I Bukhari, F Al Mulhim, R Al Hoqail, Hyperlipidemia and Lipedematous Scalp. 2004; 24(6): 484-485 Lipedematous scalp is the localized accumulation of fatty tissue in the subcutaneous layer of the scalp, without hair abnormalities. Lipedematous alopecia differs in that the localized accumulation of fatty tissue in the subcutaneous layer is associated with alopecia. Only two cases of lipedematous scalp without alopecia and five cases of lipedematous scalp with alopecia have been reported in the literature. We report the third case of lipedematous scalp without alopecia in an Arabic woman, in whom the condition progressed insidiously for 6 months before presentation. Case A 57-year-old southern Saudi woman presented to the dermatology clinic with a gradual diffuse swelling and a heavy feeling over her scalp for the previous 6 months. There was no history of hair loss or pain or trauma in the head. Her medical history included supraventricular tachycardia and hypercholesterolemia. Treatment included aspirin for 8 years, atenolol for 8 years, simvastatin for 3 years, and celecoxib (Celebrex) for 3 months. The family history was negative for conditions similar to lipedematous scalp. On examination the scalp was smooth, remarkably thick, spongy, fluctuant on palpation and mildly tender. No clinical signs of inflammation or irregularities were apparent. Hair density and length were normal. She had skin type 4. Microscopic evaluation of pulled hair found no abnormalities. Serum cholesterol was 265 mg/dL (normal, <200 mg/ dL), and HDL cholesterol was 65 mg/dL (normal, 35-60 mg/dL). Thyroid function tests and growth hormone levels were normal. Tests for connective tissue disorders such as ANA (antinuclear antibody) and anti-DNA were negative. Computed tomography of the skull showed a scalp thickness of 21.2-16.9 mm at the high occipitoparietal region with subcutaneous fat being 19.2-mm thick (Figure 1, 2). An incisional biopsy of the occipitoparietal region revealed a remarkable increase in the thickness of the subcutaneous fat layer with normal epidermal and dermal layers (Figure 3). Extension of the hair follicles into the subcutaneous fat was normal and no fibrosis was detected. In addition, there was a mild superficial peivascular lymphocytic infiltrate with no accumulation of mucin in the dermis or subcutaneous tissue, as confirmed by alcian blue stain. Cultures for aerobic, anaerobic and fungal organisms from the biopsy site were negative. Therefore, the patient was diagnosed as having lipedematous scalp. She has been followed in the clinic with no change in her condition, and is currently being treated for hyperlipidemia and the cardiac condition.
We report an unusual phenotype of congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome most likely resulting from a novel X-chromosomal microdeletion encompassing the promoter region and exon 1 of the nicotinamide adenine dinucleotide phosphate steroid dehydrogenase-like protein gene, the neighboring gene CETN2, and more than 10 kb of noncoding deoxyribonucleic acid.
Mycosis fungoides is the most common primary cutaneous T-cell lymphoma. The approach to diagnosis and further follow-up is outlined. Evidence for interventions is based classically on a Tumor Node Metastasis Blood TNMB “stage-based” approach. The treatment options in India are limited. The options as per risk stratification and prognostic index are discussed. Early stages and low-risk patients can be managed with expectant policy or skin-directed therapies including topical steroids and phototherapy; intermediate-risk patients can be opted for interferons or retinoids or low dose methotrexate along with radiotherapy including total skin electron beam therapy while high-risk patients are managed most often with single agent or multiagent palliative chemotherapy. Patients who are intermediate- or high-risk need management by a multispecialty team at tertiary care centers.
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