Centrifugal lipodystrophy of the scalp presenting with an arch‐form alopecia: A 10‐year follow‐up observation

Abstract

We describe a 10-year follow-up observation of progressive arch-form alopecia caused by centrifugal lipodystrophy (CLD) in a Japanese boy. A 2.5-year-old boy developed a slightly depressed lesion demarcated by a horseshoe-shaped erythematous border on his right neck, which then extended to the scalp. Four years later, arch-form alopecia became apparent in the right temporal region along with an erythematous border. The arch-form alopecia gradually expanded centrifugally, leaving a slight residual depression, but hair regrowth was seen within the area of alopecia. Histological examination of the erythematous border revealed non-specific inflammatory changes in the subcutaneous fat. Magnetic resonance imaging findings revealed a loss of subcutaneous fat inside the lesion. The alopecia continuously extended until he was 12 years old, but, thereafter, expansion ceased and hair regrowth gradually occurred in the arch-form alopecia. A linear non-hairy lesion 5 cm in length still remained when he was 13 years old. CLD might involve the scalp and cause linear, arch-form alopecia.

Loss of Mpzl3 Function Causes Various Skin Abnormalities and Greatly Reduced Adipose Depots

“…In a large-scale knock-out project of secreted and transmembrane proteins, reduced body weight, fat mass and development of alopecia were also reported in Mpzl3 knockout mice (Tang et al , 2010). Adipocytes within the skin and subcutis are becoming recognized for more than just their roles in energy storage (Klein et al , 2007; Schmidt and Horsley, 2012), as alopecia, hirsutism and hypertrichosis have been documented in cases with lipodystrophy (Agarwal and Garg, 2006; Fukumoto et al , 2009; Savage et al , 2007; Schmidt and Horsley, 2012) and obesity (Samara-Boustani et al , 2012). It remains to be investigated whether alopecia in older Mpzl3 −/− and rc / rc mice is directly related to the reduced dermal adipose tissue or a systemic effect of reduced adipose depots.…”

Adipocytes in Skin Health and Disease

“…In addition, loss of adipocytes in lipodystrophic conditions has been associated with alopecia (Fig. 4) (Hegele et al 2002;Hegele 2005;Agostini et al 2006;Fukumoto et al 2009;Jeninga et al 2009). The most prevalent form of inherited partial lipodystrophy results from a mutation in the LMNA gene, encoding nuclear envelope proteins lamin A and C. Lamin A has been shown to influence adipocyte differentiation, potentially through PPARg and insulin signaling (Agostini et al 2006;Boguslavsky et al 2006).…”

“…Lipedematous scalp and lipedematous alopecia are rare disorders characterized by a thick and spongy scalp with dysfunction in skin associated adipose tissue, hyperkeratosis (Fukumoto et al 2009), lymphocytic infiltrates (Bridges et al 2000;Fiorenza et al 2010) and androgenic alopecia (Piraccini et al 2006). In addition, loss of adipocytes in lipodystrophic conditions has been associated with alopecia (Fig.…”

The Role of Adipocytes in Tissue Regeneration and Stem Cell Niches

“…Various lipodystrophic conditions are characterized by hair loss or alopecia concomitantly with a loss of adipose tissue; however, a causative role of human dWAT in hair follicle maintenance has not been described (Fukumoto et al 2009, Hegele 2005, Jeninga et al 2009). Additionally, as many as 61% of patients suffering from anorexia have hair loss or fragile hair (Strumia 2009), along with lower levels of leptin (Janas-Kozik et al 2011, Uzum et al 2009).…”

“…Additionally, as many as 61% of patients suffering from anorexia have hair loss or fragile hair (Strumia 2009), along with lower levels of leptin (Janas-Kozik et al 2011, Uzum et al 2009). Hyperkeratosis, immune cell infiltration, and alopecia can also be observed in the scalps of patients experiencing lipoedema (Bridges et al 2000, Fukumoto et al 2009, Piraccini et al 2006), and these symptoms can worsen as adiposity increases. Moreover, lipodystrophic individuals with mutations in PPARG frequently exhibit hyperpigmentation, hirsutism, and cutaneous eruptive xanthomata (Hegele 2005, Herrmann et al 2003).…”