sporadic case of ichthyosis hystrix: a rare entity

The term ichthyosis hystrix encompasses several rare ichthyosis form disorders characterized by massive hyperkeratosis with an appearance like spiny scales. This is a rare form of ichthyosis with autosomal dominant inheritance and a very few cases have been reported. We report a case of ichthyosis hystrix in a 22-year-old male patient with hyperkeratotic, dry, warty, scaly excrescences on most of the body surface in a linear pattern. Palms, soles and nails were spared and family history was negative. The case is being reported in view of the rarity of disease and its sporadic occurrence

familial case of pachyonychia congenital

Pachyonychia congenita [PC] comprises a group of rare autosomal genodermatosis caused by mutation in any of the four genes KRT6A, KRT6B, KRT16 or KRT17. Classically, it is subdivided into two major variant types, PC-1 [Jadassohn-Lewandowski syndrome] and PC-2 [Jackson-Lawler syndrome]. We hereby report a case of 22-year-old, married woman with progressive thickening and discoloration of all 20 nails, multiple, hyperkeratotic lesions present all over the body with oral lesions since childhood. She had a 2-month-old male baby [the only child] who presented with similar lesions of yellowish discoloration and nail thickening of both nails and foot since birth. She was diagnosed as PC type 1

HAART – The best treatment modality for widespread and disfigured giant molluscum contagiosum

Molluscum contagiosum [MC] is a common viral infection caused by molluscum contagiosum virus [MCV], a double-stranded DNA virus which belongs to Poxviridae family. It is frequently seen in young children and sexually active adults. The transmission of spread occurs both by sexual and nonsexual routes like direct contact with infected skin, mucous membrane or via fomites. The incubation period varies from two to six months. In immunocompetent persons, the molluscum lesions typically appear as painless, flesh-colored or skin-coloured, dome shaped papules of size 2-3mm with central umbilication that can appear anywhere on the body, but most often located on the face, neck and intertriginous sites like axilla and popliteal fossa. However, atypical forms [giant, verrucous, agminate forms] with involvement of genitalia are more common in immune – compromised persons particularly in sexually active adults. It is usually self-limited in immunocompetent individuals while in HIV-infected patients with advance disease, the lesions are more widespread and extensive. Due to its longer period of persistence and poor response to conventional therapies, the clinicians often face difficulties to treat it. Several reports document that variety of treatment options had been tried for widespread molluscum contagiosum but none had been considered to be the ideal and standard treatment. Herein, we report a case of 6-year-old HIV-infected boy with multiple, tumour like nodules [agminate variety]; a rare presentation of molluscum contagiosum over face and other body parts which successfully resolved with exclusive HAART therapy.

Amyloidosis cutis dyschromica

Amyloidosis cutis dyschromica [ACD] is a rare form of cutaneous amyloidosis, characterized by generalized, asymptomatic hyperpigmentation intermingled with several hypopigmented spots without papulation, atrophy and telangiectasia. Its onset usually begins before puberty. We hereby describe two female siblings of ACD belonging to tribal groups with no systemic involvement. This condition should be considered as a separate entity and need to be differentiated from other variants of primary cutaneous amyloidosis.

Amyloidosis cutis dyschromia: a case report

Amyloidosis cutis dyschromia is a very rare variant of primary cutaneous amyloidosis clinically characterized by hyper- and hypopigmented/depigmented macules in generalized distribution. Diagnosis is based on histopathological demonstration of amyloid deposits in papillary dermis and its confirmation by Congo red staining. We hereby report a case of amyloidosis cutis dyschromia.