|Ahead of print publication
Lipedematous scalp with varied presentations: A case series of four patients
Priyadarshini Sahu1, Bhavya Sangal1, Surabhi Dayal1, Sanjay Kumar2
1 Department of Dermatology, Venereology, Leprology, Pt. B D Sharma, University of Health Science, Rohtak, Haryana, India
2 Department of Pathology, Pt. B D Sharma, University of Health Science, Rohtak, Haryana, India
|Date of Web Publication||29-May-2019|
Department of Dermatology, Venereology and Leprology, Pt B. D. Sharma, University of Health Sciences, Rohtak - 124 001, Haryana
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Lipedematous scalp (LS) is a rare cutaneous disorder of unknown etiology, characterized by significant thickening of subcutaneous tissue which results in a thick, boggy, scalp swelling. To the best of our knowledge, less than 50 cases are reported till date. Hereby, we present a total of four cases of LS,that is, two cases of LS and two cases of unusual associations of LS with alopecia areata and intradermal nevus. LS with intradermal nevus and alopecia areata are the first of its kind, not yet reported till date.So far, only two cases of LS had been reported from Indian population. Hence, our effort is to bring into notice this growing yet an uncommon condition in the Indian population.
Keywords: Alopecia areata, intradermal nevus, lipedematous scalp
|How to cite this URL:|
Sahu P, Sangal B, Dayal S, Kumar S. Lipedematous scalp with varied presentations: A case series of four patients. Indian Dermatol Online J [Epub ahead of print] [cited 2019 Jun 27]. Available from: http://www.idoj.in/preprintarticle.asp?id=259300
| Introduction|| |
Lipedematous scalp (LS) is a rare clinicopathological disorder of unknown etiology. It is characterized by significant thickening of subcutaneous tissue resulting in a thick, boggy scalp swelling, usually localized on the vertex or occipital area. In our case series, we report four cases of LS, of which two cases are with unusual association i.e., along with intradermal nevus and alopecia areata.
| Case Reports|| |
A 50-year-old female presented with asymptomatic gradually increasing swelling over the scalp since 10 years and headache since 2 years. The patient had no history of hair loss or trauma. On examination, scalp skin was normal without any alopecia, scaling, or erythema. On palpation, the scalp had a thick, soft, and spongy consistency [Figure 1]a. Magnetic resonance imaging (MRI) of brain was advised, and it revealed diffuse thickening of the scalp measuring almost 12–16mm with no brain tissue abnormality pointing towards a diagnosis of LS [Figure 1]b. Punch biopsy of scalp was consistent with our diagnosis [Figure 1]c.
|Figure 1: (a) Soft, boggy, and swollen area present over vertex and parieto-occipital area. (b) On MRI, scalp thickness varies from 12 to 16 mm without any cortical abnormality. (c) Histopathology (H and E; ×10) revealed mild hyperkeratosis of epidermis with mild dermal lymphocytic infiltrate mainly perivascular and extension of subcutaneous fat into dermis (marked with yellow arrow)|
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A 45-year-old female presented with chief complaint of gradually progressive soft swelling of her whole scalp since 6 years. Swelling was not associated with headache or with any history of trauma or hair loss at the same site. On inspection, there were no areas of alopecia, erythema, or scaling, but was associated with darkening of scalp skin. On palpation, soft swelling of spongy consistency was present on bilateral parietal and occipital region of the scalp [Figure 2]a. MRI of the brain showed diffuse thickening of scalp soft tissue varying from 10 to 20mm, with maximum bulge on the right parietotemporal region [Figure 2]b. Skin biopsy sections examined showed deep intradermal nevus along with features of LS [Figure 2]c. These nevi cells were strongly positive for S-100 and Melan A and negative for HMB-45 [Figure 2]d. This zeroed our diagnosis on LS with intradermal nevus.
|Figure 2: (a) Thickened, boggy, and spongy scalp with darkening of underlying scalp skin. (b) MRI brain showed diffuse scalp thickening varying from 16mm on right parietotemporal region. (c) Histopathological section (H and E; ×10) showed increased subcutaneous adipose tissue around the hair follicles with perifollicular fibrosis and dermal edema. (d) Photomicrograph (H and E; ×40) revealing nevi cells (marked by blue arrow)|
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A 29-year-old male visited with chief complaints of a single patch of hairloss on the scalp since 6 months which was spontaneous in onset and gradually progressive in nature. There was no history of any prior lesion at the site of hairloss. On inspection, an alopecia patch of size 3 × 3 cm2 was present at the left parietotemporal region of the scalp, without any erythema or scaling [Figure 3]a. On palpation, a soft, spongy swelling with doughy consistency was present underneath the alopecia patch. Scalp thickness of 15mm was noticed at the parietal region on MRI of the brain [Figure 3]b. On dermoscopy, features of alopecia areata (AA) were seen [Figure 3]c. The patient refused for scalp biopsy, and therefore diagnosis was made on the basis of clinical, dermoscopy, and radiological finding as LS with AA.
|Figure 3: (a) A circumscribed patch of alopecia on left parietotemporal region of scalp with soft, spongy swelling and doughy consistency. (b) MRI brain showed scalp thickness of 15mm noticed at parietal region with normal cortical findings. (c) Dermoscopic features of alopecia areata, i.e., yellow dots (marked by black arrow), black dots (marked by black circle), and broken hairs (marked by green arrow) (polarized; ×10)|
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A 45-year-old female presented with thick, boggy scalp swelling, which was insidious in onset and gradually progressive in nature since 7–8 years, with no history of trauma, headache, or hair loss at the sight of swelling. The patient also complained of pain and dysesthesia. On examination, there was a single soft, doughy swelling on the left parieto-occipital region [Figure 4]a. MRI of the brain was performed which showed scalp thickness of 15–17 mm on the left parietal region [Figure 4]b. Following this, a scalp biopsy was performed, and histopathology was consistent with the diagnosis of LS [Figure 4]c.
|Figure 4: (a) Soft thickening of scalp without any underlying abnormality. (b) MRI brain revealed scalp thickness of 15 mm on left parietal region without any cortical abnormality. (c) Histopathological section (H and E; ×10) revealed thinned out epidermis, hyperkeratosis, and mild spongiosis. The upper and mid-dermis showed mixed lymphomononuclear infiltrate with subcutaneous fat reaching upto upper dermis and entrapping the hair follicles|
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There were no signs of inflammation and hair abnormalities in any of our patient. Family history was insignificant in all the cases. Features of acromegaly, pachydermoperiostosis, or myxedema were not present in either of our patients. All routine investigations were normal in the four patients. All patients were reassured about its benign nature and asked to follow-up every 3 months.
| Discussion|| |
LS was first described by Cornbleet in 1935. Although no definite cause is defined as yet, the proposed pathogenesis includes leptin hormone which plays a role in subcutaneous fat hyperplasia and displacement of adipose tissue into dermis. Lymphangiectasia and compression of the superficial blood vessels are two mechanisms that can lead to varying degree of alopecia and shortening of hair in cases of LS. In addition, pressure on dermal nerves by dermal edema and thickening can be the cause behind headache and dysesthesia in our patients.
According to an Indian study, the mean scalp thickness on MRI at the frontal, parietal, and occipital areas is 5.5 mm (2.8–8.1 mm), 7.1 mm (4–11 mm), and 7.75 mm (5–11 mm), respectively. The scalp thickness in patients with LS ranged from 9.2 to 16 mm. According to these data, the scalp thickness was increased in all our patients. The majority of the cases were described in middle-aged females with insignificant medical history. Similar findings were seen in our case series also, where three out of four patients were middle-aged females. From India, only two cases of LS without any other associations had been reported. This entity has been earlier reported in adult black females and Japanese women; however, we believe that either its occurrence in the Indian population has risen or this condition was so far under reported.
As of now, treatment protocol for this entity has not yet been published. Intralesional steroids and systemic steroids have been used with no promising results. A single study each on debulking and scalp reduction and use of mycophenolatemofetil in a case of lipedematousalopecia has given good results. In our cases, we reassured our patients, considering the probability of persistence of this condition. Two out of the four cases presented with unusual presentation. LS with intradermal nevus opens door to one possibility that nevus can also lead toLS or vice versa. Second, coexistence of well-defined alopecic patch with LS with specific dermoscopic features cannot rule out the possibility of occurrence of AA with LS. Thus, the association of LS with AA is a coincidence or co-relation is yet to be defined. Hence, our aim of presenting this case series is to bring into light theoccurrence of LS in Indian population along with some of its unusual associations which have never been discussed before.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]