Progressive Hemifacial Atrophy in a Patient with Lupus Erythematosus

Roddi, Roberto; Riggio, Egidio; Gilbert, Philip M.; Vaandrager, J. Michiel; Meulen, J. C. H. van der

doi:10.1097/00006534-199404001-00027

Cited by 26 publications

(9 citation statements)

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“…Concomitant occurrence of autoimmune diseases such as systematic lupus erythematosus (SLE) and PHA has been reported. [1,6] Autoimmunity-related antibodies are often found positive in PHA patients. [21–23] In line with previous reports, increased serum levels of ANA and γ IgG were observed in this patient, which suggests a common autoimmune-related background.…”

Section: Discussionmentioning

confidence: 99%

“…[2,5,6] In this report, we describe a case of PHA who manifested serial changes in brain lesions on magnetic resonance imaging (MRI). The case suggests that PHA may have a “relapsing–remitting” disease course and that periphery hepatitis B virus (HBV) infection associated auto-immune inflammatory process may be one of the triggers for relapse of PHA.…”

Section: Introductionmentioning

confidence: 99%

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Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

Zhang

Zhu

et al. 2016

Medicine

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Introduction:Progressive hemifacial atrophy (PHA) is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, and fat, muscle, and osteocartilagenous structures creating a sunken hemiface appearance.Etiopathogenesis of PHA is poorly understood; no definitive treatment is currently available.Clinical Findings:We report a 41-year-old woman with PHA who showed an uncharacteristic “relapsing–remitting” evolution of brain lesions and was seropositive for hepatitis B virus (HBV). She presented with a history of recurrent tonic-clonic seizures. Magnetic resonance imaging (MRI) showed progressive atrophy and multiple white matter lesions in the left side of the brain. Interestingly, the serial MRI examination (4 MRI scans over a period of 9 years) showed a “relapsing–remitting” pattern of brain lesions akin to that observed in a subtype of multiple sclerosis. Autoimmune-related investigations revealed increased serum levels of immunoglobulin (Ig) G, anti-nuclear antibody (ANA), and γ-IgG. Infection is considered as one of the possible causes of PHA. However, the association of peripheral infection such as HBV infection with PHA has not been reported.Conclusion:Our experience with this case suggests that PHA may have a relapsing–remitting disease course. Autoimmune inflammatory response to chronic HBV infection may have triggered the relapse in this case. This case underlines a novel etiopathogenetic mechanism of PHA.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

Zhang

Zhu

et al. 2016

Medicine

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“…174 Although the exact cause is unknown, an autoimmune type of disease has been strongly implicated. 175 Other possible causes include viral, neural, or traumatic. 176,177 Some distinguish Parry-Romberg syndrome from a similar form of scleroderma known as linear scleroderma.…”

Section: Parry-romberg Syndrome (Disease)mentioning

confidence: 99%

Craniofacial Syndromes

Buchanan

Xue

Hollier

2014

Plastic and Reconstructive Surgery

166

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Craniofacial syndromes fall into two major categories-those associated with craniosynostosis, and those associated with clefts. Each has a different set of potential complications requiring a unique approach for surgical management. Craniosynostosis is a congenital disorder in which one or more of the cranial sutures fuses prematurely. The most common syndromes associated with this condition include Crouzon, Apert, Pfeiffer, Muenke, and Saethre-Chotzen syndromes. Surgical management of these children requires a multidisciplinary approach and close involvement of the family. Operations must take into consideration the growing potential of the bony structures. Common syndromes associated with clefts include Pierre Robin, Treacher Collins, Nager, Binder, and Stickler syndromes. Many of these children have severe airway issues requiring immediate address before operative reconstruction. As with syndromes associated with craniosynostosis, the key to management is a multidisciplinary approach focused on the right timing. The purpose of this article is to review the clinical presentation, care, and treatment of these patients.

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“…It is characterized by progressive wasting of one side of the face, involving primarily the skin, fat and muscles; bone and cartilage are rarely affected unless onset occurs before the second decade, when face and skull structures are not fully developed. This entity is a process of unknown aetiology that evolves with invalidating lesions that may be associated with different neurological, cutaneous, ocular, autoimmune or analytical abnormalities 3–6 . There are few reports showing the association between PRS and borreliosis.…”

Section: Introductionmentioning

confidence: 99%

Parry–Romberg syndrome: A possible association with borreliosis

Şahin

Barış

Karaman

2004

Acad Dermatol Venereol

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Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue and the underlying bony structures. This syndrome has many features of linear scleroderma 'en coup de sabre' but is distinguished by more extensive involvement of the lower face with only slight cutaneous sclerosis. The onset typically occurs in childhood or young adult years. The aetiology of the condition is unknown. A case of progressive hemifacial atrophy occurring in a 30-year-old woman is reported, in which the aetiology is thought to be Lyme disease, a borrelial infection first recognized in 1975 that has since become the most commonly reported vector-borne disease in the world. No sure link is established between these two disease states, but their coincident occurrence in this patient is noted. We hypothesize that the aetiology of Parry-Romberg syndrome involves borreliosis.

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Progressive Hemifacial Atrophy in a Patient with Lupus Erythematosus

Cited by 26 publications

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Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

Craniofacial Syndromes

Parry–Romberg syndrome: A possible association with borreliosis

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