Ultrasound Is Not Useful in Monitoring Lipedematous Alopecia: A Clinical, Trichoscopic, Histologic, and Ultrasound Analysis of 2 Cases

Abstract

Introduction

Lipedematous scalp (LS) is a rare condition characterized by thickened adipose tissue in the subcutaneous layer of the scalp resulting in a soft, spongy, or thick consistency of the scalp. When associated with hair loss, this condition is called lipedematous alopecia (LA). Various imaging modalities have been used to diagnose LS and LA along with histopathology.

Case Presentation

We present 2 cases of LS: a 56-year-old female with a 1-year history of hair thinning, pain, and tenderness at the vertex scalp and a 60-year-old female with a 5-year history of lichen planopilaris presenting with a 1-year history of itching and soreness on the crown of her head. Ultrasound (US) was used for diagnosis, treatment response surveillance, routine clinical examination, and symptom assessment. Follow-up US revealed no improvement in scalp thickness in either case despite symptom improvement and visual improvement in hair growth.

Discussion/Conclusion

US has been reported as a helpful tool in the diagnosis of LS; however, treatment response was better approximated by hair growth and symptom alleviation. We found that once the diagnosis with made with US, clinical monitoring is adequate as symptom improvement and hair growth may not correlate with a change in scalp thickness.

Established Facts

• Lipedematous scalp is a rare condition characterized by thickened adipose tissue in the subcutaneous layer of the scalp resulting in a soft, spongy, or thick consistency of the scalp.

• Few successful treatments for LS have been identified.

Novel Insights

• US is cost-effective and has been reported as a helpful diagnostic tool in the diagnosis of LS; however, we found that ultrasound did not add additional relevant information in monitoring treatment response over clinical examination.

Introduction/Literature Review

Lipedematous scalp (LS) is a rare condition characterized by thickened adipose in the subcutaneous layer of the scalp resulting in a soft, spongy, or thick consistency of the scalp [1]. When associated with a lack of hair, this condition is called lipedematous alopecia (LA). LA and LS are said to be different spectrums of the same disease [2]. Females are predominately affected [1]. Symptoms of LS include paresthesia, headache, burning sensation, and thickening of the scalp with localized or generalized sensitivity of the scalp or itching [1]. Various imaging modalities such as magnetic resonance imaging (MRI), ultrasonography (US), and computed tomography (CT) have been used to diagnose LS and LA along with histopathology. Here, we discuss biopsy and ultrasound findings as well as review the literature for imaging techniques for diagnosis. Scalp imaging with a high-resolution transducer and color Doppler imaging techniques can be used to evaluate the thickness of the dermal and subcutaneous tissues, as well as the scalp-calvarium interface [3].

Case Report/Case Presentation

Case 1

A 56-year-old female presented with a 1-year history of hair thinning at the vertex scalp with localized pain and tenderness in the area. There was no associated scale or pruritus. On physical examination, the scalp was noted to be boggy and tender to palpation with minimal erythema in the vertex. She underwent biopsy showing slight miniaturization of hairs without scarring or inflammation (Fig. 1). US of the scalp showed gradual increased thickness without presence of a focal mass measuring 3 mm at the forehead and 6 mm at the crown of the head (Fig. 2). LS was suspected. At this time, the findings were not conclusive of LS. Follow-up US 7 months later showed a gradual increase in tissue thickness at the area of palpable transition without evidence of a focal mass or fluid collection. She was treated with mycophenolate mofetil 1 g daily and topical minoxidil. She noticed improvement in hair density and scalp pain, but repeat measurements of the scalp revealed an increase in scalp thickness (5 mm at the forehead and 1.1 cm at the crown). Mycophenolate mofetil was discontinued at this time, and the patient reported return of scalp pain.

Fig. 1.

Histologic findings of the patient from case 1. a, b Scalp biopsy showing slight miniaturization of hairs without scarring or inflammation.

Fig. 2.

High-resolution ultrasound images using a Philips EPIQ EL18-4 transducer. Case 1: a Gray scale image showing markedly thickened subcutaneous tissue to the right of the midline, extending anterior to posterior in the longitudinal (sagittal) plane. b Perfusion imaging showing a small amount of flow in the markedly thickened subcutaneous tissue to the left of the midline, transverse plane (color). Case 2: c Gray scale image of the forehead in the longitudinal (sagittal) plane shows markedly thickened subcutaneous tissue, increased at the crown (X cursors) and slightly decreased at the forehead (+cursors). d Perfusion imaging in the transverse plane at the crown shows a small amount of flow in the subcutaneous tissue (color).

Case 2

A 60-year-old female with a 5-year history of lichen planopilaris presented with a 1-year history of hair localized itching and soreness on the crown of her head. On examination, she was noted to have diffuse scarring alopecia with perifollicular erythema and scale and bogginess of the scalp. Biopsy showed no active inflammation with normal hair count and slight miniaturization of the hair units (Fig. 3). Scalp US revealed 1.1-cm thickness of the right scalp subcutaneous soft tissue and up to 1.2 cm of the left scalp subcutaneous soft tissues (Fig. 2). These measurements confirmed the diagnosis of LS. Prior treatments had included potent topical steroids and doxycycline hyclate. She was restarted on 3 months of doxycycline and a potent topical steroid. Hydroxychloroquine 200 mg BID was added to her regimen. The patient reported decreased scalp pain after 6 months; however, there was no clinical change in scalp thickness after treatment.

Fig. 3.

Scalp biopsy from case 2. a, b No active inflammation with preserved sebaceous lobules. No shift in the number of catagen/telogen hairs and slight miniaturization of the hair units.

Discussion

The cause of LS is largely unknown. It is postulated that there is some hormonal role in the pathogenesis of the disease based on the increased prevalence in females [4]. LS was initially thought to primarily affect black patients, but there has been an increase in the number of reported cases in white and Asian patients suggesting race has less of an impact on development of the disease [4].

There have been an increasing number of case reports documenting the various modalities for diagnosis of LS (Table 1). Table 1 shows the increasing supplementary use of MRI, CT, and US for the diagnosis of LS. Previously, the diagnosis of LS was focused on findings from histopathology; however, more studies are revealing biopsy may not be necessary in all cases. Notably, no imaging modality can determine the etiology of the thickened subcutaneous fat tissue which is a limitation in their use in the diagnosis of LS. One study by Kavak et al. [5] suggested that US was adequate for the follow-up evaluation of LS patients. These findings are supported in other case reports such as the one by Müller et al. [6], which stated that the use of all diagnostic modalities (MRI, US, CT, and histopathology) depends mainly on the subjective psychological impact on the patient. Our patient in case 1 had inconclusive biopsy results leading to the use of US for confirmation of LA and scalp thickness monitoring. In case 2, US was used to identify LS in the setting LPP. During treatment, however, we found that monitoring treatment response with US was not helpful as there was reported symptom improvement and hair growth by the patients, despite continued abnormal subcutaneous fat thickening measured at ultrasound.

Table 1.

Imaging modalities used in the diagnosis of LS

Author	Age, years, sex	Disease	Imaging modality(s) used	Histopathological findings	Scalp thickness, mm
Yaşar et al. [4]	62 F	LS	MRI	Increased subcutaneous fat layer and hyperkeratosis, epidermal atrophy, and scant perivascular mononuclear infiltrate	18
	45 F	LA	MRI	Increased subcutaneous fat layer and epidermal atrophy with coarse collagen degeneration in the superficial dermis	10
	49 M	LA	MRI	Increased subcutaneous fat layer with mild perivascular lymphatic infiltration	12
Lee et al. [7]	32 F	LA	CT	Increased subcutaneous fat layer	10.7
Kavak et al. [5]	50 F	LS	US, CT,MRI	Increased subcutaneous fat layer with mild perivascular lymphatic infiltration and marked hyperplasia of subcutaneous adipose tissue with mature adipocytes	8.6
Fernández-Torres et al. [8]	55 F	LS	MRI	Increased subcutaneous fat layer with dilated lymphatic vessels in the upper dermis	22
Peter et al. [9]	57 F	LS	X-ray	Encroachment of thickened subcutis into the dermis	16.2
	40 F	LS	CT	Encroachment of thickened subcutis into the dermis	22.4
Wang et al. [10]	20 M	LA	CT	Increased subcutaneous adipose tissue and mild perivascular mononuclear infiltration into the superficial dermis with perifollicular mononuclear infiltration accompanied by perifollicular fibrosis	15
Mansur et al. [11]	46 F	LS	MRI	Increased subcutaneous fat layer	13
Bosschaert and Deprez [12]	16 F	LS	MRI, US	Marked hyperplasia of subcutaneous adipose tissue with mature adipocytes	9
Fernández-Canga et al. [13]	70 F	LS	US, CT	Increase in thickness of subcutaneous adipose tissue with edema extending into the dermis but without inflammatory infiltrates	9
Gönül et al. [14]	31 F	LA	US	Sparse, perivascular lymphocytic infiltrate and a dramatically expanded subcutaneous layer, prominently diminished follicles, lymphangiectasias, and mild perifollicular infiltration	12.5
González-Guerra et al. [15]	52 F	LA	CT	Increase in thickness of the subcutaneous fatty tissue	15
Wylie et al. [16]	56 F	LS	MRI	Thickening of the subcutaneous tissue due to expansion by fat, with some disruption of the normal fat architecture and more superficial merging of fat with the dermis	12
Accaputo et al. [17]	66 F	LA	MRI	Expansion of subcutaneous tissue accompanied by infiltration of fat into the superficial dermis	12
Yorulmaz et al. [18]	45 F	LA	n/a	Mild perifollicular fibrosis, dilated lymphatics, thickening of collagen bundles, increased subcutaneous adipose tissue, and mild mucin deposition	n/a
Piraccini et al. [19]	48 M	LA	n/a	Subcutaneous adipose tissue was thickened and mildly edematous	11
	52 M	LA	n/a	Subcutaneous adipose tissue was thickened and mildly edematous	12
Sahu et al. [20]	50 F	LS	MRI	Increased subcutaneous fat layer	16
	45 F	LS	MRI	Increased subcutaneous fat layer	20
	29 M	LS	MRI	Increased subcutaneous fat layer	15
	45 F	LS	MRI	Increased subcutaneous fat layer	17
Kilinc et al. [21]	33 F	LS	US, MRI	Increased subcutaneous fat layer	12
	36 F	LA	CT	Mild perivascular inflammation composed of lymphocytes and eosinophils	11
Martínez-Morán et al. [22]	77 F	LS	CT	Subcutaneous tissue thickening that even extended to the dermis	15.2
Carrasco-Zuber et al. [23]	48 F	LS	US	Thickened edematous subcutis without any inflammatory infiltrate	10
Cunha et al. [24]	13 F	LS	US	Increased subcutaneous fat layer with focal perifollicular inflammatory infiltrate and edema	9.1
Bukhari et al. [25]	57 F	LS	CT	Increase in the thickness of the subcutaneous fat layer with normal epidermal and dermal layers; mild superficial perivascular lymphocytic infiltrate with no accumulation of mucin in the dermis or subcutaneous tissue	21.2
Müller et al. [6]	15 M	LS	n/a	Increased subcutaneous fat layer	n/a
Chen et al. [26]	46 F	LA	CT	Decreased hair follicle density and a pauci-inflammatory process with thickening of collagen bundles and increased space between bundles suggestive of edema	14
Martín et al. [27]	48 F	LS	US	Increased subcutaneous fat layer	10.8
	77 F	LA	US	Increased subcutaneous fat layer with dilated dermal lymphatic vessels	11
	59 F	LS	US	Increased subcutaneous fat layer with mild edema and dilated lymphatic vessels	9.2
Al Gaadi et al. [28]	59 F	LS	CT	Increased subcutaneous fat layer	14.4

LS, lipedematous scalp; LA, lipedematous alopecia; MRI, magnetic resonance imaging; US, ultrasonography; CT, computed tomography.

No prior case reports have reported concurrent LPP with LS to our knowledge; however, the corresponding author has seen soft spongy scalps in patients with cicatricial alopecias in her practice. Further investigation is needed to determine if there is a relationship between scarring alopecias and LS.

Few successful treatments of LS or LA have been identified. Recent studies reported improvement with 10 months of mycophenolate mofetil 1 g/day [29] and surgical debulking of the scalp [30]. The patient from case 1 was treated with mycophenolate mofetil which resulted in hair growth and improved symptoms but worsened scalp edema. The patient from case 2 was treated with a combination of hydroxychloroquine, betamethasone ointment, and doxycycline. She reported some hair growth and decreased hair loss with this regimen. There was no change in scalp thickness. US is cost-effective and has been reported as a helpful diagnostic tool in the diagnosis of LS; however, we found that it was not helpful in the monitoring of treatment response. The lack of clinical change in scalp thickness does not necessarily mean that US is not useful for monitoring LS, but that US is correlated with the clinical finding of no change in scalp thickness. It appears treatment response was better approximated by hair growth and symptom alleviation. In conclusion, US is useful in aiding in the initial diagnosis of LS but provides little to no additional benefit when coupled with symptom monitoring.

Statement of Ethics

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. Ethical approval was not required for this study in accordance with national guidelines.

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

This manuscript did not receive any funding.

Author Contributions

Sydney Weir contributed to the conception of the work and aided in drafting and revising the article. Olufolakemi Awe contributed to the conception of the work and aided in drafting and revising the article. Michelle Robbin contributed to the conception of the work and aided in drafting and revising the article. Tiffany Mayo contributed to the conception of the work and aided in drafting, revising, and providing final approval.

Data Availability Statement

All data generated or analyzed during this study are included in this article. Further enquiries can be directed to the corresponding author.