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.

Linear morphea with secondary mucinosis.

Secondary mucin deposition in the skin is a common feature of lupus erythematosus and dermatomyositis. In scleroderma, it occurs uncommonly or in small amount. We describe a 7-year-old boy with progressive, linear, bound-down plaques involving the thighs, lower abdomen and back with no systemic involvement. Histopathology showed features of scleroderma with abundant mucin deposition in the reticular dermis. This report highlights excessive mucin deposition in lesions of morphea.

Generalized morphea, lichen sclerosis et atrophicus associated with oral submucosal fibrosis in an adult male.

Generalized morphea is a disease characterized by wide-spread sclerosis of the skin. A 39-year-old man presented with history of multiple pigmented and bound-down plaques on the body along with mucosal involvement. Dermatological examination showed multiple indurated and sclerosed plaques with follicular plugging in few of them and gross thickened eroded and glazed tongue. The constellation of these findings with histopathological correlation led us to diagnosis of this spectrum of cutaneous involvement. The coexistence of localized morphea with lichen sclerosis et atrophicus has been reported earlier but existence of these entities with submucosal fibrosis in a same patient is documented here and is the first of its kind.

Childhood sclerodermatomyositis with generalized morphea.

Systemic sclerosis (SS) and dermatomyositis (DM) are both multisystem disorders and share some common clinical features. We report here an 11 year-old girl whose disease showed a changing clinical pattern from juvenile systemic sclerosis (JSS) to slowly progressing juvenile dermatomyositis (JDM) and had associated generalized morphea. Serological studies revealed antinuclear antibodies (ANA) with a speckled pattern. Topoisomerase-I (Scl-70), U1 RNP (ribonucleoprotein), anti-Ro, anti-La and anti Jo-1 antibody tests were negative. Electromyography (EMG) was suggestive of primary muscle disease and histopathological findings indicated scleroderma. The patient fulfilled the American College Rheumatology (ACR) diagnostic criteria for JSS as well as Bohan and Peter criteria for JDM separately and hence, was diagnosed to have sclerodermatomyositis (SDM). Mixed connective tissue disease (MCTD) and antisynthetase antibody syndrome (ASS) which share same clinical features with SS and DM were excluded by immunological studies.

Unilateral linear pansclerotic morphea affecting face and limbs.

Disabling pansclerotic morphea is a rare atrophying and sclerosing disorder of the subcutaneous tissue, muscle and bone. It is characterized by atrophy of the skin, subcutaneous fat, muscle and bone involving half of the face. In some patients the atrophic lesions extend to involve the ipsilateral or contralateral upper and lower limbs with radiological evidence of hemiatrophy. The patients may present with arthralgia, convulsions or cramps. We report a case of a woman with deformity of face, and left upper and lower limbs that had started as an indurated plaque on the left half of forehead at the age of 5 years and had gradually enlarged, followed by the development of atrophic changes in left eye. The case is being reported in view of its rare occurrence.

[Pseudoscleroderma and sclerodermiform states]. / Pseudo-sclérodermies et états sclérodermiformes.

Pseudo-scleroderma should not be confused with true scleroderma, the prognosis of which is unpredictable and often serious. Progressive acrosclerosis must be differentiated from Raynaud's disease, congenital or hereditary disorders of unknown aetiology: Werner's syndrome, acrogeria and progeria; Rothmund-Thomson's syndrome, Steinert's disease, phenylketonuria, disorders of glycogen metabolism; metabolic disorders: mutilating acropathies, scleromyxoedema, porphyria cutanea tarda; occupational and iatrogenic disorders: acroosteolysis, toxic epidermic syndrome (Spain), scleroderma-like change induced by bleomycin, chronic graft-versus-host disease; and leprosy. Acute diffuse scleroderma should not be confused with Buschke's scleroedema, sclerema neonatorum, systemic amyloidosis and scleroderma-like changes in hypothyroidism. Linear pseudo-scleroderma is suggested by the following scleroderma-like conditions: facial hemiatrophy, acrodermatitis atrophicans, melorheostosis, pseudo-scleroderma after corticosteroid injection, and cutaneous lesions in carcinoid syndrome. Scleroderma in plaque must be differentiated from hypodermitis sclerotisans, panatrophy and localized lipoatrophies, hypodermitis after vitamin K injection, basal cell carcinoma, necrobiosis lipoidica, vitiligo, chronic radiodermatitis, cutaneous lymphatic invasion. Scleroderma-like changes after drug injection (vitamin B12, progestin), anetoderma barely resemble morphea guttata.