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Objective: To compare the frequency of genital lichen sclerosus (LS) in patients with morphea with that of control patients., Design: A prospective multicenter study., Setting: Four French academic dermatology departments: Strasbourg, Montpellier, Tenon Hospital Paris, and Henri Mondor Hospital Créteil., Patients: Patients were recruited from November 1, 2008, through June 30, 2010. Seventy-six patients with morphea and 101 age- and sex-matched controls, who underwent complete clinical examination, were enrolled., Interventions: A complete clinical examination and, if deemed necessary, a cutaneous biopsy., Main Outcome Measure: The frequency of genital LS., Results: There were 58 women and 18 men (a 3:1 ratio) with a median age of 59 years. Mean (range) age at diagnosis was 54 (13-87) years. Forty-nine patients had plaque morphea, 9 had generalized morphea, and 18 had linear morphea. Three patients (3%) in the control group and 29 patients (38%) with morphea had LS (odds ratio, 19.8; 95% CI, 5.7-106.9; P < .001). Twenty-two patients with plaque morphea (45%) and only 1 patient with linear morphea (6%) had associated genital LS., Conclusions: Genital LS is significantly more frequent in patients with morphea than in unaffected individuals. Forty-five percent of patients with plaque morphea have associated LS. Complete clinical examination, including careful inspection of genital mucosa, should therefore be mandatory in patients with morphea because genital LS bears a risk of evolution into squamous cell carcinoma and thus needs treatment with topical corticosteroids.

Objective: To examine the level of psychological distress and factors contributing to distress in patients with morphea or eosinophilic fasciitis., Design: Cross-sectional study., Setting: Dermatology outpatient clinic of a university hospital., Participants: Of 120 patients with morphea or eosinophilic fasciitis diagnosed between December 1, 1994, and July 15, 2007, who were enrolled in the study, only 74 completed questionnaires were suitable for data analysis., Main Outcome Measures: Self-reported responses on the Impact of Chronic Skin Diseases on Daily Life scale measure psychological distress, specifically anxiety and depressed mood., Results: Psychological functioning was generally impaired in patients with skin disease, particularly among patients with generalized morphea and eosinophilic fasciitis. Twenty-eight patients (38%) were at risk of depression or anxiety. Higher levels of psychological distress were significantly related to greater severity of skin disease; more pain and fatigue; impact of disease on daily life; more perceived stigmatization; illness cognitions of greater helplessness; and less acceptance and less perceived social support., Conclusions: Physical and psychosocial aspects play a substantial role in the quality of life for patients with morphea. Physicians should be encouraged to assess the physical and psychosocial factors when treating patients with sclerotic skin diseases. This approach could improve quality of life and ultimately lead to improved dermatological treatment outcomes.

Objective: To determine the prevalence of extracutaneous manifestations and autoimmunity in adult and pediatric patients with morphea., Design: A retrospective review of 245 patients with morphea., Setting: University of Texas Southwestern Medical Center-affiliated institutions. Patients Patients with clinical findings consistent with morphea., Main Outcome Measures: Prevalence of concomitant autoimmune diseases, prevalence of familial autoimmune disease, prevalence of extracutaneous manifestations, and laboratory evidence of autoimmunity (antinuclear antibody positivity). Secondary outcome measures included demographic features., Results: In this group, adults and children were affected nearly equally, and African Americans were affected less frequently than expected. The prevalence of concomitant autoimmunity in the generalized subtype of morphea was statistically significantly greater than that found in all other subtypes combined (P = .01). Frequency of a family history of autoimmune disease showed a trend in favor of generalized and mixed subgroups. The linear subtype showed a significant association with neurologic manifestations, while general systemic manifestations were most common in the generalized subtype. Antinuclear antibody positivity was most frequent in mixed and generalized subtypes., Conclusions: High prevalences of concomitant and familial autoimmune disease, systemic manifestations, and antinuclear antibody positivity in the generalized and possibly mixed subtypes suggest that these are systemic autoimmune syndromes and not skin-only phenomena. This has implications for the management and treatment of patients with morphea.

Objectives and Design: In vivo capillaroscopic examination was performed on patients with localized scleroderma to determine whether nailfold capillary abnormalities seen in systemic scleroderma (systemic sclerosis) were also present in the localized form. Twenty-seven patients (24 women, three men) were examined by this technique., Results: Only two patients exhibited scleroderma-type nailfold capillary abnormalities similar to those seen in systemic sclerosis. Both patients also suffered from Raynaud's phenomenon and showed evidence of coexisting systemic sclerosis, one on first examination, the other 1.5 years later. Our results are compared with earlier studies reporting such rare coexistence of the two forms of scleroderma. Earlier capillaroscopic work in this disorder is also reviewed., Conclusions: These results suggest that the presence, in a patient with localized scleroderma, of nailfold capillary abnormalities similar to those seen in systemic sclerosis should alert the physician to a possible association with systemic sclerosis.

Histological studies using three lectins, lens culinaris agglutinin, soybean agglutinin, and Ulex europaeus agglutinin-I, were carried out in a case of coexistent lichen sclerosus et atrophicus and morphea, five cases of morphea, and two cases of lichen sclerosus et atrophicus. The lectin staining patterns of the formaldehyde-fixed epidermis of patients with morphea were not different from those of normal epidermis, but epidermis of patients with lichen sclerosus et atrophicus showed different staining patterns. Lens culinaris agglutinin stained the basal and the spinous layers of the normal epidermis and that of patients with morphea but stained only the basal cells of the epidermis from patients with lichen sclerosus et atrophicus; epidermal Ulex europaeus agglutinin binding was observed only in the cases of lichen sclerosus et atrophicus. Moreover, in the patient with coexistent diseases, the morphea lesion showed the staining profiles of morphea and the lichen sclerosus et atrophicus lesion showed the staining patterns of lichen sclerosus et atrophicus, respectively.

Six patients with well-circumscribed, indurated, inflammatory plaques of the lower extremity are described. Incisional biopsy specimens in each patient revealed fat necrosis, sclerosis, and a lobular panniculitis. These patients are similar to those described under the designations hypodermitis sclerodermaformis or lipodermatosclerosis. The name sclerosing panniculitis is proposed to unify these conditions. The pathogenesis is unknown, although a role for venous stasis or previous thrombophlebitis has been proposed. Systemic or local anti-inflammatory therapies have not been clinically effective.

Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.

A subcorneal pustular dermatosis and a vesicobullous eruption with the clinical ad histologic features of localized bullous pemphigoid(BP) were observed in a 44-year-old woman with morphea and a recent history of phenytoin sodium-induced toxic epidermal necrolysis. Localized BP is rare and has been previously described in association with other cutaneous disorders. The coexistence of localized BP, morphea, and subcorneal pustulosis suggests that immunologic factors may play a role in all three conditions.

Two patients had a distinctive variant of localized scleroderma. Both have a history of sclerodermatous changes of the skin over the face developing relatively late in life and accompanied by hair loss, cutaneous calcification, and prominent beaking of the nose. A striking lack of systemic involvement also was noted.

In this article, we describe a patient with progressive localized scleroderma, who subsequently developed pemphigus vulgaris on the previously affected sclerodermatous skin, and on the oral mucosa, the nasal mucosa, the laryngeal mucosa, and the scalp. The coexistence of pemphigus vulgaris with localized scleroderma and the histopathologic documentation of pemphigus vulgaris arising on a sclerodermatous skin have not been previously reported. Whether this is a random association or an association based on common immunologic pathomechanisms in pemphigus and localized scleroderma may be important in understanding these diseases.

During an 18-month period, four patients with scleroderma were found to have nail findings suggestive or pterygium inversum unguis, a recently described condition. In the same time period, two other examples of the disorder were seen. Pterygium inversum unguis-like change may represent an additional nail sign in scleroderma. There are possible mechanisms for its development in patients with Raynaud's phenomenon, connective tissue diseases, or peripheral vascular disease.

On the basis of clinical features and histological findings, cutaneous manifestations of linear melorheostotic scleroderma are apparently derived from a localized proliferative disorder and are not secondary to bone changes. If the hypertrichosis has the same origin as the osteocutaneous changes, melorheostosis may represent a congenital disorder with both ectodermic and mesodermic components.

A 56-year-old woman with generalized morphea was treated with penicillamine. Four months after commencing treatment, she developed an eruption clinically, histologically, and immunopathologically consistent with pemphigus. The eruption disappeared following withdrawal of the drug. It is suggested that penicillamine initiated epidermal damage.

Two mentally retarded siblings, one with severe segmental scleroderma and the other with atrophoderma of Pasini and Pierini, were found at the ages of 6 and 10 years to have phenylketonuria (PKU). The belief that a common pathomechanism exists between morphea and atrophoderma of Pasini and Pierini is supported by the case of the two siblings. Disorders in tryptophan metabolism can occur in both PKU and scleroderma. For a low phenylalanine diet to be effective in PKU, it has to be instituted at an early age. Phenylketonuria should be considered in infants and children with sclerodermatous skin lesions.

A 32-year-old man suffering from linear frontoparietal scleroderma was found to have low (less than 10% normal) serum classical pathway activity although C1q, C3, C4, C5, and total alternative pathway activity was normal. Addition of purified C2 led to complete restoration of the total hemolytic activity of the classical pathway. The C2 hemolytic assays showed that the patient was not totally deficient in C2. He had about 30% of the normal C2 level. Studies on his available nucleus family members in the Netherlands also showed that the deficiency was inherited; one of the patient's brothers and one of his daughters had half of the normal C2 levels. The C2 deficiency could not be corrected by a three-week regimen of danazol. To the best of our knowledge, this is the first documented case concerning an association of linear frontoparietal scleroderma with C2 deficiency.

Systemic scleroderma and localized scleroderma (morphea) show comparable changes on skin biopsy specimens, and a distinction has often been made on the basis of Raynaud's phenomenon, organ involvement, and laboratory abnormalities characteristically seen in systemic scleroderma. Critical evaluation not only of patients with localized scleroderma but also of those with eosinophilic fasciitis, morphea profunda, and acral pansclerotic morphea has disclosed Raynaud's phenomenon, organ involvement, and laboratory abnormalities typical of systemic scleroderma in a small percentage of patients. Histologically, all five conditions show similar inflammation and sclerosis of the skin, the primary difference being the depth at which these changes occur. These conditions may possibly be related, and the clinical and laboratory differences observed may result from variations in depth, nature, and intensity of the cutaneous and subcutaneous inflammatory sclerosis.

We treated a case of subcutaneous sclerosis after phytonadione injections (Texier's disease) that was noteworthy for two associated features: sclerosis with inflammatory infiltrate in the fascia, and blood eosinophilia. A peculiar type of cutaneous sclerosis after phytonadione injections was described in 1972. The sclerosis in that case was first confined to the buttocks, progressively extended to both thighs, and slowly resolved. In the 38 reported cases, neither sclerosis of the fascia nor blood eosinophilia was observed.

Two patients with lobular lymphohistiocytic panniculitis had chronic lesions that produced subcutaneous atrophy and that were responsive to antimalarial drugs. Massive lymphocytic infiltrate was associated with caseation necrosis of the fat lobules. In both patients, an unusual antibody to extractable nuclear antigen was seen, and antinuclear antibody was sometimes present. The unusual manifestation, course of the disease, serologic findings, and response to therapy suggest that the panniculitis is related to (but does not evolve into) lupus erythematosus panniculitis or subcutaneous morphea or both. A satisfactory term for this entity would be "connective tissue panniculitis."

Of 2,141 patients with scleroderma who were seen at the Mayo Clinic, Rochester, Minn, between 1959 and 1975, 78 had 87 internal malignancies. The relative frequency of the types of cancers was similar to that for the general population. The increased numbers of patients with breast and uterine carcinomas were consistent with the female preponderance in scleroderma. Contrary to previous reports, carcinoma of the lung was not the most frequent malignancy associated with scleroderma. The lymphoma-leukemia malignancies were second only to breast carcinoma in frequency, comprising about 17% of the malignancies. Both conditions developed within a three-year period in 68% (45/66) of our patients affected with systemic scleroderma, and this subgroup comprised a high mortality group. This study is not an epidemiologic one in the strictest sense, but it is a review of the experience of the Mayo Clinic with patients who have had both scleroderma and an internal malignancy.

We investigated 24 juvenile cases of linear scleroderma for the presence of systemic disease and serologic abnormalities. Thirteen of 24 patients had antinuclear antibodies (ANA) at titers of 1:40 or greater. Rheumatoid factor (titers greater than or equal to 1:20) was detected in seven of 17 patients tested, five of whom also had ANA. Two of five patients with ANA and rheumatoid factor had systemic diseases, such as nephritis and Raynaud's phenomenon. One patient with ANA developed typical dermatomyositis. Consequently, patients with linear scleroderma may be at some risk for developing systemic collagen-vascular diseases. On initial presentation, patients with linear scleroderma should give a complete history and receive a thorough physical examination as well as undergo laboratory evaluations for the presence of ANA and rheumatoid factor. Long-term observation with periodic reevaluation is appropriate for many members of this group.