Localized scleroderma (also called morphea) is a term encompassing a spectrum of sclerotic autoimmune diseases that primarily affect the skin, but might also involve underlying structures such as the fat, fascia, muscle, and bones. Morphea profundus presenting with atrophic lesions has rarely been reported in the literature. Here we report two cases of morphea profundus presenting with noninflammatory depressed plaques, without any significant skin induration, pigmentation or textural change. Histopathology was confirmatory for morphea profundus.
A 32-year-old male with a history of albinism and farmer by occupation presented with an ulcerated plaque on the right wrist. The patient had light eyes, hair, and skin. Physical examination showed extensive photodamage. A skin biopsy specimen from the plaque revealed a welldifferentiated squamous-cell carcinoma. Wide surgical excision was done. The most common types of oculocutaneous albinism (OCA), OCA 1 and OCA 2, are autosomal recessive disorders of pigmentation that commonly affect the skin, hair and eyes. Photodamage and skin cancers plague patients with albinism. Albinos face a myriad of social and medical issues. Importance of photoprotection, skin cancer surveillance and treatment has been stressed upon in this report.
BACKGROUND Vitiligo is an acquired depigmentary disorder, where approximately 50% of the cases have the onset of their disease prior to the age of 20 years and 25% prior to the age of 14 years. There is limited data on the clinical characteristics including associated cutaneous and ocular abnormalities in childhood vitiligo. AIMS To evaluate the various clinical characteristics and associated cutaneous and ocular abnormalities of childhood vitiligo. METHODS In a prospective, hospital based study over a period of two years; the epidemiology of childhood vitiligo was studied including associated cutaneous and ocular abnormalities. RESULTS Of the total 122 children studied, majority of them were females (n=75, 61.5%), and the rest males (n=47, 38.5%). The mean age of presentation was 8 years. Progression of lesions was present in 36 children (29.5%). The most common site of initial lesion was head and neck followed by lower limbs, genitalia, trunk and upper limbs. Eight children (6.6%) had a history of trauma prior to onset of vitiligo. Eighteen children (14.8%) had a family history of vitiligo. The most common type was vitiligo vulgaris seen in 45 children (36.9%) followed by segmental type in 33 children (27%). Leukotrichia was seen in 51 children (41.8%), while Koebner phenomenon was observed in 30 children (24.6%). Fifteen children (12.3%) had an associated cutaneous disorder. These associated disorders were halo nevi in 6 children (4.9%), alopecia areata in 3 children (2.5%), canities in 2 children (1.6%), and cafe au lait macule, nevus depigmentosus, lichen nitidus, lichen striatus in 1 each (0.8%). Thirty children (24.6%) had an associated ocular disorder. These associated disorders were eyelid vitiligo in 26 children (21.3%), depigmented spots in the iris in 2 patients (1.6%), lamellar cataract and persistent papillary membrane in 1 each (0.8%). CONCLUSIONS Childhood vitiligo in Bangalore showed preponderance in females and greater number of children (72.4%) present with depigmentation in the age group of 7 to 12 years. Majority of patients (77.9%) had less than 5% body surface area involvement. Low incidence of ocular pigmentary abnormalities in comparison with adult population might suggest that childhood vitiligo patients do not have ocular pigmentary abnormalities in the beginning, but as they age or as the disease progresses they may develop ocular pigmentary changes.
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