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Amelanocytic Anhidrotic Alopecia Areata-like Phenotype After Allogeneic Hematopoietic Cell Transplant

Edidong Celestine Ntuen Kamińska, MD; Richard A. Larson, MD; Vesna Petronic-Rosic, MD, MSc
Arch Dermatol. 2012;148(8):931-934. doi:10.1001/archdermatol.2012.335.
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Background  Diffuse alopecia areata or canities subita is a rare variant of alopecia in which hair loss is associated with regrowth of white hairs and possible lightening of the skin. Preferential loss of pigmented hair in this disorder may be related to the melanin pigment system and/or melanocytes. Acquired generalized anhidrosis can be associated with autoimmune disease, cancer, graft-vs-host disease, or medications or can be idiopathic. Extensive anhidrosis may cause hyperpyrexia on exposure to heat, and protection from overheating is essential.

Observations  A 38-year-old man with idiopathic autoimmune aplastic anemia developed permanent diffuse skin and hair whitening and generalized anhidrosis after a successful allogeneic hematopoietic stem cell transplant. Results of a histopathologic examination, which has previously not been reported in this disease, demonstrated a complete lack of epidermal and follicular melanocytes and a dense perifollicular and periadnexal lymphocytic infiltrate.

Conclusions  The hair- and skin-lightening phenomena in diffuse alopecia areata may be the result of an overstimulated immune system that targets epidermal and follicular melanocytes. Destruction results in irreversible pigmentary loss of the skin. In a patient with multiple risk factors for anhidrosis, a specific cause may be difficult to determine.

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Figure 1. Diffuse alopecia sequence of events. A, Photograph of the patient immediately after completing chemotherapy for preparation of stem cell transplant; 2 weeks later the patient experienced anagen effluvium, followed by regrowth of brown hair. B, Active shedding of brown hair 6 months after chemotherapy that occurred within 24 hours, followed by regrowth of all white hair (the patient shaved to minimize hair loss). C, Eighteen months after chemotherapy, white hairs persisted and patchy hair loss (insets) continued.

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Grahic Jump Location

Figure 2. Results of a punch biopsy from the right arm. A, Hematoxylin-eosin–dense lymphocytic infiltrates surround a hair follicle and eccrine glands (original magnification ×100). B, Immunohistochemical microphthalmia-associated transcription factor (MITF) staining reveals internal control of mast cells highlighted with brown (original magnification ×200); no melanocytes were noted with MITF and melan-A stain (not shown).

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