Rectangular-patterned Occipital Alopecia Areata: A Report of Three Cases.

Many reports have described the presence of alopecia areata (AA) associated with other autoimmune diseases, which support the autoimmune nature of AA. Additionally, AA has been reported in association with malignancy as a paraneoplastic symptom. In this report, we describe three patients with diffuse large B-cell lymphoma, alveolar soft part sarcoma, and cavernous sinus arteriovenous fistula with embolization treatment, respectively, who characteristically presented with rectangular-patterned occipital AA.

INTRODUCTION

Alopecia areata (AA) is a common inflammatory disorder characterized by a sudden loss of hair without scarring. Although the exact pathogenesis remains speculative, emerging evidence regards AA as an organ-specific autoimmune disease mediated by either CD4+ or CD8+ T cells.[12] Many reports have described the presence of AA associated with other autoimmune diseases, including autoimmune thyroid disease, vitiligo, and autosomal recessive autoimmune polyglandular syndrome, which support the autoimmune nature of AA.[12] Additionally, AA has been reported in association with malignancy as a paraneoplastic symptom.[3-5] In this report, we describe three patients with diffuse large B-cell lymphoma, alveolar soft part sarcoma, and cavernous sinus arteriovenous fistula with embolization treatment, respectively, who presented with rectangular-patterned occipital AA.

CASE REPORTS

Case 1

A 47-year-old Korean female was referred for a palm-sized rectangular shaped area of alopecia on the occipital scalp that had progressed over 1 month. Past medical history revealed that she had begun treatment for pulmonary tuberculosis 6 months before presentation. On her first visit, rectangular-patterned occipital hair loss and a positive hair pull test with dystrophic hairs were noted. For the treatment of AA, systemic steroid and intralesional triamcinolone acetonide injections were administered; however, the alopecic patches progressed to involve the entire posterior scalp. After 2 months, the patient presented to the emergency department because of a sudden attack of right arm weakness and dysarthria. Magnetic resonance imaging (MRI) of the brain was performed and revealed a cystic mass, 3.3 cm in diameter, on the left frontal area [Figure 1a and b]. Craniotomy with diagnostic needle aspiration biopsy of the supratentorial brain tumor revealed diffuse large B-cell lymphoma. Systemic chemotherapy with methotrexate was delivered for the treatment of lymphoma; however, the lesions of AA progressed and the patient died from sepsis.

Figure 1

Magnetic resonance imaging of diffuse large B-cell lymphoma in a 47-year-old Korean female (Case 1): (a) horizontal view of the T2- weighted image; (b) coronal view of the T2-weighted image; (c) Occipital rectangular-patterned alopecia areata in a 22-year-old Korean female (Case 2); (d) 18F-fluorodeoxyglucose (FDG) uptake in the alveolar soft part sarcoma (Case 2; arrow; coronal view of an FDG-positron emission tomography/computed tomography scan)

Case 2

The second case was a 22-year-old female patient who presented with a 2-month history of rectangular-patterned hair loss on the occipital area [Figure 1c]. The patient had undergone a computed tomography (CT) examination of the head and neck for the evaluation of prolonged facial swelling and difficulties opening her mouth, which had persisted for longer than 5 months. The CT examination showed a 4-cm hypervascular soft tissue tumor at the left infratemporal fossa, and whole-body 18F-fluorodeoxyglucose (FDG) positron emission tomography/CT also demonstrated increased FDG uptake in the mass, with no evidence of distant metastasis [Figure 1d]. The biopsy specimens obtained from surgical excision of the tumor revealed an alveolar soft part sarcoma. On her first visit, she had rectangular-patterned occipital hair loss and a positive hair pull test with dystrophic hairs. Monthly intralesional triamcinolone acetonide injections were provided, and the alopecic patches improved. Then, the patient underwent adjuvant radiotherapy for the treatment of the alveolar soft part sarcoma, with no progression of the alopecic patches.

Case 3

The third case was a 52-year-old male patient who presented with a 2-week history of rectangular-patterned hair loss on the occipital area. Several months ago, he had first had the symptom of horizontal diplopia and was evaluated with brain MRI which showed the result of left cavernous sinus dural arteriovenous fistula. After diagnostic angiography, cerebral embolization was performed. Two weeks after the procedure, the patient developed rectangular shaped occipital AA and also hair loss of the left temporal area, locating in the related place where the arteriovenous fistula had been treated [Figure 2]. On the skin biopsy, increased numbers of catagen hair follicles with peribulbar lymphocytic infiltration was seen. Monthly intralesional triamcinolone acetonide injections were provided, and the alopecic patches improved.

Figure 2

(a) Rectangular-patterned occipital alopecia areata in a 52-year-old Korean male (Case 3); (b) Profile of the same patient showing temporal area alopecia areata; (c) Coronal view and (d) right anterior oblique view of cerebral embolizations

DISCUSSION

Several reports have demonstrated that AA can accompany either solid or hematologic malignancies such as acute lymphoblastic leukemia and Hodgkin's disease.[1-3] Because AA commonly occurs in worldwide populations, the association between AA and malignancy can be interpreted as a coincidental phenomenon. However, some authors have suggested that AA may be accompanied in malignancies either as a result of an autoimmune process caused by the altered cellular immune system characteristically found in hematologic malignancies or as a paraneoplastic symptom.[3]

In our cases, the first patient had diffuse large B-cell lymphoma and the second patient had alveolar soft part sarcoma, while the third patient was diagnosed with cavernous sinus arteriovenous fistula and treated with embolization. It is known that the intensity of vascular endothelial growth factor and factor VIII immunostaining in alopecic hair follicles was significantly lower than in healthy scalp tissue.[6] Considering that our third case developed AA following the embolization of the vascular malformation, abrupt changes in blood flow could have resulted in specific pattern of alopecia. We also guessed that the vascular structures could have been affected by the space-occupying lesions of the tumor, or micro-embolization of the tumor cells could have associated with AA.

Alopecia neoplastica, which refers to the neoplasm-associated hair loss of scalp, should be ruled out in patients with underlying malignancies.[7] Malignancies associated with primary alopecia neoplastica reportedly include basal cell carcinoma, squamous cell carcinoma, malignant melanoma, extra-mammary Paget's disease, dermatofibrosarcoma protuberans, angiosarcoma, and cutaneous T-cell lymphoma.[7] The first patient in this report with diffuse large B-cell lymphoma initially presented rectangular-patterned occipital hair loss and then progressed to involve the entire posterior scalp. Although the patient demonstrated the unusual pattern of hair loss, the possible diagnosis of alopecia neoplastica could not be ruled out by the lack of pathological confirmation.

The specific patterns of hair loss associated with diseases such as malignancies or vascular malformation have not been fully emphasized. In this report, we described three cases of rectangular-patterned occipital AA that were associated with diffuse large B-cell lymphoma, alveolar soft part sarcoma, and cavernous sinus arteriovenous fistula, respectively. We recommend that imaging studies, including CT and MRI, of the head and neck should be used to evaluate for associated malignancies or vascular deformities in patients with sudden and extensive atypical patterned hair loss.