A case of incontinentia pigmenti, a rare genodermatosis, with nail dystrophy and onycholysis is being reported.

A case of erythrokeratoderma variabilis (EKV) in a male patient, who presented with reddish-brown, well-defined, hyperkeratotic, bilaterally symmetrical plaques on the extensor aspects of upper and lower extremities accompanied by migratory, erythematous patches forming bizarre geometric pattern over the rest of the body which varied in number, size and shape, is being reported. Patient also had palmoplantar keratoderma. Family history was negative. Hisotpathology confirmed the diagnosis of EK.

A 35 year old man presented with multiple indurated plaques, studded with pus discharging sinuses and scarring on both legs for last 15 years. He was also having lichen amyloidosis on both forearms, Pus culture yielded growth of Staphylococcus aureus and tissue culture excluded deep fungal infection and mycobacterium tuberculosis bacilli.

Association of scabies with a bullus pemphigoid - like eruption is being reported because of its rarity. Histopathology revealed subepidermal bullae with intact basal layer. Treatment with topical gammabenzene hexachloride and systemic antibiotics was effective.

A 52 and 15-year-old father and son respectively presented with multiple neurofibromas. On detailed examination diffuse plexiform neurofibroma was noticed in son. Its presence over the lower extremity (a rare site), genu valgum deformity and splenic hamartoma were the interesting features in our case.

A rare case of bilateral facial hemi-atropy is being presented in a 21-year-old woman

A middle aged man presented with features of acquired ichthyosis with Hoffman’s syndrome. Laboratory tests support hypothyodism. Myoedema and hypertrophy of muscles were present. Patient was previously treated for Pellagra.

A 30-year-old man presented with multiple nodules on the frontal region of scalp. Nodules varied from 2 to 4 cms in size, firm in consistency, not mobile, not tender. Oral mucous membrane and genitalia were normal. Systemic examination did not reveal any abnormality. He was provisionally diagnosed as a case of pilomatrixoma. However biopsy revealed the diagnosis of diffuse neurofibroma.

Ecthyma gangrenosum is one of the most serious and specific cutaneous infection caused by pseudomonas seruginosa. We report a case of ecthyma gangrenosum in a new born child who responded poorly to the antipseudomonas treatment to hightlight the poor prognosis in new born.

Lumbar hernia as a complication of herpes zoster is exceedingly rare. Herein we report a case who developed lumbar hernia following herpes zoster T 10, 11 dermatome.

A 54-year-old man, presented with leonine facies and multiple tumours more over the exposed parts of the body. On investigation, it turned out to be a case of primary cultaneous B-cell lymphoma with a distrinctive histopathology. Chemotherapy was given with a good therapeutic response.

A 3-year-old girl presented with sensoryneural deafness and depigmented macular lesions in characteristic pattern since birth. She also had heterochromia of iris and lateral displacement of inner canthi which pointed towards this rare entity.

Erythropoietic protoporphyria (EPP) is an inherited inborn error of porphyrin metabolism which manifests as variable degree of photosenitivity. We report a case of EPP presenting with severe photosensitivity and extensive scarring of 23 years duration without systemic involvement.

Two patients young brother a sister presented with multiple depressed scars over face and trunk since early childhood. Both had hoarsencess of voice since birth. Multiple infiltrated lesions consisting of papules, warty nodules were present on forearms and elbows. The oral mucosa, tongue, lips, larynx and vocal cords also showed infiltration. The characteristic beaded papules on eyelid margin clicked the very rare diagnosis of lipoid proteinosis.

A 25 year old male presented with unusual, multiple, broad-based lipofibromas on the right leg. These were soft, sessile, each measured form 1 to 2 cms and composed of loosely arranged collagen with a central core of mature adipose tissue. Even though skin tags of less than 5 mm size are usual, multiple bag-like protuberances of more than 1cms size are extremely rare. the large size and multiple numbers of lipofibromas prompted us to publish this case.

A young male patient, having linearly arranged nodular lesions on lower extremity was diagnosed to have lymphocutaneous variety of cutaneous nocardiosis. This is a rare entity and has to be differentiated form other causes of nodular lymphangitis. The patient responded dramatically to Cotrimoxazole therapy.

Semi-malignant CBCL, manifesting initially as chronic lymphatic leukemia in a 45 year old female is described.

A case of papuloerythroderma of Ofuji, who presented with erythroderma comprising of multiple coalescing papules, deck chair sign and eosinophilia, is reported. Parior case reports of this syndrome have variably mentioned associated features like lymphadenopathy, nailfold and buttock infarcts and palmoplantar keratoderma, visceral malignancies, laboratory features like circulating Sezary cells, and biopsy findings like exocytosis, vessel wall thickeningor extravasation of red blood cells, which were not evident in this patient. This inconsistency in findings points towards heterogeneous nature of the disorder.

5-flourouracil (5-FU) has been used for the treatment of various malignant and pre-malignant conditions. It has also been used to treat viral warts as a topical agent. It usually does not produce any significant adverse effects when used topically. Severe necrotizing cutaneous reaction following topical 5-FU used for the treatment of warts is being reported.

An 85-year-old male presented with erythematous and tender nodular lesions, since last 15 years, some arranged in a linear fashion, over the anterolateral aspect of 40 year old below knee amputation stump. There were no discharging sinuses. X-ray of the amputation stump showed chronic osteomyelitis of the left tibia. Skin biopsy form one of the nodules showed a solitary intradermal granule with gram-positive filamentous bacteria. Both aerobic as well as anaerobic culture from the nodule did not reveal any growth. Diagnosed as a case of actionomycosis of the amputation stump, with a possible underlying chronic actinomycotic osteomyelitis of the left tibia. Patient was treated with intramuscular penicillin, followed by oral doxycycline and dapsone. The nodular lesions disappeared after five months of follow-up. Chronic osteomyelitis of tibia of below knee amputation stump due to Actinomycetes with granlomatous involvement of the overlying skin, presenting 25 years after sustaining injury and persisting for the next 15 years, without any draining sinuses, is an interesting feature in this case.

Clear cell hidradenoma is a rare and benign cutaneous neoplasm of the eccrine sweat glands. Malignant transformation of these lesions is very rare. In the present communication, a histologically benign clear cell hidrademoma having an aggressive clinical behavior with rapid growth, pigmentation and ulceration is described. Differential diagnosis and management of atypical clear cell hidradenoma is discussed.

Generalized Morphea is uncormmon in children. A 9-year-old female child developed generalized prgressive tautness of skin and joint pains following measles.

A 13-yeat-old male patient born of non consanguineous marriage with history of recurrent urticaria and angioedema for the past 2 years presented with wrinkling and laxity of the skin over the face, axilla and abdomen. Histopathology was consistent with cutis laxa. We are reporting a rare case of acquired cutis laxa due to recurrent urticaria.

Dorsal perforation of prepuce has been reported as a sequelae of both infectius and non-infectious diseases. The increased susceptibility of the dorsal prepuce to perforation is probably due to its deficient blood supply. A patient with dorsal perforation of prepuce due to invasive squamous cell carcinoma is described.

A 7-year-old girl presented with recurrent episodes of petechiae, purpura and ecchymoses since six months of age and recurrent episodes of mild to severe epistaxis since two years of age requiring repeated blood transfusions. In April ‘99 while being investigated for a massive epistaxis, she was found to have platelet function defect with abnormal aggregation of platelets to ADP, epinephrine, collagen as well as to ristocetin. Further investigations ruled out the possibility of Glanzmann’s disorder and von-Willebrand’s disease as to its cause. In May 2001 she was referred to the dermatologist for evaluation of subcutaneous tumours, which had developed since the last six months. On clinical evaluation, she was found to be having mild hyperextensibility of the skin, joint hypermobility, atrophic scars over knee, spontaneous bruises over right forearm and left thigh and nontender firm to hard subcutaneous nodules over both wrists, both shoulders, right index finger and dorsum of right foot consistent with a clinical picture of a mild form of Ehlers-Danlos syndrome (EDS). Histopathology of the nodule from left wrist was consistent with molluscoid tumour of EDS and skin histopathology and ultrastructure studies showed thick irregular collagen fibrils. Only other sibling, a five-year-old male also had history of repeated mild to moderate epistaxis and on examination was found to have a milder variant of EDS. Born out of I degree consanguineous marriage of normal parents with mildly affected other sibling, she was diagnosed to be suffering from EDS with autosomal recessive inheritance, most probably EDS type X due to the associated platelet aggregation defect. Only one such family with EDS type X has been reported so far.

A 3-year-old child diagnosed as varicella bullosa is being presented

Granulomatous rosacea, a subtype of rosacea showing non-caseating epithelioid cell granulomas is difficult to differentiate form lupus miliaris disseminatus faciei. Although appearently similar, the clinical and pathologic features, and the natural course of both are different. The similarities and differentiation of rosacea from lupus miliaris disseminatus faciei is discussed.

Eventhough psoriasis is common id Down’s syndrome only few cases are reported in literature. Both Down’s syndrome and psoriasis are associated with elterations in cyclic nucleotides. This case is reported to heighten the awareness of common biochemical defects in psoriasis and Down’s syndrome.

A case of multiple apocrine hidrocystoma at unusual sites in a middle-aged lady is reported.

Rothmund-Thomson Syndrome is a rare but important cause of childhood photosensitivity. Here, three children with early onset photosensitivity and poikiloderma have been described with discussion of the differential diagnosis.

A 50-year-old male presented with multiple, small, hyperkeratotic papules on palms and soles since 30 years. Elimination criteria for other clinical conditions and a histopathological evaluation helped to consider a diagnosis of Punctate Palmoplantar keratoderma. This cas is presented for its clinical rarity.

A male aged 57 years with multiple discharging sinuses on both sides of chest, multiple ulcers on the back, painful ankylosis of right shoulder since 2 months. Chest examination showed reduced expansion and decreased breath sounds on right side. Large boggy swelling on right hemithorax with multiple discharging sinuses was seen. VDRL was reactive in high dilutions and he was also ELISA - HIV positive. X-ray of chest showed few opacities in right lung field. A provisional diagnosis of Gumma - Syphilis/Tuberculous was considered. Sensorineural deafness was also present.

Pleomorphic adenoma is a benign tumor of the salivary glands that has elements of both epithelial and mesenchymal tissues. The tumor most commonly arises in the parotid or submandibular glands. Infrequently, it may arise from the minor salivary glands and present as an intraoral mass over the palate or lip. We report a patient with pleomorphic adenoma over the hard palate, which resembled common intraoral diseases like condyloma acuminata, oral papilloma and squamous cell carcinoma.

A case of Ochronosis is discribed in a 40-year-old man. He had pigmentation of palms, nose, cheeks and forehead, alkapton uria, arthritis and Osler’s sign in the eyes, as manifestations of ochronosis.

Dermatitis artefacta is a psychocutaneous disorder most likely to be seen by a dermatologist rather than a psychiatrist. We report an unusual and interesting case of dermatitis artefacta in a 17-year-old female.

A 21-year-old female presented with erythematous, painful, crusted plaques on her right arm and left thigh of 4 month’s duration. There was no history of preceding trauma or constitutional symptoms. Response to oral antibioticas was poor. Clinical differential diagnoses of pyoderma gangrenosum (PG) and granulomatous skin diseases such as deep fungal infection, lupus vulgaris and sarcoidosis were considered. Histopathology revealed features of granulomatous variant of PG. The patient was initially treated with dapsone and topical clobetasone propionate but she developed dapsone induced hepatitis and anemia warranting stoppage of the drug. She recovered completely from these side effects and was subsequently treated successfully with intralesional triamcinolone injections.

A case report of plaque and nodular type of lichen myxoedematosus of sudden onset and rapid progression without any systemic manifestation in a pregnant female.

Pseudocyst of the auricle is a rare, asymptomatic intracartilaginous swelling of the auricle resulting form accumulation of yellow viscous fluid with unknown cause. Various methods of treatment such as aspiration, corticosteroid injections and surgical intervention have been advocated for this condition. We report two cases treated successfully with a combination of aspiration, pressure dressing and oral corticosteroid therapy.

A case of Reither’s syndrome in a 32 years old HIV patient not responding to non steroidal anti-inflammatory drugs, PUVASON and topical therapy treated with combination of Zidovudine, Lamivudine and Nevirapine with dramatic response in six weeks is reported.

A 37-year-old man presented with fever, loss of weitht and multiple raised skin lesions on face, neck and hands of 3 months duration. Skin biopsy and bone marrow aspirate revealed PAS posittive intracellular organism. He was treated with Amphotericin and Ketoconazole with excellent response.