Premature graying of hair.

Premature graying is an important cause of low self-esteem, often interfering with socio-cultural adjustment. The onset and progression of graying or canities correlate very closely with chronological aging, and occur in varying degrees in all individuals eventually, regardless of gender or race. Premature canities may occur alone as an autosomal dominant condition or in association with various autoimmune or premature aging syndromes. It needs to be differentiated from various genetic hypomelanotic hair disorders. Reduction in melanogenically active melanocytes in the hair bulb of gray anagen hair follicles with resultant pigment loss is central to the pathogenesis of graying. Defective melanosomal transfers to cortical keratinocytes and melanin incontinence due to melanocyte degeneration are also believed to contribute to this. The white color of canities is an optical effect; the reflection of incident light masks the intrinsic pale yellow color of hair keratin. Full range of color from normal to white can be seen both along individual hair and from hair to hair, and admixture of pigmented and white hair is believed to give the appearance of gray. Graying of hair is usually progressive and permanent, but there are occasional reports of spontaneous repigmentation of gray hair. Studies evaluating the association of canities with osteopenia and cardiovascular disease have revealed mixed results. Despite the extensive molecular research being carried out to understand the pathogenesis of canities, there is paucity of effective evidence-based treatment options. Reports of repigmentation of previously white hair following certain inflammatory processes and use of drugs have suggested the possibility of cytokine-induced recruitment of outer sheath melanocytes to the hair bulb and rekindled the hope for finding an effective drug for treatment of premature canities. In the end, camouflage techniques using hair colorants are outlined.

Bullous pilomatricoma: a stage in transition to secondary anetoderma?

Pilomatricoma is an uncommon hamartomatous tumor of the hair matrix. Bullous and anetodermic changes over pilomatricoma are rare. We report an 18-year-old male with an asymptomatic nodule with overlying cystic changes on the left arm of 6-month duration with clinical and histological features of both bullous and anetodermic modifications. We also reviewed the associated literature to conclude that there is sufficient overlap in these two entities. Both variants show a bullous or pseudobullous appearance clinically and loss of elastin, sparse collagen bundles separated by intense edema, and dilated lymphatics/blood vessels in the dermis overlying the tumor mass histologically. We therefore propose that bullous, pseudoampullary, anetodermic, and lymphangiectatic forms should be considered as synonymous or transitional to the ultimate scar-like anetodermic appearance.

Pilomatricoma: a tumor with hidden depths.

BACKGROUND: Pilomatricoma is a benign tumor of hair matrix differentiation and has been classically described as comprising of basaloid and shadow cells admixed with multinucleated giant cells and areas of calcification. However, there are a diverse range of histologic features this tumor displays that are often unrecognized. AIMS: This study was undertaken to record the histopathologic features of pilomatricoma with an emphasis on the occurrence of other forms of differentiation. METHODS: The study included all skin biopsy specimens over a 13-year period from 1995 to 2007 that had a histologic diagnosis of pilomatricoma. Hematoxylin and eosin-stained slides were reviewed. RESULTS: This study included 21 cases of pilomatricoma. Supramatrical differentiation was seen in all cases and three-quarters of the cases showed matrical differentiation. Also observed in some of the cases were clear cell differentiation toward the outer root sheath, infundibular differentiation, calcification, ossification and secondary inflammation with a foreign body giant cell reaction. Epidermal induction in the form of a downward plate-like growth of the epidermis was seen in a few cases. CONCLUSION: Pilomatricoma, although considered a tumor of hair matrix differentiation, can show cellular evolution toward the other parts of the hair follicle, such as the outer and inner root sheaths, sebaceous and infundibular components and, therefore, can be considered a panfollicular neoplasm.

Naxos disease: a rare occurrence of cardiomyopathy with woolly hair and palmoplantar keratoderma.

Naxos disease is a rare genodermatosis with woolly hair, keratoderma of palms and soles and cardiomyopathy. A seven-year-old boy presented with woolly hair and hyperkeratotic lesions on the palms and soles since birth. His cardiac status was evaluated and echocardiography revealed early cardiomyopathy. Scalp biopsy revealed hair shaft in an angulated outline suggestive of woolly hair. So the diagnosis of Naxos disease was made. Since he was asymptomatic no treatment was offered but a regular follow-up of the patient and treatment of emergent symptoms should prevent sudden death.

EEC syndrome sans clefting: variable clinical presentations in a family.

Ectrodactyly, ectodermal dysplasia and cleft palate/lip syndrome (EEC) is a rare autosomal dominant syndrome with varied presentation and is actually a multiple congenital anomaly syndrome leading to intra- and interfamilial differences in severity because of its variable expression and reduced penetrance. The cardinal features include ectrodactyly, sparse, wiry, hypopigmented hair, peg-shaped teeth with defective enamel and cleft palate/lip. A family comprising father, daughter and son presented to us with split hand-split foot deformity (ectrodactyly), epiphora, hair changes and deafness with variable involvement in each family member.

Eyelid changes in long-standing leprosy.

To describe eyelid changes in ocular leprosy, 74 patients (148 eyes or 296 eyelids) were examined, focusing on eyelid abnormalities. The adnexal examination included evaluation of the upper eyelid crease pattern, qualitative assessment of the orbicularis oculi muscle function, measurement of the distance between the corneal reflex and the upper eyelid margin (margin reflex distance), and slit-lamp biomicroscopy of the eyelashes and tarsal conjunctiva. Eyelash ptosis was a common finding associated with a multiple upper eyelid crease pattern and trichiasis. In the past, eyelash ptosis has probably been diagnosed as upper eyelid entropion or trichiasis, but in this series entropion was not observed. The distinction between eyelash ptosis, trichiasis, and upper eyelid entropion is important because the surgical management for each is different. Other true leprotic abnormalities of the eyelids are lagophthalmos and lower lid ectropion.