Good syndrome associated with lichen planus: a case report and review

Fatrez, Béatrice; Cuny, J.-F.; Bursztejn, Anne‐Claire; Schmutz, J.‐L.; Moawad, S.

doi:10.1684/ejd.2019.3582

Cited by 4 publications

(4 citation statements)

References 6 publications

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“…3 Diarrhea is present in 50% of patients and lichen planus (LP), in 12%. 4,5 All of the reported cases of Good syndrome-associated LP, patients presented with erosive lesions; skin involvement was only described in approximately 50%. Associated autoimmune diseases do not always improve after thymectomyhypogammaglobulinemia usually persists, and substitutive treatment with immunoglobulins becomes mandatory to prevent infections.…”

Section: Discussionmentioning

confidence: 99%

“…Good syndrome is correlated with poor survival associated with severe infections and autoimmune-associated diseases that occur in 59% of cases, which often require immunosuppressive therapy . Diarrhea is present in 50% of patients and lichen planus (LP), in 12% . All of the reported cases of Good syndrome-associated LP, patients presented with erosive lesions; skin involvement was only described in approximately 50%.…”

Section: Discussionmentioning

confidence: 99%

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Oral Lichenoid Lesions Associated With Mediastinal Mass and Hypogammaglobulinemia in a Middle-aged Man

2022

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White man in his 50s with a clinical history of recurrent respiratory infections and diarrhea presented with painful oral erosions that began 2 years before. Results of a previous intestinal biopsy had shown unspecific findings and absence of apoptosis. He reported a 5-kg weight loss that he attributed to impaired intake owing to the oral lesions. He was also being evaluated for a mediastinal mass detected in radiography of the chest performed for pneumonia. Physical examination revealed whitish edematous lacy patches with erosions on the dorsal and lateral aspects of the tongue and buccal mucosa (Figure , A); skin and nails were not involved. A lingual biopsy specimen was obtained for pathologic evaluation and direct immunofluorescence (DIF; Figure , B and C). The result of indirect immunofluorescence (IIF) testing, using monkey esophagus, salt-skin split, and rat bladder as substrates, was negative. Enzyme-linked immunosorbent assay (ELISA) and immunoblot analysis did not detect any desmoglein-1 or -3, bullous pemphigoid-180 or -230, collagen VII, desmocollin-1 to -3, laminin-332, envoplakin, or periplakin antibodies. Results were normal for liver function; negative for hepatitis B and C; indicative of hypogammaglobulinemia, with a decreased immunoglobulin (Ig) G serum level of 465 mg/dL (normal, 750-1600 g/L; for g/L, multiply by 0.01); and normal for IgA and IgM levels. Positron emission tomography revealed a 10-cm hypercapturing extrapulmonary mass (Figure , D). Surgical excision of the mediastinal mass was performed; its histopathologic features were consistent with a type AB thymoma, Masaoka-Koga stage 2A. Clinical image A Original magnification ×100 B Immunofluorescence C Positron emission tomography-computed tomography D A, Lacy, white erosive lesions on the tongue. B, Epidermis with acanthosis, parakeratosis, necrotic keratinocytes, and dermal band lymphocytic infiltrate with effacement of the dermoepidermal junction (hematoxylin-eosin). C, Fibrogen deposition along the basement membrane. D, Hypercapturing, heterogeneous mass in the right anterior hemithorax. WHAT IS YOUR DIAGNOSIS? A. Paraneoplastic autoimmune multiorgan syndrome B. Thymoma-associated multiorgan autoimmunity C. Good syndrome D. Paraneoplastic lichen planus Quiz at jamacmelookup.com Clinical Review & Education

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Oral Lichenoid Lesions Associated With Mediastinal Mass and Hypogammaglobulinemia in a Middle-aged Man

2022

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“…7 In certain cases, LP lesions may be improved or resolved after thymectomy. 8 One interesting aspect of this case is that since the patient presented with severe pneumonia during the COVID-19 epidemic, the initial diagnosis of COVID-19 was at the top of the list. Multiple reports have emerged linking the complicity of COVID-19 in patients with GS.…”

Section: Clinical Featuresmentioning

confidence: 99%

Thymoma-Associated Good Syndrome: A Case Report and Literature Review

Yazid,

Chung,

Senusi

2024

EMJ Allergy Immunol

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Thymoma is often associated with many other autoimmune disorders and clinical conditions. Good syndrome is one of the rare associations between thymoma and immune deficiency that occurs in both males and females in the 4th or 5th decade of life. Patients can present with various invasive encapsulated organisms, and opportunistic viral and fungal infections, due to immune defects. The authors report a case of a 57-year-old male with underlying thymoma and lichen planus which were diagnosed 3 years apart. He presented with atypical pneumonia during the COVID-19 pandemic, and was treated for multiple atypical infections, including cytomegalovirus and Pneumocystis carinii pneumonia. His immunological investigation panel revealed low IgA and IgG levels with normal IgM levels, and an overall deficient total B cell count. His CD4:CD8 ratio was reversed at 0.28. The patient recovered well after Ig replacement therapy once the Good syndrome diagnosis was made. The authors highlight the importance of a high index of clinical suspicion in dealing with this uncommon concomitant occurrence of Good syndrome during the COVID-19 pandemic for a swift and timely diagnosis and management. Immunological investigation panels, including T cell subsets, B cells, and quantitative Ig levels, should be considered routinely in patients with underlying thymoma presenting with opportunistic infections.

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“…Low or absent B lymphocytes and CD4 + cell lymphopenia with inverted CD4/CD8 ratio are often present 3 . Lichen planus is described in 12% of patients with GS, mostly oral erosive lichen planus 4 . GS predisposes to severe infections due to a predominantly humoral deficiency, with a worse prognosis than CVI.…”

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confidence: 99%

Oral lichenoid lesions as a sign of Good’s syndrome

2021

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basement membrane zone with C3 (Figure 3). In addition, there were few colloid bodies in the upper dermis staining with IgG, IgM, and IgA. Systemic steroids were instituted at a dose of 1 mg/kg, which was gradually tapered over a period of 3 months.The disease responded well to the treatment.LPP was earlier thought to be an overlap of bullous pemphigoid and LP but is increasingly considered a unique disease entity in itself. As the majority of cases of LPP have lesions of LP preceding bullous eruption, it is proposed that the lichenoid inflammation might actually promote the development of an autoimmune response, which targets proteins of the epidermal basement membrane. 1-3 Differential diagnoses having similar clinical and histopathological features include bullous lichen planus, erythema multiforme, atypical subacute cutaneous lupus erythematosus, and paraneoplastic pemphigus. Diagnosis is made by a thorough history taking, examination, histopathology, immunofluorescence, and immunoblotting.The case reported is typical of LPP with classical clinical features with lesions having a propensity to appear more on the peripheral sites rather than in a generalized fashion. 4 The histopathology and direct immunofluorescence were also suggestive of LPP. Unfortunately, immunoblotting could not be performed because of unavailability at the institute. We postulate that the Wickham striae (WS) visualized on dermoscopy corresponds to the beaded hypergranulosis seen on histopathology, which is visible as the cleft is subepidermal in nature. WS is considered the hallmark of LP and is pathognomic of the disease. 5 The central dimple, that is, structured white globule, might correspond to the infundibulum of hair.

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Good syndrome associated with lichen planus: a case report and review

Cited by 4 publications

References 6 publications

Oral Lichenoid Lesions Associated With Mediastinal Mass and Hypogammaglobulinemia in a Middle-aged Man

Oral Lichenoid Lesions Associated With Mediastinal Mass and Hypogammaglobulinemia in a Middle-aged Man

Thymoma-Associated Good Syndrome: A Case Report and Literature Review

Oral lichenoid lesions as a sign of Good’s syndrome

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