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We report two patients who developed localized scleroderma-like lesions on the legs following bone marrow transplantation. These changes were associated with a polyneuropathy in the same distribution.
We report a case of the linear type of lichen sclerosus et atrophicus (LSA). The patient had linear atrophic lesions from the left upper back to the left hand. Histological findings obtained from the upper back were those of typical LSA. Histology of the forearm showed mild changes suggestive of LSA. There were no histological features suggesting any association with localized scleroderma in either specimen. The relation between LSA and localized scleroderma is unclear at present. This case suggests that there is a linear form in LSA as already recognized in localized scleroderma.
Blood flow measurements by laser-Doppler flowmetry were performed on 15 patients with localized scleroderma (morphoea). Thirty morphoea plaques were studied by means of measurements of the sclerotic centre, the perilesional area, and regional control of the normal-appearing skin of the individual patients. Blood flow was increased (P less than 0.01) in the sclerotic area of all patient material and in the 10 patients having one or a few plaques (P less than 0.01). Five patients with generalized morphoea showed the same tendency. Blood flow was also increased in the perilesional area (P less than 0.01) of the total material, and in the patients with one or a few plaques (P less than 0.05), particularly in the case of a 'lilac ring'. 133Xenon washout measurements in 3 patients showed parallel results. The blood flow of plaques with clinically 'advanced' scleroderma was higher (P less than 0.01) as compared to plaques with 'slight' scleroderma. Measurements in pigmented spots after previous morphoea showed normal blood flow.
Synthesis of collagen and non-collagenous proteins was measured in fibroblast cultures derived from different layers of the dermis from a patient with an early stage of localized scleroderma. Increased synthesis of collagen was found in fibroblasts grown from the subcutaneous fat of this patient, whereas cells obtained from the papillary dermis revealed normal metabolism. These data agree with the results obtained in previous experiments with cells derived from patients with progressive systemic sclerosis in primary culture, thus indicating that the two diseases have a common pathomechanism. Several subcultures of the activated fibroblast populations were also studied. Normal collagen synthesis in these cultures was observed after the fifth passage, probably indicating selection of cell populations or loss of the previous phenotype.
The natural history of the clinical and pathologic features of skin disease was reviewed prospectively in 30 patients with the L-tryptophan-associated eosinophilia-myalgia syndrome. Overall, cutaneous manifestations developed in 26 patients (87%). Early lesions were nonspecific and characterized predominantly by an erythematous macular eruption on the trunk and extremities. The most characteristic abnormality noted was the spectrum of sclerodermatous disease in 15 patients (50%) often after a subacute stage of peripheral or truncal edema. Clinical and/or biopsy evidence of eosinophilic fasciitis was seen in nine patients (30%). Findings consistent with diffuse, limited, or localized scleroderma were subsequently observed in nine patients (33%). Small mucinous papules, similar to those seen in scleromyxedema, were found in five patients (17%). Alopecia, frequently a late sequela, developed in 11 (37%). Common histologic features included papillary dermal fibrosis, dermal and fascial infiltrates consisting of mononuclear cells and eosinophils, deposition of glycosaminoglycans in the dermis, and, in some patients, numerous mast cells.
Morphea, or localized scleroderma, of the eyelids is an uncommon disease. Morphea usually involves the thorax, trunk, lower and upper extremities, face, and genitalia. In the present report a patient with a biopsy-proven morphea of both upper eyelids is described. The salient histopathologic features included thinning of the epidermis with thickening and sclerosis of the collagen fibers in both the papillary and reticular dermis. There was a marked decrease in the fibrocytes. The eccrine sweat glands were entrapped by sclerotic collagen fibers. The pilosebaceous units were markedly decreased in number. There was a moderate lymphocytic infiltration in the dermis and a prominent lymphocytic perivasculitis. The clinical and histopathologic features of morphea are compared with those of lichen sclerosus et atrophicus.
Twenty-six cases of enophthalmos were reviewed. The causes in order of frequency were: orbital asymmetry (8); trauma (5); orbital metastasis (4); microphthalmos (2); orbital varix (2); maxillary mucocele (2); localized scleroderma (1); absence of sphenoid wing (neurofibromatosis) (1); post irradiation atrophy (1). Only six of the patients (23%) were referred with the diagnosis of enophthalmos, suggesting the sign maybe subtle and is frequently missed or misdiagnosed. The nature of the causes underscore the need for careful and thorough diagnosis. In particular, the therapeutic implications of diagnosing metastatic disease, maxillary mucocele, and orbital varices is noted. A review of etiology and mechanisms of enophthalmos point to the diversity, importance and conditions causing this sign.
A patient with generalized morphea and thrombocytopenia is reported. The thrombocytopenia responded promptly to corticosteroid and immunosuppressive therapy but recurred when the steroids were discontinued. The occurrence of thrombocytopenia in generalized morphea suggests a common immune mechanism and emphasizes the need to look for concomitant autoimmune hematologic disorders in patients with systemic, as well as localized, scleroderma.
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).
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.
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.
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.
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.
Specific (usually papules and nodules showing a granulomatous histology) and non-specific (e.g. erythema nodosum) cutaneous lesions presenting manifestations of sarcoidosis have been well described. Two patients with unique presentations are here described; one with ichthyosiform cutaneous lesions and one with not previously described cutaneous lesions which mimicked linear morphea (localized scleroderma). The association of articular manifestations without pulmonary involvement was also unusual.
The most important morphological aspects and pathogenesis of necrobiosis lipoidica concerning histological and clinical aspects are reported. In case of diabetes we find more frequently necrobiosis lipoidica localized out of the shank than in cases of necrobiosis lipoidica without diabetes. Necrobiosis lipoidica must be differentiated from granulomatosis disciformis, localized scleroderma and atrophy of the skin of another origin. Beside normalization of diabetes and application of corticosteroids physical and surgical treatment is recommended.
The fascia had received little attention until Shulman's delineation of EF. Evidence is now accumulating that in addition to EF and scleroderma, significant fascial inflammation may be seen in polymyositis, dermatomyositis, eosinophilic polymyositis, systemic lupus erythematosus, and mixed connective tissue disease. It is still unclear whether EF represents a variant of scleroderma; however, it is becoming increasingly recognized that scleroderma shares many features in common with EF including eosinophilia, hypergammaglobulinemia, positive ANA and rheumatoid factor, and an association with hematologic disease. The rarity of Raynaud's phenomenon and significant visceral changes help distinguish EF from systemic scleroderma. In this regard, however, EF more closely resembles the localized scleroderma syndromes, especially morphea profunda and pansclerotic morphea. Biopsy in EF, systemic scleroderma, and localized scleroderma will show comparable changes, the essential difference being the levels at which they occur.
A case of facial hemiatrophy and homolateral cervical band of scleroderma, complicated by hypothyroidism is reported. This case raises two problems: one is the problem of distinction between Romberg's disease and facial hemiatrophy due to a genuine localized scleroderma; the other concerns the relationship between localized scleroderma and dysthyroidism. The generalized scleroderma-dysthyroidism association has now been recognized, but the coexistence of thyroid disease and localized scleroderma has not yet been reported. Several pathogenetic hypotheses on this association are discussed.
Four patients with systemic scleroderma and 1 patient with localized scleroderma were treated with ciclosporin (CS), given in daily doses between 2.2 and 5.6 mg/kg body weight for 3-26 months. Under this medication clinical improvement was observed in 4 patients with partial regression of cutaneous sclerosis and inflammation, healing of fingertip ulcerations or leg ulcers and improvement of articular mobility. However, in 1 patient with rapidly advancing systemic scleroderma a short-term therapy with CS in low doses (2-3 mg/kg body weight) resulted in arterial hypertension and renal dysfunction. Therefore careful selection of patients and close-meshed controls are indicated when CS is considered as anti-inflammatory treatment in scleroderma.