The second case of this serial reports under the title of unusual cutaneous manifestations of connective tissue diseases is a woman with primary idiopathic dermatomyositis who developed cutaneous ulcerations on her arms and legs, which is thought to b a rare manifestation of adult dermatomyositis. These multiple ulcerations are about bean sized, polymorphous, and grouped on the poikilodermatous lesional skin. In this patient these ulcerative lesions became regressive and healed with disfiguring scars after the activity of the disease has controlled by prednisolone and methotrexate.
Adult ; Arm ; Cicatrix ; Connective Tissue Diseases* ; Connective Tissue* ; Dermatomyositis* ; Female ; Humans ; Leg ; Methotrexate ; Prednisolone ; Skin ; Ulcer*

Localized scleroderma is a connective tissue disorder characterized by thickening and fibrosis of the skin due to excessive collagen deposition in the dermis. Dystrophic calcinosis occurs when calcium is deposited in the previously damaged skin tissue, and calcium and phosphorous levels in the blood are within normal limits. It occurs secondary to connective tissue disease, infection, inflammatory processes, chronic venous stasis, cutaneous neoplasm, and trauma. We report a rare case of dystrophic calcinosis cutis with transepidermal elimination secondary to localized scleroderma.
Calcinosis ; Calcium ; Collagen ; Connective Tissue ; Connective Tissue Diseases ; Dermis ; Fibrosis ; Scleroderma, Localized ; Skin

Congenital cutis laxa is a rare inherited disorder of connective tissue manifested by loose and hanging skin, resulting in a prematurely-aged appearance. Cutis laxa may be inherited in an autosomal dominant, autosomal recessive and X-linked recessive pattern or an acquired form. In this report we describe a patient with congenital cutis laxa and growth retardation. The skin showed loose folds, wrinkling, and sagging of the face, abdomen, and both thighs. His weight and height were below the 3rd percentile. He had no family history for this skin disease.
Abdomen ; Connective Tissue ; Cutis Laxa* ; Elastic Tissue ; Humans ; Skin ; Skin Diseases ; Thigh

Congenital cutis laxa is a rare inherited disorder of connective tissue manifested by loose and hanging skin, resulting in a prematurely-aged appearance. Cutis laxa may be inherited in an autosomal dominant, autosomal recessive and X-linked recessive pattern or an acquired form. In this report we describe a patient with congenital cutis laxa and growth retardation. The skin showed loose folds, wrinkling, and sagging of the face, abdomen, and both thighs. His weight and height were below the 3rd percentile. He had no family history for this skin disease.
Abdomen ; Connective Tissue ; Cutis Laxa* ; Elastic Tissue ; Humans ; Skin ; Skin Diseases ; Thigh

Ehlers-Danlos syndrome (EDS) is an inherited connective tissue disorder characterized by skin hyperelasticity, joint hypermobility and tissue fragility. The clinical manifestations vary depending on the type of disease. Due to multi-organ involvement and varied clinical presentations, no uniform anesthetic recommendations can be made. We report a case of a patient with EDS of an unknown type who successfully underwent an emergency cesarean section under general anesthesia.
Anesthesia* ; Anesthesia, General ; Cesarean Section* ; Connective Tissue ; Connective Tissue Diseases ; Ehlers-Danlos Syndrome* ; Emergencies ; Female ; Humans ; Joint Instability ; Pregnancy ; Skin

Morphea is an autoimmune condition characterized by excessive accumulation of collagen in the skin and underlying tissues. Lichen sclerosus (LS) is another connective tissue disease for which an autoimmune cause has been proposed, given the high association with other autoimmune diseases. The coexistence of morphea and lichen sclerosus has been sometimes reported in the literature, and is suggestive of a common pathogenic background between the two diseases. Among various types of morphea, generalized morphea has been associated with an increased rate of autoimmune disease, including LS. We report a case of extragenital LS during the progression of linear morphea into generalized morphea.
Autoimmune Diseases ; Collagen ; Connective Tissue Diseases ; Lichen Sclerosus et Atrophicus* ; Lichens* ; Scleroderma, Localized* ; Skin

The calcinosis, dystrophic soft tissue calcification, occurs in damaged or devitalized tissues normal calcium/phosphorus metabolism. It is the subcutaneous tissues of connective tissues disease ?primarily systemic lupus erythematosus, scleroderma, or dermatomyositis - and may involve a relatively localized area. The calcinotic accumulations may result in muscle atrophy, joint contractures, and skin ulceration complicated by recurrent episodes of local inflammation and infection. Calcinosis may be the source of both pain and disability in connective tissue disease patients. While various therapeutic modality have been used, no treatment has convincingly prevented or reduced calcinosis. We report two cases of calcinosis cutis combined with rheumatic disease.
Calcinosis* ; Connective Tissue ; Connective Tissue Diseases ; Contracture ; Dermatomyositis ; Humans ; Inflammation ; Joints ; Lupus Erythematosus, Systemic ; Metabolism ; Muscular Atrophy ; Rheumatic Diseases ; Skin Ulcer ; Subcutaneous Tissue

Scleroderma is a connective tissue disease characterized by excessive accumulation of collagen in skin and visceral organs due to increased collagen production by scleroderma fibroblasts. The basic etiology of this collagen accumulation is not known. We examined the expression of various extracellular matrix genes in cultured fibrolasts using Northern blot and slot-blot hybridization. The scleroderma fibroblasts exhibited characteristic mRNA size of extracellular matrix genes and prominanty increased type I and III procollagen mRNAs levels compared to control fibroblasts cultures from univolved skin. The ratios of type I /IE procollagen in scleroderma cell lines were not so much different to the controls. These results indicate that increases of collagen biosynthesis in scleroderma can be a accounted for, at least in part, by an increased content of transcriptable type I and type JE procollagen mRNAs, both.
Blotting, Northern ; Cell Line ; Collagen ; Connective Tissue Diseases ; Extracellular Matrix ; Fibroblasts* ; Procollagen ; RNA, Messenger ; Skin

Generalized granuloma annulare (GGA) is a rare benign granulomatous dermatosis characterized by disseminated necrobiotic dermal papules. Histologically, it presents as a lymphohistiocytic granuloma associated with varying degrees of connective tissue degeneration. It usually occurs in adults and rarely affects infants. Herein, we report an interesting case of GGA which occurred in a 3 month-old girl in association with Bacillus Calmette-Guerin vaccination.
Adult ; Bacillus ; BCG Vaccine ; Connective Tissue ; Granuloma ; Granuloma Annulare ; Humans ; Infant ; Skin Diseases ; Vaccination

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon skin eruption associated with immune-mediated connective tissue diseases such as rheumatoid arthritis, lupus erythematosus, and Behcet's disease. This condition is also described as Winkelmann's granuloma, linear subcutaneous bands, rheumatoid papules, rheumatoid neutrophilic dermatitis, interstitial granulomatous dermatitis with cutaneous cords and arthritis, or Churg-Strauss disease. PNGD shows variable clinical manifestations and is characterized histopathologically by step pattern of early leukocytoclastic vasculititis, progressing to form palisaded granulomatous inflammation, and surrounding zones of necrobiosis in the later stages. We report a 66-year-old woman with multiple skin nodules, who had concomitant rheumatoid arthritis. Histopathologic findings revealed palisaded neutrophilic and granulomatous dermatitis.
Aged ; Arthritis ; Arthritis, Rheumatoid ; Connective Tissue Diseases ; Dermatitis ; Female ; Granuloma ; Humans ; Inflammation ; Necrobiotic Disorders ; Neutrophils ; Skin