Diffuse Alopecia Areata Associated with a Solid-Organ Malignancy: Case Report and Literature Review

  • International Hair Research Foundation
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Alopecia areata (AA) is an autoimmune disease, manifesting as non-scarring hair loss. Here we report a 57- year-old woman, who presented with sudden diffuse non-scarring hair loss on the scalp. Histology confirmed the diagnosis of AA. Imaging studies revealed the presence of an irregular mass in the left kidney, and histology demonstrated spindle cell-type renal cell carcinoma. There was spontaneous regrowth of hair after surgical and chemotherapy treatments. Reports of AA as first manifestation of malignancy are uncommon, and to our knowledge, this is the first report of AA as a paraneoplastic manifestation of renal cell carcinoma. This report also suggests that screening for malignancy should be considered in older patients, who present with sudden and diffuse AA.

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A 17-year-old girl presented with a 3 month history of alopecia areata, a generalized scaling skin, enlargement of the inguinal lymph nodes and severe back pain. Staging procedures revealed multifocal bone disease and generalized lymphadenopathy. The diagnosis of nodular sclerosing Hodgkin's disease was established by biopsies of the os ileum and a left inguinal lymph node. Complete clinical remission was achieved after 2 OPPA (vincristine, prednisone, procarbazine, and doxorubicin) and 4 COPP (cyclophosphamide, vincristine, prednisone, and procarbazine) cycles and treatment completed with involved-field irradiation. After the completion of therapy, skeletal lesions had mostly resolved or become sclerotic and the patient had normal hair regrowth and skin appearance. Conclusively, this case illustrates that alopecia areata may occur as a paraneoplastic phenomenon or an autoimmune process related to the deranged cellular immune system in children and adolescents with Hodgkin's disease.
Figure.A, Singular patch of alopecia areata on the left parietal side (X) of the patient's scalp. B, Total canities. The time lapse between A and B was 6 months (normal photodocumentationinterval).Marie Antoinette syndrome designates the condition in which scalp hair suddenly turns white. The name alludes to the unhappy Queen Marie Antoinette of France (1755-1793), whose hair allegedly turned white the night before her last walk to the guillotine during the French Revolution. She was 38 years old when she died. Although the actual incidence is rare, this stigmatizing phenomenon, which has captured storytellers' imagination like few other afflictions, occurs to protagonists as a sign of grave sorrow in religious texts as early as the Talmud. History also records that the hair of the English martyr Sir Thomas More (1478-1535) turned white overnight in the Tower of London before his execution. More modern accounts refer to the turning white of hair in survivors of bomb attacks during World War II. In 1957, an American dermatologist witnessed a 63-year-old man's hair turn white over several weeks after he had fallen down some stairs. The patient noticed loss of hair but no bald patches and 17 months later had extensive vitiligo.1 The term canities subita has also been used for this disorder.2- 3 Today, the syndrome is interpreted as an acute episode of diffuse alopecia areata in which the very sudden “overnight” graying is caused by the preferential loss of pigmented hair in this supposedly immune-mediated disorder.4 This observation has led some experts to hypothesize that the autoimmune target in alopecia areata may be related to the melanin pigment system.5
Self tolerance loss is fundamental to autoimmunity. While understanding of immune regulation is expanding rapidly, the mechanisms causing loss of tolerance in most autoimmune diseases remain elusive. Autoimmunity is believed to develop when genetically predisposed individuals encounter environmental agents that trigger the disease. Recent advances in the genetic and environmental contributions to autoimmunity suggest that interactions between genetic elements and epigenetic changes caused by environmental agents may be responsible for inducing autoimmune disease. Genetic loci predisposing to autoimmunity are being identified through multi-center consortiums, and the number of validated genes is growing rapidly. Recent reports also indicate that the environment can contribute to autoimmunity by modifying gene expression through epigenetic mechanisms. This article will review current understanding of the genetics and epigenetics of lupus, rheumatoid arthritis, multiple sclerosis and type 1 diabetes, using systemic lupus erythematosus as the primary example. Other autoimmune diseases may have a similar foundation.
A 57-year-old Japanese woman who suffered from alopecia areata associated with myasthenia gravis (MG) and thymoma responded well to thymectomy and high doses of glucocorticosteroid administration. Several treatments for alopecia areata including administration of systemic prednisolone were attempted, but loss of hair on the scalp progressed. After thymectomy and high level glucocorticosteroid administration for MG, the lesions on the scalp improved within four weeks. Consequently, we suggest that this thymectomy and high level glucocorticosteroid administration assisted in improving the immune dysfunction causing the alopecic lesions in this patient.
A 34-year-old previously healthy man presented to his general practitioner with nocturnal sweating, feeling full and a weight loss of 6 kg in three months. Over a period of one year clubbing of the fingers had developed and alopecia areata had been noted six months before the diagnosis was established. Out-patient sonography revealed two large, partly cystic-necrotic space-occupying lesions in the liver. Sonographically guided fine-needle puncture showed cells of a mesenchymal tumor with great cell density and extensive necroses. Gastroscopy and endosonography demonstrated, as a possible primary tumor, a submucosal space-occupying lesion, ca. 3 x 5 cm, in the distal esophagus. Immunochemical tests revealed a leiomyosarcoma. A gastrectomy and resection of the distal esophagus were performed, together with a liver wedge resection. Histopathological examination of the surgical specimen confirmed the diagnosis of leiomyosarcoma. Further immunochemical tests provided the diagnostic criteria of a gastrointestinal stroma tumor (GIST). The patient died one year after operation from hepatic and peritoneal metastases. Tumors of the gastrointestinal tract may for a long time produce no or, as in this case, only nonspecific symptoms. An early diagnosis is, however, possible if little known early paraneoplastic phenomena are noted in the clinical examination.
The goal of this review is to introduce the immunologic community to alopecia areata as a model system for the study of tissue directed autoimmune disease. Alopecia areata is marked by autoimmune assault on the hair follicle resulting in hair loss. It is linked to HLA-DQ3 and evidence suggests it is mediated by T-lymphocytes with a TH1 cytokine profile. Hair follicles are an immune protected site with deficient MHC expression. Evidence is presented suggesting that alopecia areata results from loss of immune privilege with presentation of autoantigens. Alopecia areata is one of the most common human autoimmune conditions, with a lifetime risk of approximately 1.7%. Study of alopecia areata in humans is facilitated by the accessibility of scalp for biopsy. It is possible to transfer the condition with lesional human lymphocytes in a human scalp graft/SCID mouse model. There are also spontaneous animal models which share the features of the human condition. For these reasons, alopecia areata is a powerful model for study of the induction and pathogenesis of tissue directed autoimmune disease.
We report a patient who presented simultaneously with primary cutaneous follicle center lymphoma (PCFCL) of the face and scalp and alopecia areata of the scalp and beard bearing a clonal T-cell receptor gene rearrangement. To our knowledge, alopecia areata has not been previously reported in association with PCFCL. Both lesions regressed with topical imiquimod and systemic steroids, which suggests an inter-relationship in this case between the clonal B-cell and T-cell populations in driving outgrowth of these lesions.