases of morphea feature thickened collagen bundles in the deep reticular dermis, sometimes also extending into the superficial dermis or into the subcutis. We present six cases of morphea in which typical histologic features were restricted to the superficial dermis and contrast these with 27 additional biopsies of conventional morphea seen during the same time period. Sections were stained for elastic fibers, and dermal dendritic cells were labeled with antibodies to CD34 and Factor XIIIa. All six cases showed thickened collagen bundles restricted to the superficial dermis, sparing the deep dermis and without associated evidence of lichen sclerosus et atrophicus (LSA). Dermal elastic fibers were not appreciably decreased in number. There was loss of CD34-positive dermal spindle cells in each of our six superficial examples of morphea, which was restricted to the area of altered collagen in four of the six cases. This report highlights the distinctly uncommon phenomenon of morphea presenting solely as alteration of the superficial reticular dermis, without features of LSA. The selective loss of CD34-labeled spindle cells may provide information regarding the role of these putative immune accessory cells in morphea. Recognition of this manifestation of morphea may be helpful diagnostically.

Adult↗

PubMed1999

A case of generalized morphea with a high titer of anti-Borrelia burgdorferi antibodies.

A 69-year-old male had noticed pruritus on the back for the previous 3-4 years and cutaneous sclerosis with swelling of the dorsum of the neck had developed in the last one and a half years. However, he had never complained of Raynaud's phenomenon of the fingers, dry mouth, or dry eyes. At this first visit to our hospital, he complained of erythematous cutaneous sclerosis with swelling of the dorsum of the neck. Histopathological findings biopsied from the neck showed epidermal hyperplasia with elongation of rete ridges and homogeneous and fibromatous changes of the dermis with dense perivascular cell infiltration consisting of mononuclear cells or lymphocytes with several nests of incontinentia pigmenti. However, there were no sclerotic changes in blood vessels in the upper dermis biopsied from the forearm skin, although slightly homogeneous and fibromatous changes of the dermis were seen. In the clinical course, the cutaneous sclerotic change enlarged to extend to the bottom of the cheek, forearm, and lower legs. These clinical features and histopathological findings led to the diagnosis of generalized morphea. Hematologic examination showed positive anti-Borrelia burgdorferi IgM antibodies, although there were no positive anti-Borrelia burgdorferi IgG antibodies. These results revealed that there can be a close association of localized scleroderma with Borrelia burgdorferi and that generalized morphea may also represent a Borrelia infection.

Aged↗

PubMed2003

Pansclerotic morphea treated with UVA: a case report.

We present an 8-year-old girl with pansclerotic morphea of childhood, a rare, severe variant of localized scleroderma. Various systemic agents and PUVA are used in the treatment of this disease. We used UVA therapy in the treatment of this case.

Child↗

PubMed1979

Diameter of the collagen fibrils in the sclerodermatous skin of porphyria cutanea tarda.

The diameter of collagen fibrils was studied in a patient with porphyria cutanea tarda in a specimen obtained from the sclerodermatous skin of the back. A bimodal distribution of the diameter of the fibrils was observed with maxima at 33.5 nm and 68.7 nm. The mean diameter was 67 nm with a standard deviation of 10.6 nm. These results are similar to those obtained previously in localized scleroderma (morphoea).

Aged↗

PubMed1993

Solitary fibrosing paraspinal plaque: solitary morphoea profunda?

Solitary morphoea profunda is a recently described morphological variant of localized scleroderma. Two of the five reported patients had a solitary fibrotic plaque in the paraspinal region. We report a 16-year-old boy with a solitary fibrotic paraspinal plaque which, on histological examination, showed dermal and subcutaneous sclerosis, with a polymorphous infiltrate including plasma cells and eosinophils. Laboratory tests were either negative or normal, except for mild peripheral blood eosinophilia. Antinuclear antibodies were not detected, and the patient had no evidence of Borrelia infection. Magnetic resonance imaging (MRI) showed that the process involved the subcutaneous tissue, but did not extend to the underlying bone. As half of the patients with this entity (three of six) now described have had a solitary fibrotic plaque in a paraspinal location, we suggest that it may be premature to classify all these cases as being a variant of morphoea. We propose the use of the descriptive term solitary fibrosing paraspinal plaque, until the aetiology or aetiologies of this condition are better understood.

Adolescent↗

PubMedDate not supplied

Histologic observations of pleomorphic, variably acid-fast bacteria in scleroderma, morphea, and lichen sclerosus et atrophicus.

Variably acid-fast coccoid forms, suggestive of cell wall deficient forms of mycobacteria, were observed in the dermis in microscopic sections of skin from six patients with generalized scleroderma, 10 patients with localized scleroderma (morphea), and four patients with lichen sclerosus et atrophicus (LSA). These coccoid forms were found within the collagen bundles, around the adnexae (hair shafts, pilosebaceous units, eccrine glands), and less commonly around the blood vessels and nerves. These coccoid forms may be related to cocci and also to granular coccoid elements of corynebacteria-like coccobacilli, which, on occasion, can be cultured from the skin of these three diseases. The findings in this study support the three-decade old hypothesis concerning the constant association of pleomorphic acid-fast bacteria with scleroderma. The study also suggests that closely related diseases, such as morphea and LSA, are also associated with the presence of similar appearing microbes.

Adolescent↗

PubMed1996

Pseudoscleroderma secondary to phytomenadione (vitamin K1) injections: Texier's disease.

Cutaneous reactions to vitamin K1 (phytomenadione) are uncommon. They can present as acute eczematous reactions or late reactions that resemble localized scleroderma after vitamin K1 injections. A case is reported here of a patient who developed bilateral sclerodermoid plaques in a cowboy's holster pattern, which persisted for more than 10 years after subcutaneous vitamin K1 injections. Positive intradermal test with vitamin K1 that persisted as an erythematous indurated plaque at the test site for more than 5 months confirmed marked cutaneous hypersensitivity to vitamin K1 in this patient. Serial biopsies of the erythematous plaque at the test site showed transition from spongiotic eczematous features initially to inflammatory morphoea-like histology over a 5 month period. Possible pathogenic mechanisms for phytomenadione-induced pseudoscleroderma are discussed.

Aged↗

PubMed1991

Solitary morphea profunda in a 5-year-old girl: case report and review of the literature.

A 5-year-old girl had a solitary sclerotic plaque on the back of recent onset. The histopathologic features were consistent with morphea profunda. Thickening and homogenization of collagen bundles were demonstrated in the dermis and subcutaneous tissues, admixed with a prominent lymphocytic and plasma cell inflammatory infiltrate. Solitary morphea profunda is a variant of localized scleroderma that has not been reported previously in childhood. Cases described in the literature as nodular or keloid morphea may represent a similar entity.

Child, Preschool↗

PubMedDate not supplied

Dermatofibrosarcoma protuberans in childhood.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a possibly misleading presentation. It is rare in childhood. We report an 18-month-old child with an acquired atrophic plaque of the left ankle. Based on clinical findings, initial proposed diagnoses included localized scleroderma and localized lipoatrophy. At the age of 5 years the plaque had increased in size and become a purplish color. Several firm nodules were seen at its periphery. A diagnosis of DFSP was proposed and confirmed histologically. A large surgical resection was performed. This case illustrates that DFSP may occur in childhood, during which initial diagnosis is generally more difficult than in adulthood.

Age Factors↗

PubMed1996

Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases.

Morphea (localized scleroderma), and lichen sclerosus et atrophicus (LSA) share common features with acrodermatitis chronica atrophicans (ACA), a known chronic form of borreliosis. These include similar histologic findings such as diffuse dermal fibrosis. These observations have led several investigators to consider the possibility of Borrelia burgdorferi (Bb) as a common etiologic factor among all of these diseases. The aim of this study is to investigate the role of Bb in the pathogenesis of morphea and LSA, by assaying for its presence in lesional skin biopsies from patients with these diseases. We utilized the nested polymerase chain reaction (PCR) technique to selectively amplify a longer segment of a Bb-specific somatic gene, on DNA from paraffin-embedded, formalin-fixed tissues. The results revealed no Bb-specific DNA sequence in 28 specimens of morphea/scleroderma and 7 of LSA with varying stages of disease. Furthermore, confirmatory Southern blot of the PCR product, resulted in similar findings. These data seriously question the role played by this spirochete in the pathogenesis of morphea and LSA, at least in the southeastern part of the USA.

Adult↗

PubMed1995

Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea.

BACKGROUND: There are divergent opinions whether atrophoderma Pasini-Pierini (APP) is a nosologic entity or a primary atrophic morphea. OBJECTIVE: Since usually single cases are reported without a long-term follow-up the present study was performed in order to elucidate the natural history of the disorder. METHODS: We followed a large series of 139 patients, 91 adults and 48 children, for 4-30 years (mean over 10 years). RESULTS: APP was found to be 6 times more frequent in females and not uncommon in children (10% of our series of localized scleroderma). At some time during the follow-up period, indurations appeared in the central parts of the lesions in 17% of the patients, and in 22% they coexisted with morphea plaques outside the atrophies. The histological pattern was similar to morphea at the stage of atrophy. No case developed full-blown morphea. CONCLUSION: APP appears to be an abortive morphea, in which the indurations failed to develop. The differentiation from morphea is of practical importance because of different management and prognosis.

Adult↗

PubMed1992

Vitiligo-like depigmentation and morpheas after specific intralymphatic immunotherapy for malignant melanoma.

We report the case of a patient with stage 2 malignant melanoma (MM), who received a specific immunotherapy consisting of intralymphatic injections of irradiated MM cells. She developed subsequently vitiligo-like leukoderma and several plaques of localized scleroderma. Simultaneously an increase in the patient's cytotoxic activity against MM cells was detected in vitro. The responsibility of immune phenomena and specific immunotherapy for the appearance of depigmentation and morpheas is discussed.

Aged↗

PubMed2003

Generalized morphea.

A 69-year-old man presented with symmetric erythematous, hyperpigmented, and ivory-colored plaques on the trunk and extremities. His clinical history, laboratory analysis, and histopathologic examination were consistent with generalized morphea, a subtype of localized scleroderma.

Aged↗