Hyperplasia of the subcutaneous adipose tissue is the primary histopathologic abnormality in lipedematous scalp

A 51-year-old white woman presented with thickening of the scalp located at the vertex and left lateral occiput without hair abnormalities or alopecia. Skin biopsies of the thickened scalp showed thickening of the subcutaneous tissue with proliferation of mature subcutaneous fat cells but no signs of inflammation or hair abnormalities. During 2.5 years of follow-up, scalp thickening progressed over the entire hair-bearing scalp and persisted without signs of further progression at 3.5 year follow-up. Lipedematous scalp is an extremely rare diagnosis. It is defined by a thickening of the subcutaneous layer of the scalp and can be distinguished from lipedematous alopecia, in which subcutaneous thickening is associated with diffuse alopecia and shortening of scalp hairs. A total of seven cases of lipedematous alopecia and two cases of lipedematous scalp have been reported. We report the third case of lipedematous scalp in a 51-year-old white woman associated with early symptoms of meningitis. Additional features described in the literature include pruritus, pain, and paresthesia of the scalp as well as associated medical problems such as hyperelasticity of skin and laxity of joints, renal failure, and diabetes mellitus. This sporadic disorder is predominantly located at the vertex and occiput. The etiology and pathogenesis of lipedematous scalp and alopecia remain unclear. The treatment is symptomatic.     

脂肿性头皮伴脂肿性脱发一例

患者,女,52岁。头皮肿胀增厚伴脱发3个月余就诊。皮肤科查体：头皮明显肿胀增厚,头顶部增厚区域界限清楚的脱发斑。皮肤镜检查见脱发区毛囊明显减少,毛囊周围见褐色环状结构。组织病理示：皮下脂肪组织明显增厚,位置上移,由成熟脂肪细胞组成。诊断：脂肿性头皮及脂肿性脱发。     

Lipedematous alopecia of the scalp

Lipedematous scalp is a rare disorder, mainly described in adult African-American females. We report 2 adult caucasian males with lipedematous scalp associated with androgenetic alopecia. Patients were studied by dermoscopy and histopathology; they were treated with finasteride 1 mg. In our patients, lipedematous scalp affected the occipital and the vertex areas and pathologically exhibited mild edema and thickening of the adipose subcutaneous layer. At videodermoscopy, lipedematous scalp areas showed linear areas of teleangiectasia within the scalp creases, possibly caused by compression of the superficial blood capillaries by the increased volume of the subcutaneous fat layer within the thickened scalp. Finasteride at a dose of 1 mg per day for 1 year induced mild improvement of androgenetic alopecia in one patient and stabilization of the disease in the other. The lipedematous scalps remain unchanged. Lipedematous scalp is apparently a rare disease even though the condition is probably underdiagnosed. As a matter of fact, we diagnosed lipedematous scalp in our patients during a clinical examination for androgenetic alopecia, which was the patients' complaint. The association of lipedematous scalp and androgenetic alopecia in our two patients appears to be coincidental.     

脂水肿性脱发1例

 报告1例脂水肿性头皮伴脂水肿性脱发。患者女,44岁,因头皮肿胀、增厚伴脱发5年余就诊。皮肤专科检查：患者头顶部可见一面积约9 cm×18 cm的梭形毛发稀疏区域,残留断发＜2 cm,脱发区头皮呈黄褐色,稍隆起,明显增厚,触之柔软,似海绵状;拉发试验阴性。头部MRI示头皮下脂肪层不均匀增厚。皮损组织病理示：毛囊数量减少,毛囊由增生的纤维组织替代,少许淋巴细胞浸润。诊断为脂水肿性头皮伴脂水肿性脱发。患者及家属拒绝治疗,随访半年余,病情未进展。     

Lipedematous alopecia: spongy scalp syndrome

Lipedematous scalp and lipedematous alopecia are rare and closely related entities of unknown etiology Almost all cases have occurred in black women. We report a case of lipedematous alopecia that is characterized by a boggy spongy thickening of the scalp, an increase in subcutaneous fat, short hairs, patchy alopecia, scarring, atrophy, and depigmentation. The histologic finding of an increase in subcutaneous adipose tissue is notable.     

Cuero cabelludo lipedematoso

Lipedematous scalp is a rare condition first described by Cornbleet in 1935. An increased thickness of subcutaneous tissue in the scalp gives rise to a soft spongy appearance of the surface and occasionally causes pruritus and pain in the affected area. When hair loss is also associated with the condition, it is described as lipedematous alopecia. To date, 10 cases of lipedematous scalp and 13 of lipedematous alopecia have been reported.We present the case of a 77-year-old white women who developed dysesthesia on her scalp 5 months after the death of her husband. Biopsy revealed subcutaneous tissue thickening that even extended to the dermis. Computed tomography showed thickening of subcutaneous tissue at the vertex and in the occipital region. We diagnosed a new case of lipedematous scalp in a white women. This case highlights the importance of differential diagnosis in cases of dysesthetic syndrome of the scalp.     

Lipedematous scalp in a child

A nine year old Maori girl was noted to have a spongy consistency to her posterior scalp. The overlying skin appeared normal, as was hair growth in that area. The abnormal findings became more extensive over the subsequent year. A computerized tomography scan showed diffuse thickening of subcutaneous tissues posteriorly on the scalp. A skin biopsy specimen showed a normal epidermis with a thick layer of mature subcutaneous fat and some disruption of the subcutaneous architecture. The clinical features and investigations were consistent with a diagnosis of lipedematous scalp, which has been described predominantly in adult women and is usually associated with alopecia. This condition has not been reported previously in the pediatric literature.     

Lipedematous Alopecia in an Asian Woman: Is It an Advanced Stage of Lipedematous Scalp?

Lipedematous alopecia (LA) is a rare disease entity that manifests as increased thickness of subcutaneous fatty tissue in the scalp with a variable degree of acquired, non-scarring alopecia. Although the pathogenesis of alopecia in LA is not clear, it is assumed that the increased thickness of subcutaneous fat retards hair growth by disturbing the peripheral microenvironment of the hair bulb. LA is clinically distinguishable from lipedematous scalp (LS) in that LS is not associated with any hair abnormalities, while LA is characterized by variable degree of hair loss. However, both LA and LS share increased scalp thickness. Here, we describe a rare case of LA on the frontal scalp of an Asian woman, further characterize the clinical and histologic features, and suggest applying an integrated diagnosis as lipedematous diseases of the scalp.     

Congenital Lipedematous Alopecia: Adding to the Differential Diagnosis of Congenital Alopecia

Lipedematous alopecia is a rare condition of unknown etiology characterized by a thick boggy scalp with varying degrees of hair loss. It is usually seen in adult African-American females, and a case in a 9-year-old was the youngest patient reported thus far. We report on the appearance of this condition in two children, a 6-year-old child and a 10-year-old child. Each presented with congenital patchy hair loss on the occipital area and the left temple. A boggy hairless scalp with soft swelling was detected in both patients. Histological examination showed increased thickness of the subcutaneous fat tissue with a decrease in hair follicles. These features were consistent with a diagnosis of lipedematous alopecia. We report two cases of congenital lipedematous alopecia, which has not been reported previously. Although congenital, these distinct clinical features should be kept in mind in the diagnosis of alopecic hair loss.     

Lipedematous alopecia: a clinicopathologic, histologic and ultrastructural study

Lipedematous alopecia is a rare condition of unknown etiology characterized by a thick, boggy scalp with varying degrees of hair loss that occurs in adult black females, with no clearly associated medical or physiologic conditions. The fundamental pathologic finding consists of an approximate doubling in scalp thickness resulting from expansion of the subcutaneous fat layer in the absence of adipose tissue hypertrophy or hyperplasia. Observations by light and electron microscopy detailed in this report suggest that this alteration principally manifests by localized edema with disruption and degeneration of adipose tissue. Some diminution in the number of follicles as well as focal bulb atrophy is noted. Aberrant mucin deposition such as that seen in myxedema or other cutaneous mucinoses is not a feature. The histologic findings bear some resemblance to those seen in lipedema of the legs, a relatively common but infrequently diagnosed condition. We present a case of lipedematous alopecia with emphasis on histologic and ultrastructural features. The etiology is unknown.     

Successful surgical management of lipoedematous alopecia

A 67-year-old Caucasian woman presented with an area of alopecia over the right occipital scalp, which had slowly expanded over the last 10 years. The skin beneath the alopecia felt soft and boggy although the epidermis looked unremarkable. Ultrasound showed thickening of the underlying subcutaneous tissue. Scalp histology showed enlarged fat lobules within the subcutis that infiltrated along fibrous tracts into the mid-dermis. There was a complete loss of hair follicles. These findings were consistent with lipoedematous alopecia of the scalp. Surgical debulking with scalp reduction produced an acceptable result in our patient with no evidence of relapse after 12 months.     

Lipedematous alopecia

Lipedematous alopecia is an unusual disorder characterized by short, brittle scalp hairs associated with thickening of the scalp by adipose tissue. All except one reported case have been in African-American females. It is our purpose to report the seventh case of this condition.     

Clinical and pathological features of 31 cases of lipedematous scalp and lipedematous alopecia

Little is known about lipedematous scalp (LS) and lipedematous alopecia (LA). We investigated the clinical and histopathological features of LS and LA with a 7-year retrospective re-evaluation of 31 patients. 23 cases were LS and 8 LA, with 25 females and 6 males. The overweight and obese groups contained 15 patients with 16 within the normal weight range. Scalp thickness varied between 9-18 mm in our patients by magnetic resonance imaging. Thickening of the subcutaneous adipose tissue layer was present in all cases. Dermal edema was seen in 22 patients, lymphatic dilatation in 17 and elastic fiber fragmentation in 21. When the relationship between dermal edema and elastic fibers was investigated, elastic fiber fragmentation was found in 86.4% of cases with dermal edema. Collagen fragmentation and coarsening were seen in two cases, and collagen was normal in 24 cases. The number of follicles was decreased in 9 cases and normal in 17. The clinical and histopathological findings were not statistically different between LS and LA groups (p>0.05). The majority of the patients in our study were females, suggesting an underlying hormonal pathology. The association with obesity suggested that anatomical differences can be present in lipid distribution. Dermal edema and lymphatic dilatation suggested the primary pathology is lymphatic system.     

脂水肿性脱发1例

患者女,62岁,头皮肿胀且局部横沟伴压痛半年。皮损组织病理示:表皮大致正常,真皮浅中部血管周围少量淋巴单核细胞浸润,胶原间纤维母细胞及胶原增生,真皮中下部毛囊周围见成熟脂肪细胞增生;黏蛋白染色示:毛囊周围及毛乳头见少量黏蛋白沉积。综合临床和病理表现,该患者诊断为脂水肿性脱发,脂水肿性头皮。     

Lipedematous scalp and lipedematous alopecia: report of three cases in white adults

Lipedematous scalp (LS) and lipedematous alopecia (LA) are rare conditions of unknown etiology characterized by a thick and boggy scalp due to increase in the subcutaneous fat layer. Besides the changes in the texture of skin, varying degrees of hair loss are seen in patients with LA. In this report, we present two cases with LA and one case with LS in white adults. On examination, a boggy and soft swelling of the scalp was detected in all patients. In addition, the patients with LA had alopecic areas over the boggy scalp. Histopathologic examinations of skin biopsy specimens and magnetic resonance imaging of the scalp showed increased thickness of subcutaneous fat tissue in all patients. The exact etiopathogenesis of LA and LS remain unknown. These conditions may be either reactive or compansatory responses versus yet undetected stimuli, or represent a genetic tendency. Both of the these entities have been initially reported mostly in adult black females, however, we believe that LA and LS may well occur in white subjects and may be more widespread and frequently observed than previously supposed.     

Tumefactive lipedema with pseudoxanthoma elasticum-like microscopic changes

BACKGROUND: Lipedema is a condition characterized by diffuse, bilaterally symmetrical, painful swelling of the legs and buttocks. Microscopically, there are dermal and septal edema, adipocyte degeneration, and numerous mast cells, features held in common with lipedematous alopecia. CASE REPORT: We present the case of a 60-year-old woman with a long history of bilateral leg masses with microscopic features of lipedema. In addition, elastic-fiber changes typical of pseudoxanthoma elasticum (PXE) were discovered within the subcutaneous septa in three separate specimens obtained from an affected extremity. The patient did not have other clinical findings of PXE, although there was a history of both hypertension and congestive heart failure. CONCLUSION: This tumefactive presentation of lipedema has not been previously described. Regarding the elastic-tissue abnormalities, the patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic-tissue changes resulting from the massive edema. If the latter is the case, then this could represent an unusual manifestation of localized acquired cutaneous PXE (calcific elastosis).     

Lipedematous alopecia: an uncommon clinicopathologic variant of nonscarring but permanent alopecia

A 52-year-old black woman presented with a 5-year history of gradual swelling and slowed hair growth involving the vertex and both parietal regions of the scalp. Gradually, the swelling progressed to involve the entire scalp, only sparing a peripheral crown. She reported no history of trauma or medications. Slight pruritus of the involved area was the only accompanying symptom. There was no family history of a similar condition. Her past medical history included surgery for ovarian cysts, 10 years previously, and cholelithiasis. Physical examination revealed diffuse hair thinning and alopecia, more prominent along the vertex and parietal regions (Fig. 1a). There was no evidence of scalp inflammation, scaling, or increased hair fragility. The scalp was mildly tender on palpation and had a boggy, spongy consistency. The hairs which still remained in the involved areas were thin, short, and soft (Fig. 1b). The involved area was slightly hypopigmented when compared with adjacent noninvolved scalp. The rest of the physical examination was within normal limits. No abnormalities of the hair shaft were observed on microscopic examination of several plucked hairs. Laboratory investigations, including a complete blood cell count, blood chemistry, urinalysis, sedimentation rate, antinuclear antibodies, and serologic tests for syphilis, hepatitis B and C virus, and human immunodeficiency virus, were negative. A computed tomography scan of the skull demonstrated diffuse and regular thickening of subcutaneous fatty tissue of the scalp, disclosing a maximum scalp thickness of 15 mm at the vertex (Fig. 2). The biopsy from the vertex revealed a normal epidermis and dermis, with diffuse loss of hair follicles. The most striking feature consisted of a large increase in thickness of the subcutaneous fatty tissue (Fig. 3a). Pre-existing hair follicles were replaced by vertical fibrous tracts of lamellar fibroplasia with no inflammatory infiltrate (Fig. 3b). Adipocytes showed a normal size and shape, but the connective tissue septa, which are normally present separating the subcutaneous tissue into fat lobules, were lacking, and subcutaneous fatty tissue consisted of a continuous and diffuse sheet of mature adipocytes (Fig. 3c). Orcein stain revealed normal contents of elastic fibers with foci of condensation at the sites of disappeared pre-existing hair follicles (Fig. 3d). Colloidal iron and Alcian blue (pH 2.5) stains revealed no mucin deposits.     

Lipedematous scalp: a rare entity

Lipedematous scalp is characterized by increased subcutaneous thickness of the scalp without any hair change. A 50-year-old female presented with thickening of the scalp without alopecia. She had no disorder other than hypertension. Monthly sonographic measurements without any treatment showed a resolution tendency in some areas and an increased thickness in others. We discuss the characteristics of this rare and possibly underestimated entity, including differential diagnoses.     

以斑状脱发为首发症状的弥漫性神经纤维瘤一例

患者男,42岁,因头部斑状脱发伴头皮肿胀2年余就诊。皮肤科检查：左侧头皮见一10cm×8cm的肿块,境界清楚,略高起皮面,表面毛发脱落,触之柔软。皮损组织病理检查：肿瘤位于真皮深部和皮下脂肪组织,主要由短梭形细胞构成,可见特征性Meissner小体样结构;肿瘤基质疏松,散在肥大细胞。免疫组化：50％肿瘤细胞表达S-100。诊断：弥漫性神经纤维瘤。确诊后,鉴于皮损累及头部面积较大,患者拒绝手术治疗,目前仍在随访中。