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.     

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.     

Encephalocraniocutaneous Lipomatosis (Haberl and syndrome): A Case Report and Review of Literature

Encephalocraniocutaneous lipomatosis (ECCL) is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Dermatological examination showed alopecia on right side of the scalp and ipsilateral limbal dermoid and nodular skin tags over the upper eyelid. The computerized tomography scan of the brain revealed porencephalic cyst, cerebral calcifications and atrophy of right brain. The histopathology of the skin lesions showed lipomatous hamartoma and features of non scarring alopecia. The constellation of these findings and in adherence to the diagnostic criteria of ECCL proposed in 2009, we consider this report as a definite case of ECCL.     

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.     

A case of lipedematous alopecia occurring in a male patient

A 30-year-old Japanese man presented with a thickening of the scalp and diffuse alopecia on the vertex. A skin biopsy specimen of the thickened scalp showed an increased thickness of subcutaneous adipose tissue and a marked decrease in the number of hair follicles. By an MRI examination, the irregularly thickened subcutaneous fatty tissue was visualized. These findings indicated that the patient had been suffering from lipedematous alopecia. This is a rare condition characterized by diffuse alopecia due to the thickening of the layer of adipose tissue covering the scalp. Only 4 female cases of lipedematous alopecia have been reported previously. MRI is thought to be useful for the differentiation of lipedematous alopecia from lipoma. The present case is considered to present the first male patient with lipedematous alopecia.     

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。     

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。     

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.     

Pleomorphic fibroma of the skin with MDM2 immunoreactivity: A potential diagnostic pitfall

Pleomorphic fibroma is a rare benign cutaneous neoplasm characterized by spindle‐shaped cells and multinucleated giant cells scattered throughout collagenous stroma. These morphologic features can lead to diagnostic confusion, including atypical lipomatous tumor as one consideration. In contrast to atypical lipomatous tumor, previous studies have found pleomorphic fibroma to be negative for MDM2 immunohistochemical staining and MDM2 gene amplification. Here, we present a case of pleomorphic fibroma of skin with nuclear MDM2 immunoreactivity in the absence of MDM2 gene amplification, underscoring the superiority of fluorescence in situ hybridization as a diagnostic test in this differential diagnosis. The RB1 locus is also explored for differential diagnosis with pleomorphic/spindle cell lipoma and related entities.     

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。     

Pleomorphic fibroma and dermal atypical lipomatous tumor: are they related?

Pleomorphic fibromas represent dome‐shaped or polypoid cutaneous lesions characterized by a paucicellular and densely fibrotic background punctuated by scattered atypical to pleomorphic spindle and multinucleated giant cells. Some of these tumors will have incorporated adipose tissue, although these adipocytic areas lack distinct cytologic atypia and may represent entrapped normal periadnexal or subcutaneous adipose tissue. Nonetheless, owing to the similarity of some of the morphologic features of pleomorphic fibroma with cutaneous atypical lipomatous tumor, diagnostic confusion can ensue. The potential diagnostic challenges are further highlighted by a recent report of a lesion with histopathologic features of both. In response, we studied the presence of 12q15/ MDM2 amplification by fluorescence in situ hybridization and MDM2 expression by immunohistochemistry in a series of 15 pleomorphic fibromas to investigate whether these two entities share a common pathogenic origin. One case of cutaneous atypical lipomatous tumor was used as positive control for 12q15 amplification. All 15 cases were negative for MDM2 by immunohistochemistry with no demonstrable 12q15/MDM2 amplification by fluorescence in situ hybridization. Therefore, these two entities are best regarded as pathogenetically distinct. MDM2 immunohistochemistry or fluorescence in situ hybridization studies can be used to differentiate between the two if needed.     

Type 1 interferon signature in the scalp lesions of alopecia areata

Background Autoimmune attack of the bulbar region of anagen phase hair follicles by CD8+ T cells and Th1 cytokines has been proposed to result in hair loss in alopecia areata (AA). The initiating stimuli are unknown. As interferon‐α therapy may trigger AA, we propose that type 1 interferons are involved in the induction of disease. Objectives To compare lesional scalp from patients with AA with scalp lesions of cutaneous diseases associated with local type 1 interferon‐related protein expression. Methods Lesional scalp of patients with AA, discoid lupus erythematosus, lichen planopilaris and androgenetic alopecia was examined by immunohistochemistry for expression of the type 1 interferon‐inducible myxovirus protein A (MxA), the chemokine receptor CXCR3, and the cytotoxic proteins granzyme B (GrB) and T‐cell intracytoplasmic antigen 1 (TiA‐1). Results MxA was expressed in the intradermal and subcutaneous compartments of the hair follicle including sebaceous glands in inflammatory AA similar to lesions of cicatricial alopecia (discoid lupus erythematosus, lichen planopilaris) but not in the epidermal compartment of AA, and not at all in noninflammatory AA or androgenetic alopecia. The location of CXCR3‐expressing cells correlated with MxA expression. The inflammatory cells around the hair follicle in AA included a lower number of GrB+ and TiA‐1+ cells compared with cicatricial alopecia and demonstrated predominant TiA‐1+ expression. Conclusions We demonstrate the expression of type 1 interferon‐related proteins in the inflammatory lesions of AA. The distribution pattern of the interferon signature and cytotoxicity‐associated proteins in AA differs from cicatricial alopecia.     

'Black dots' seen under trichoscopy are not specific for alopecia areata

Background. ‘Black dots’ are macrocomedo‐like round structures localized to the follicular ostium, and are considered a specific trichoscopic feature of alopecia areata (AA). Aim. To characterize specific features of ‘black dots’, and assess their possible presence in common hair and scalp disorders. Methods. In total, 107 patients with hair loss [30 with alopecia areata (AA), 37 with androgenetic alopecia (AGA), 17 with chronic telogen effluvium (TE), 23 with other hair and scalp diseases] and 93 healthy controls were examined, using a videodermoscope with 20–70 times magnification. Results. There was a correlation between the black dots and the early acute phase of the various alopecia types with the presence of the black dots. Black dots were found in 11% (22/107) of patients with hair loss, including 53.3% (16/30) with AA; in 40% (2/5) of patients with severe chemotherapy‐induced alopecia, and in 100% of patients with dissecting cellulitis of the scalp (n = 2), hypotrichosis simplex (n = 1), and congenital aplasia cutis (n = 1). No black dots were seen in patients with AGA or TE. Conclusions. Black dots are not specific for AA, and may be present in other hair and scalp diseases.     

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 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.     

Melanocortin receptor type 2 (MC2R,ACTH receptor) expression in patients with alopecia areata

Please cite this paper as: Melanocortin receptor type 2 (MC2R, ACTH receptor) expression in patients with alopecia areata. Experimental Dermatology 2010; 19 : 1020–1022. Abstract: Human skin expresses elements of the hypothalamo‐pituitary‐adrenal (HPA) axis that function as a local stress response system. Because adrenocorticotropic hormone (ACTH) is an intermediate in the HPA axis from corticotropin‐releasing hormone (CRH) signal to cortisol secretion, MC2R that binds only ACTH may be important in the stress response of skin. We investigated the local expression of MC2R by immunohistochemistry to identify the role of ACTH/MC2R in stress‐associated alopecia areata (AA). MC2R appeared to be highly compartmentalized in scalp skin including the epidermal cells of hair follicles and epidermis, sebaceous and eccrine glands, as well as dermal fibroblasts. The expression of MC2R was lower in AA lesions than in normal scalp tissue in almost all scalp skin cells, especially in epithelial cells. These findings demonstrate that MC2R expression is aberrant in AA and suggest a deficit in ACTH/MC2R activity may play an important role in the pathophysiology of AA.     

Role of Trichoscopy in Children's Scalp and Hair Disorders

Hair and scalp disorders in children may originate from the hair itself, scalp skin, or infectious causes and be congenital or acquired. The most common sign is alopecia, frequently brought on by tinea capitis, patchy alopecia areata, or trichotillomania. Sometimes less frequent and clinically more elusive conditions such as initial androgenetic alopecia, congenital triangular alopecia, or alopecia areata incognita may be responsible for hair loss. The noninvasive technique known as trichoscopy is being used more frequently, aiding in the prompt differential diagnosis and follow‐up of many of these diseases, oftentimes providing further examination before a treatment decision is made. This review of trichoscopy of the main scalp and hair disorders afflicting children and adolescents discusses the most important dermoscopic criteria and the usefulness of this technique.     

Encephalocraniocutaneous lipomatosis: clinical spectrum of systemic involvement

Encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome with involvement of ectomesodermal tissues. Unilateral lipomatous hamartomas of the scalp and eyes are the hallmarks of this disorder. Associated findings are extremely variable, ranging from minor abnormalities to severe, debilitating disease. We report two unrelated Brazilian patients with encephalocraniocutaneous lipomatosis, one presenting with the full-blown spectrum of cutaneous, ocular, and central nervous system manifestations, and the other with minor extracutaneous manifestations after a 5-year follow-up period.     

Confocal microscopic features of scarring alopecia: preliminary report

Background Lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) are the most common causes of lymphocytic primary cicatricial alopecia. The management of scarring alopecia can be difficult. The combination of clinical, dermoscopy and reflectance confocal microscopy (RCM), a noninvasive, high‐resolution imaging technique, examinations have already been demonstrated to be useful for choosing the correct biopsy site in patients with inflammatory skin disease and obtaining microscopic diagnostic criteria. Objectives We evaluated the usefulness in practice of RCM for the identification of criteria for LPP and DLE involving the scalp and their management during therapeutic follow‐up. Methods Seven white patients with a previously established histological diagnosis of DLE (three) and LPP (four), were included in the study. RCM criteria for primary scarring alopecia were selected: epidermal disarray, spongiosis, exocytosis of inflammatory cells in the epidermis, interface dermatitis, peri‐ and intra‐adnexal infiltration of inflammatory cells, dilated vessels in the dermis, dermal infiltration of inflammatory cells and melanophages and dermal sclerosis. All patients were followed up using RCM during the treatment. During follow‐up the RCM evolution of the epidermal, junctional and dermal inflammation were evaluated. Results A series of RCM features of scalp LPP and DLE were identified that show correlation with the histopathological evaluation. During the treatment follow‐up of the cases RCM was shown to be sensitive for the identification of therapeutic response. Conclusion In our preliminary study the effective usefulness of RCM for the diagnosis of scarring alopecia and follow‐up seemed to be evident. Moreover, RCM seems to be also promising for differential diagnosis between the different entities.