Search

Griether’s disease, also known as, Transgrediens et Progrediens Palmoplantar Keratoderma, is an autosomal dominant disorder, clinically characterized by diffuse palmoplantar keratoderma associated with hyperhidrosis and progressive extension of keratoderma to the dorsum of hands and feet, knees, elbows with characteristic involvement of Tendo-Achilles. We report a case of a 9 year old male with typical features of Greither’s disease.

Non-epidermolytic variety of hereditary palmoplantar keratoderma with autosomal dominant inheritance characterised by uniform, thick, yellow hyperkeratosis over palms and soles was first described separately by Thost and Unna and named as Unna-Thost Keratoderma. We present a case of Unna-Thost keratoderma in a 9 year old male child who presented with waxy, thick, yellow palms and soles with fissures and contractures in hands.

Disseminated Superficial Actinic Porokeratosis (DSAP) is a type of porokeratosis observed in parts of the skin exposed to intense sunlight and usually appears in the third or fourth decade of life. We are presenting a case report of a 30 year old male patient presenting with multiple lesions over the face and limbs.

Leprosy reactions are rare expression of immunological perturbations that interrupt the usual chronic course and the clinical stability of patients with leprosy. They generally occur during anti-mycobacterial treatment and are characterized by the appearance of crops of brightly erythematous tender nodules or plaques. Erythema Necroticans (ENe) is an uncommon manifestation of Type 2 Erythema Nodosum Leprosum (ENL) reaction, encountered in lepromatous and borderline lepromatous cases of leprosy. Severe ENL can become vesicular or bullous and break-down and is termed Erythema Necroticans.

Acral peeling skin syndrome is a rare, congenital subtype of peeling skin syndrome affecting the dorsal and volar aspects of hands and feet. The cutaneous manifestations consist of lifelong, episodic or continuous, painless, non-inflammatory peeling of dorsal and volar aspects of hands and feet. A biopsy specimen shows hyperkeratosis and splitting of the epidermis between the granular layer and the stratum corneum. This chronic disease has no known effective established treatment.

Peutz-Jeghers Syndrome (PJS) is a rare, autosomal dominant disorder responsible for mucocutaneous pigmentation and gastrointestinal hamartomatous polyps. We present a case of Peutz-Jeghers syndrome in a 12 year old male child who presented with abdominal pain, vomiting and malena. The patient had pigmented lesions on the malar area, nose, lower lip and buccal mucosa. The imaging studies revealed multiple polyps in small intestine.

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.