Spindle Cell Hemangioendothelioma: Rare Clinical Entity.

Spindle-cell hemangioendothelioma (SCHE) comprise a rare subset of vascular tumors, and here, we describe such a case and review the clinical presentation, patho-physiology, differential diagnosis of these tumors to promote early identification and discussion guidance. A 25-years-old male patient presented with multiple painful elevated swellings of both left upper and lower extremities for last 15 years without any systemic involvement. After excluding close differential diagnosis by relevant investigations an excisional biopsy was performed. Based on clinical, radiological and histopathological findings, diagnosis of SCHE was made and full thickness excision and skin grafting were performed. The case is reported due to its rarity and adds our knowledge to the existing literature.

Mucosal patch on anus: a rare sequel of sodomy.

A case of mucosal patch on the perianal area of a 15-year-old boy with history of frequent sodomy is presented here.

Familial childhood systemic lupus erythematosus: report of 2 cases.

Systemic lupus erythematosus is a multisystem organ inflammation due to damage of cells and tissues by pathogenic auto-antibodies and immune complexes. The most important factor for the causation of familial systemic lupus erythematosus is genetic on which environmental factor usually coexists. Two brothers of 7 and 3 years of age having childhood systemic lupus erythematosus are reported here because of low incidence and familial occurrence. Both the children fulfilled the American Rheumatology Association (ARA) criterion of systemic lupus erythematosus though anti-dsDNA was negative in both and antinuclear factor was positive only in the younger child. Systemic lupus erythematosus begins in childhood in 20% of adult patients and usually after the age of 8 years but here onset was even at an earlier age. The treatment of the both the siblings included administration of steroids and cyclophosphamide.

Epidermolytic hyperkeratosis with a rare digital contracture.

A sixteen year-old male patient with no history of consanguinity in the family, reported with patchy, thickened lichenified plaques over the whole body. Some areas had normal skin while some were Blaschkoid lesions. The child had delayed milestones along with hypogonadism. Digital contracture with palmoplantar keratoderma was present. Histopathology showed characteristic vacuolar degeneration of the upper epidermis and suprabasilar keratinocytes with hyperkeratosis.

Epidermolysis bullosa pruriginosa: a rare autosomal dominant variant.

A 35 years female presented with extremely pruritic, violaceous, small vesiculopapular lesions over both shins since 11/12 years of age. The intensity of pruritus slightly descreased following oozing of fluid. History of similar incidence in her mother and maternal grandfather was present. There was no toe-nail dystrophy. Histopathology report showed the lesions had hyperkeratotic, mild acanthosis, dermal lymphohistiocytic infiltrate and subepidermal cleft. The case was diagnosed to be a case of epidermolysis bullosa pruriginosa.

A rare case of fibrocystic disease at vulval accessory breast tissue.

A 40-year-old female presented with a non-itchy ulcerative nodular lesion at left labium majus since last 1 1/2 years. The lesion progressed to increase in size from 0.5 cm to 1.5 cm in diameter. It was incised and drained. After that a non-healing ulcerative nodule formed. The nodule was firm in consistency and movable on all sides. The ulcer healed with a 5 days course of ceftriaxone. If was excised and biopsy of the lesion showed fibrocystic changes of accessory breast tissue. It is a rare disease entity for which the case report is presented.