Stewart-Treves syndrome: a rare entity.

Stewart-Treves syndrome is a rare, deadly cutaneous angiosarcoma that develops in long - standing chronic lymphedema. Most commonly, this tumor is a result of lymphedema induced by radical mastectomy to treat breast cancer. Stewart-Treves syndrome occurs in 0.5% of patients, who survive mastectomy for more than 5 years. The mean age at appearance of the angiosarcoma is 62 years, and the mean interval between mastectomy and the appearance of the tumor is 10.5 years. The exact pathogenesis is not known so far. On histopathology, vascular channels infiltrate the normal structures in a disorganized fashion, as if trying to line every available tissue space with a layer of endothelial cells. The prognosis is poor as it is very aggressive. Only early diagnosis and prompt radical excision with or without adjuvant radiotherapy, can promote survival.

Spectrum of Papillon–Lefevre syndrome.

Papillon–Lefevre syndrome is an extremely rare autosomal recessive disorder characterized by severe periodontal disease with hyperkeratosis and fissuring of the palms and soles. Periodontitis is severe and destructive affecting both deciduous and permanent dentitions associated with palmo-plantar hyperkeratosis. These manifestations usually appear in childhood between 1 and 4 years of age and deciduous teeth exfoliate within or at the age of six. Permanent teeth erupt normally but soon get affected by periodontal disease. Individual becomes edentulous within teenage. Hyperkeratotic lesions extend to knees and elbows. Some cases have shown inconsistent manifestations such as calcification of falx cerebri and choroid plexus, calcification of the dura, attachment of the tentorium, thumb nail dystrophy and hyperhidrosis.

Hydroa vacciniforme: a very rare photodermatosis.

Hydroa Vacciniforme (HV) is a rare, acquired and chronic paediatric disorder that is characterized by photosensitivity and recurrent crops of skin lesions on sun-exposed skin, such as the face, ears and hands that heal with vacciniforme scarring. The pathogenesis of HV is unknown. No chromosome abnormality has been identified so far. HV patients have no abnormal laboratory results. The histopathologic features are distinctive and demonstrate intraepidermal multilocular vesicles and cellular necrosis. Most cases remit spontaneously by late adolescence.

Reactive perforating collagenosis.

Reactive Perforating Collagenosis (RPC) is a rare disease of elimination of altered collagen through the epidermis. The disease exists in childhood form with autosomal recessive mode of inheritance and an adult form acquired in association with diseases such as Diabetes Mellitus (DM), Chronic Renal Failure (CRF), hypothyroidism, lymphoma, hyperparathyroidism, neurodermatitis, AIDS, pulmonary fibrosis, scabies and herpes zoster infection.