ABSTRACT
Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, is characterized by pruritus, lichenified or nodular prurigo-like lesions, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and albopapuloid lesions, appearing at birth or later. Scarring and prurigo are most prominent on the shins. Treatment is unsatisfactory. We report three such cases: two of them first cousins, are described with history of blisters since childhood, followed by intensely pruritic lesions predominantly on the shins, and dystrophy of toenails, but no albopapuloid lesions or milia. Intact blisters were present in one case, and excoriations were seen in the other two. All of them showed encouraging response to cryotherapy.
Subject(s)
Adolescent , Adult , Cryotherapy , Epidermolysis Bullosa/diagnosis , Female , Humans , MaleABSTRACT
Pigmented xerodermoid, a rare genodermatosis, presents with clinical features and pathology similar to xeroderma pigmentosum, but at a later age. DNA repair replication is normal, but there is total depression of DNA synthesis after exposure to UV radiation. Two siblings in their teens and a man in his thirties with features of pigmented xerodermoid, e.g. photophobia, freckle-like lesions, keratoses, dryness of skin, and hypo- and hyper-pigmentation, are described. Although classically the onset of pigmented xerodermoid is said to be delayed till third to fourth decade of life, it seems the disease may appear earlier in the tropics. Early diagnosis and management could be life-saving.
Subject(s)
Administration, Topical , Adolescent , Adult , Biopsy, Needle , Female , Follow-Up Studies , Glycolates/therapeutic use , Humans , Immunohistochemistry , India , Male , Severity of Illness Index , Skin Diseases/diagnosis , Sunscreening Agents/therapeutic use , Ultraviolet Rays/adverse effects , Xeroderma Pigmentosum/diagnosisABSTRACT
Mycosis fungoides (MF), the commonest variant of primary cutaneous T cell lymphoma (CTCL), is relatively uncommon among the Asians. Hypopigmented mycosis fungoides is a rare variant usually observed in dark-skinned individuals, especially children. Hypopigmented MF usually responds well to therapy, particularly to PUVA, and has a comparatively benign course. Mycosis fungoides in a 16-year-old boy, with extensive asymptomatic hypopigmented lesions developing gradually all over the body over eight years and vitiligo-like skin lesions developing for seven years, with no systemic features, is presented for its unusual clinical features and conspicuous histopathological findings of prominent epidermotropism. The case showed fairly good response to PUVASOL therapy.