The lipedematous scalp was first described by Cornbleet in 1935 [1]. This entity of unknown etiology is described as thickening of the scalp subcutaneous tissue without capillary changes, usually accompanied by local pain and less commonly by paresthesia and pruritus [2]. The lipedematous alopecia is a similar disease associated with varying degrees of hair loss and/or inability to maintain hair growth above 2 cm, although there is no consensus if they can be spectra of the same disease [3] [4].

The objective of this study is to report the case of a patient with ankylosing spondylitis who showed clinical and histological features compatible with lipedematous scalp.

A 63-year-old white female started about three months earlier to feel pain like grip in the scalp worsened by touching. She noticed diffuse scalp thickening. The patient was previously diagnosed with ankylosing spondylitis and was positive for HLA B27. She was in use of etanercept 50 mg weekly since 2011 and prednisone 7.5 mg/day, with no joint or axial complaints. The patient denied hair pulling or excessive manipulation of the hair or scalp, local trauma, use of topical treatment or family history of similar condition. She did not notice any hair changes. Physical examination revealed diffuse thickening of the scalp with consistency of layered gauze or cotton batting, painful on palpation. There was no visual evidence of local inflammatory signs or increased hair fragility. The "pull test" was within normal limits and no abnormalities of the hair shaft were seen by light microscopic examination. Complete blood cell count and biochemical parameters were within normal limits. Metabolic screening was negative, inflammatory tests were normal, serum protein electrophoresis showed no paraprotein and research of antinuclear antibodies (ANA) and rheumatoid factor were negative. Magnetic resonance imaging (MRI) scan with focus to the scalp showed diffuse thickening of subcutaneous tissue involving the scalp, measuring 17 mm in the occipital region. The study of brain parenchyma showed a small area of hyperintensity on T2 without restriction on broadcasting in fluid attenuated inversion recovery (FLAIR) sequence in periventricular white matter consistent with microangiopathy (Figure 1). Histopathological analysis of the scalp showed thickening of the subcutaneous tissue with relative thinning of the dermis, marked edema of the dermis, vascular ectasia with discrete lymphocytic infiltrate (Figure 2). She was treated with common analgesics and NSAIDs, but no improvement of symptoms was noticed and there was no change in the thickness of the scalp, which remained stable.

We described a patient with ankylosing spondylitis who developed lipedematous scalp. There is no clear association between lipedematous scalp and a specific medical disorder and its pathogenesis remains unclear [2] [5]. There is ongoing debate involving hormonal factors, since most of the reported cases occurred in women and is proposed an association with obesity [3] [5] [6]. There is no clear association between this pathology and medical or physiologic conditions, but lipedematous alopecia was reported in a patient with skin and nail psoriasis, an autoimmune disease as the presented case [7]. The thickness of the normal scalp ranges from 0.4 to 6 mm [2]. This measurement can be performed by ultrasound, MRI, tomography or fine needle and the extent found in studies have ranged from 10–22 mm, compatible with the finding of 17 mm in the present case [3]. Histopathologic findings are nonspecific, being described thinning of the dermis, increased subcutaneous fat tissue extending to the superficial dermis in some cases, inflammatory infiltration with lymphocyte predominance, as demonstrated in this study, ectasic lymph vessels [5] [6] [8]. Some showed hyperkeratosis, but the distribution of hair follicles is normal and that differentiates lipedematous scalp from lipedematous alopecia [3]. Few studies have described about the treatment and there is no consensus regarding treatment options. Most of the studies describe the use of non-steroidal anti-inflammatory drugs (NSAIDs) and symptomatic for complaints of numbness, pruritus and pain [7]. It was reported the use of hydroxychloroquine and topical corticosteroids with little improvement of symptoms and relapse in patients with lipedematous alopecia [7] [8].

Some patients were followed without any treatment, showing that some areas may become more thickened over the time [5]. Attempts with corticosteroids local infiltration did not showed influence in reducing scalp thickening [9]. Recently, a case report described the use of mycophenolate mofetil with thickness reduction of scalp and improvement hair growth and density in lipedematous alopecia [10]. Additional studies about the pathophysiology, risk factors and mechanisms involved in its appearance are needed, so that specific therapeutic approach could be developed because of the rarity of this entity.

The lipedematous scalp is still a disease that remains with its pathophysiology poorly defined. This patient had an autoimmune disease and it might serve as a trigger for the disease because it was already demonstrated an association between lipedematous alopecia, a very similar condition, and psoriasis also, in a fill cases was noted some improvement with the use of mycophenolate mofetil.

1. Cornbleet T. Cutis Verticis Gyrata? Lipoma? Arch Dermatol Syphilol 1935;32:688.    
2. Martín JM, Monteagudo C, Montesinos E, Guijarro J, Llombart B, Jordá E. Lipedematous scalp and lipedematous alopecia: A clinical and histologic analysis of 3 cases. J Am Acad Dermatol 2005 Jan;52(1):152–6.   [CrossRef]   [Pubmed]    
3. Yasar S, Mansur AT, Göktay F, Sungurlu F, Vardar Aker F, Ozkara S. Lipedematous scalp and lipedematous alopecia: Report of three cases in white adults. J Dermatol 2007 Feb;34(2):124–30.   [CrossRef]   [Pubmed]    
4. Müller CS, Niclou M, Vogt T, Pföhler C. Lipedematous diseases of the scalp are not separate entities but part of a spectrum of lipomatous lesions. [Article in English, German]. J Dtsch Dermatol Ges 2012 Jul;10(7):501–7.   [CrossRef]   [Pubmed]    
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6. Yasar S, Gunes P, Serdar ZA, Tosun I. Clinical and pathological features of 31 cases of lipedematous scalp and lipedematous alopecia. Eur J Dermatol 2011 Jul-Aug;21(4):520–8.   [CrossRef]   [Pubmed]    
7. Fuentelsaz-del Barrio V, Parra-Blanco V, Borregón-Nofuentes P, Suárez-Fernández R. Lipedematous alopecia in a patient with scalp psoriasis. [Article in Spanish]. Actas Dermosifiliogr 2012 Jul-Aug;103(6):551–4.   [CrossRef]   [Pubmed]    
8. High WA, Hoang MP. Lipedematous alopecia: An unusual sequela of discoid lupus, or other co-conspirators at work? J Am Acad Dermatol 2005 Aug;53(2 Suppl 1):S157–61.   [CrossRef]   [Pubmed]    
9. Rowan DM, Simpson A, Wong KP. Lipedematous scalp in a child. Pediatr Dermatol 2006 May-Jun;23(3):276–8.   [CrossRef]   [Pubmed]    
10. Cabrera R, Larrondo J, Whittle C, Castro A, Gosch M. Successful treatment of lipedematous alopecia using mycophenolate mofetil. Acta Derm Venereol 2015 Nov;95(8):1011–2.   [CrossRef]   [Pubmed]