Primary Alopecia Neoplastica: A Novel Case Report and Literature Review

Abstract

Alopecia neoplastica (AN) is caused by neoplastic cells damaging hair follicles, resulting in patchy hair loss like cicatricial alopecia and alopecia areata. AN has predominantly described cutaneous metastasis to the scalp from primary visceral malignant tumors. Less frequently, AN results from a primary scalp neoplasm. Compared to “secondary AN,” there is a paucity of literature on “primary AN.” Herein, we present a comprehensive literature review of primary AN and introduce a unique case of amelanotic melanoma causing primary AN. Including our presented case, 11 cases of primary AN have been reported with causative scalp neoplasms including angiosarcoma, hemangioendothelioma, syringomatous carcinoma, ectopic extramammary Paget’s disease, and primary desmoplastic melanoma. 27.3% (3 of 11) of cases were misdiagnosed and treated for a primary alopecia, and 36.4% (4 of 11) of lesions were present for multiple years or an unknown amount of time, likely due to difficulty in recognizing scalp lesion or misdiagnosis. All patients required surgical excision with 36.4% (4 of 11) requiring chemotherapy, radiation, or photodynamic therapy. Two patients with scalp angiosarcoma died from their aggressive disease. Due to the risks of malignant primary AN if allowed to progress, primary AN should be considered in patients presenting with scarring alopecia.

Desmoplastic melanoma presenting as an alopecic patch in a young patient

“…Alopecia neoplastica (AN), cutaneous metastasis caused by neoplastic cells replacing hair follicles with fibrous tracts, 7 is believed to result from either direct fibrosis or the adjacent release of cytokines. 10 Commonly, AN is secondary to metastatic visceral malignancies from the gastrointestinal tract, breast, kidney, and lung. 10 Primary AN is a more infrequent phenomenon, occurring in 11 cases as of 2021 10 ; among these cases, primary malignant neoplasms included angiosarcomas, hemangioendotheliomas, ectopic extramammary Paget disease, syringomatous carcinoma, amelanotic melanoma, and DM.…”

“… 10 Commonly, AN is secondary to metastatic visceral malignancies from the gastrointestinal tract, breast, kidney, and lung. 10 Primary AN is a more infrequent phenomenon, occurring in 11 cases as of 2021 10 ; among these cases, primary malignant neoplasms included angiosarcomas, hemangioendotheliomas, ectopic extramammary Paget disease, syringomatous carcinoma, amelanotic melanoma, and DM. 10 While primary AN may result from benign neoplasms including neuromas, nerve sheath myxomas, or nevocellular nevi, it is important to acknowledge malignant sources with high morbidity.…”

“…Presentation as an alopecic patch is an uncommon scenario, with only 4 previous reported cases to the authors’ knowledge. 7 , 8 , 9 , 10 Here, we report a unique case of DM presenting as an alopecic patch on the scalp of a young male unresponsive to topical steroids.…”