Skin fibrosis associated with keloid, scleroderma and Jorge Lobo's disease (lacaziosis): An immuno-histochemical study.

Fibrosis is a common pathophysiological response of many tissues and organs subjected to chronic injury. Despite the diverse aetiology of keloid, lacaziosis and localized scleroderma, the process of fibrosis is present in the pathogenesis of all of these three entities beyond other individual clinical and histological distinct characteristics. Fibrosis was studied in 20 samples each of these three chronic cutaneous inflammatory diseases. An immunohistochemical study was carried out to explore the presence of α-smooth muscle actin (α-SMA) and vimentin cytoskeleton antigens, CD31, CD34, Ki67, p16; CD105, CD163, CD206 and FOXP3 antigens; and the central fibrotic cytokine TGF-ß. Higher expression of vimentin in comparison to α-SMA in all three lesion types was found. CD31- and CD34-positive blood vessel endothelial cells were observed throughout the reticular dermis. Ki67 expression was low and almost absent in scleroderma. p16-positive levels were higher than ki67 and observed in reticular dermis of keloidal collagen in keloids, in collagen bundles in scleroderma and in the external layers of the granulomas in lacaziosis. The presence of α-actin positive cells and rarely CD34 positive cells, observed primarily in keloids, may be related to higher p16 antigen expression, a measure of cell senescence. Low FOXP3 expression was observed in all lesion types. CD105-positive cells were mainly found in perivascular tissue in close contact with the adventitia in keloids and scleroderma, while, in lacaziosis, these cells were chiefly observed in conjunction with collagen deposition in the external granuloma layer. We did not find high involvement of CD163 or CD206-positive cells in the fibrotic process. TGF-ß was notable only in keloid and lacaziosis lesions. In conclusion, we have suggested vimentin to be the main myofibroblast general marker of the fibrotic process in all three studied diseases, while endothelial-to-mesenchymal transition (EndoMT) and mesenchymal stem cells (MSCs) and M2 macrophages may not play an important role.

Morphoea with prominent plasma cell endoneuritis.

Morphoea (localized scleroderma) is a cutaneous inflammatory condition characterized by the development of indurated and discoloured plaques. The histological features of morphoea typically include a superficial and deep perivascular and periadnexal chronic inflammatory infiltrate associated with variable degrees of dermal and/or subcutaneous sclerosis. The infiltrate is typically composed of lymphocytes, macrophages and conspicuous plasma cells. The early stages of morphoea may have a very prominent inflammatory infiltrate associated with subtle sclerosis. In addition, the inflammatory infiltrate may show a perineural and rarely intraneural distribution. We report two cases of morphoea that histologically showed plasma cell endoneuritis associated with subtle dermal sclerosis. These two cases highlight the potential for diagnostic confusion with infectious and inflammatory diseases, particularly leprosy and lupus.

Atypical presentations of eosinophilic fasciitis.

Eosinophilic fasciitis is an uncommon connective tissue disease that may mimic and overlap with other sclerosing disorders such as morphea and lichen sclerosus. Herein, we report four patients (two men and two women, aged 16-64 yeas) with eosinophilic fasciitis. There was overlap with both morphea and lichen sclerosus in 2 patients and with morphoea alone in 1 patient. Magnetic resonance imaging (MRI) was used for diagnosis in three patients and for assessing treatment response in one patient. Eosinophilic fasciitis may co-exist with morhoea and lichen sclerosus. In view of the overlapping clinical and histopathological features of these disorders, MRI may be helful in delineating the conditions by detecting involvement of fascia.

A Rare Combination of Pure Neuritic Leprosy with Morphea Leading to Diagnostic Confusion.

A circumscribed sclerotic plaque of morphea can sometimes be mistaken for tuberculoid leprosy and vice versa can also happen. However, the co-existence of a patch of morphea mimicking as Leprosy patch in an underlying case of neuriticleprosy, can be very misleading. We present a case with glove and stocking anaesthesia and peripheral nerve enlargement with a single large hypopigmented, non-anaesthetic macule on trunk, clinically diagnosed as Hansen's disease (Borderline Tuberculoid - BT). Slit skin smears proved to be negative for AFB and histopathology of the skin lesion was consistent with morphea, which lead us to do a nerve biopsy. Sural nerve biopsy proved it to be Hansen's neuritis with occasional bacilli. The patient was started on MDT-MB and followed up. This is a rare case of co-existing morphea with Hansen's disease. It would have been easily misclassified if we had presumed the cutaneous lesion to be a case of Hansen's (BT) patch and not done a cutaneous nerve biopsy which led to diagnosis of multibacillary leprosy.

Hutchinson-Gilford progeria syndrome.

Progeria is a rare genetic disorder characterized by premature aging, involving the skin, bones, heart, and blood vessels. We report a 4-year-old boy who presented with clinical manifestations of progeria. He had characteristic facies, prominent eyes, scalp and leg veins, senile look, loss of scalp hair, eyebrows and eyelashes, stunted growth, and sclerodermatous changes. The present case is reported due to its rarity.

Diagnostic significance of colloid body deposition in direct immunofluorescence.

BACKGROUND: Colloid bodies (CB) in direct immunofluorescence (DIF) studies are usually found in interface dermatitis. Furthermore, CB can be found in various skin diseases and even in normal skin. AIM: To evaluate the diagnostic value of CB deposits in DIF studies. METHODS: From 1996-2007, data from 502 patients where DIF studies showed immunoreactants at CB were enrolled. The definite diagnoses of these patients were based on clinical, histopathological and immunofluorescent findings. The results of DIF studies were analyzed. RESULTS: Immunoreactants at CB were detected in 44.4%, 43.8%, 4.2%, 3.8%, and 2.2% of interface dermatitis, vasculitis, autoimmune vesiculobullous disease, panniculitis, and scleroderma/morphea, respectively. The most common immunoreactant deposit of all diseases was Immunoglobulin M (IgM). Brighter intensity and higher quantity of CB was detected frequently in the group with interface dermatitis. CONCLUSIONS: Immunoreactant deposits at CB alone can be found in various diseases but a strong intensity and high quantity favor the diagnosis of interface dermatitis. CB plus dermoepidermal junction (DEJ) deposits are more common in interface dermatitis than any other disease. Between lichen planus (LP) and discoid lupus erythematosus (DLE), CB alone is more common in LP; whereas, CB plus DEJ and superficial blood vessel (SBV) is more common in DLE. The most common pattern in both diseases is CB plus DEJ. The quantity and intensity of CB in LP is higher than in DLE.

Intertriginous bullous morphea: A clue for the pathogenesis?

Bullae occurring in lesions of morphea are uncommon. The cause of bullae formation in morphea is multifactorial, although lymphatic obstruction from the sclerodermatous process is considered the likeliest cause. Bullous morphea may be confused clinically with lichen sclerosus et atrophicus since both diseases may cause bullae in sclerodermatous plaques. A 69-year-old woman presented with a history of generalized morphea diagnosed 9 years earlier; and a 1-month history of pruritic bullae on her inframammary folds, axillary regions, lower abdomen, upper extremities and inguinal folds. Physical examination revealed multiple erythematous erosions, hemorrhagic vesicles and eroded bullae with slight scale or crusts overlying hypopigmented, indurated, shiny plaques. Skin biopsy revealed prominent edema in the papillary dermis, resulting in bulla formation and thickening of collagen fibers within the dermis. Direct immunofluorescence was negative. According to histologic and clinical features, the diagnosis of bullous morphea was established.

Linear scleroderma following Blaschko's lines.

Blaschko's lines form a pattern, which many diseases are found to follow, but linear scleroderma following Blaschko's lines is a controversial entity rarely reported in the literature. A 24-year-old man presented with multiple linear, atrophic, hyperpigmented lesions punctuated by areas of depigmentations on the left half of the trunk distributed on the anterior, lateral and posterior aspects. The lesions were distributed in a typical S-shaped line. Antinuclear antibody and antihistone antibody tests were negative. Histopathological examination of the skin from the affected area showed features suggestive of scleroderma. Here, we present a case of linear scleroderma following Blaschko's lines in a male patient--an entity reported only three times so far.

[Digital necrosis of the arm excluding scleroderma. Retrospective study of 45 cases]. / Les nécroses digitales du membre supérieur en dehors de la sclérodermie. Etude rétrospective, à propos de 45 observations.

UNLABELLED: The causes of digital necrosis are so diverse that it should be considered not as a surgical but as a medical emergency. In women, digital necrosis first suggests connective tissue disease, and especially Progressive Systemic Sclerosis. In men the causes are more diverse. In this retrospective study, we have tried to identify and categorize the cause of digital necrosis in patients without Progressive Systemic Sclerosis. The medical files of all patients admitted from 1/1/89 to 1/1/93 for digital necrosis were retrospectively reviewed. All patients with Progressive Systemic Sclerosis, either diagnosed at that time or know before, were excluded. All 45 patients in the study had an evaluation of coagulation, vascular risk factors, a cardiac examination and nailfold capillary microscopy, as well as tests for antinuclear antibodies, and cryoglobulinaemia. Other tests were done according to the clinical presentation. Upper limb angiography was done 26 times. RESULTS: there were 10 women and 35 men, mean age 53 years (24-85 years). In the women, the following diagnosis were found: atherosclerosis 1, paraneoplastic syndrome 1, one thrombocythemia, and 6 connective tissue diseases (one dermatopolymyositis, 1 essential cryoglobulinaemia, 2 lupus erythematous and two suspected secondary Raynaud's phenomenon. Twelve men had arterial diseases: 6 thromboangiitis obliterans and 6 obliterans atherosclerosis. Seven times, the cause was local: 5 hammer's syndromes, 2 thoracic outlet syndrome. In the other patients, the causes were extremely diverse: cardiac embolism (4), primary antiphospholipid syndrome (3), polyarteritis nodosa (1), leprosy seroconversion (1), connective tissue disease (3). In 4 patients (3 of whom were smokers) no cause was found.(ABSTRACT TRUNCATED AT 250 WORDS)