Calcified Keratoacanthoma with Tumoral Calcinosis in a 10-year-old Boy: A mere Co-incidence?

Keratoacanthoma (KA) is a rapidly evolving benign cutaneous tumor, occurring in elderly individuals with a tendency towards spontaneous regression and histopathologic similarity to squamous cell carcinoma. Tumoral calcinosis is an uncommon condition, associated with the deposition of painless calcific masses. The occurrence of these two conditions in the same patient is a rarity itself, whereas deposition of calcium within the KA lesion in our 13-year-old patient makes it even more intriguing. Such an association has been seldom reported in the literature, and this prompted the current report.

Effectiveness, safety and tolerability of cyclosporine versus supportive treatment in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A record-based study.

BACKGROUND: Toxic epidermal necrolysis and Stevens-Johnson syndrome comprise life-threatening, drug-induced mucocutaneous disease spectrum. Interest in cyclosporine, a calcineurin inhibitor that can block the function of T-cells, has increased with the discovery of the importance of granulysin in apoptosis in toxic epidermal necrolysis. In our hospital, cyclosporine is given to Stevens-Johnson syndrome/toxic epidermal necrolysis patients as an adjunctive therapy. AIMS: This study is an observational, record-based study comparing the effectiveness and safety of patients receiving cyclosporine versus only supportive therapy. METHODOLOGY: Medical records as bed-head tickets and laboratory investigation reports of Stevens-Johnson syndrome/toxic epidermal necrolysis patients admitted in the hospital over a period of 1 year were collected. Data regarding clinico-demographic profile, suspected drug causing Stevens-Johnson's syndrome/toxic epidermal necrolysis, SCORTEN, body surface area involved, treatment received and outcome were obtained. RESULTS: Twenty-eight patients were analyzed. Nineteen belonged to the cyclosporine group (supportive treatment + cyclosporine), nine to supportive treatment only group. Among the suspected drugs, antiepileptics formed the major group (28.6%). Five patients in the supportive only group and one in the cyclosporine group died. Time for stabilization and reepithelialization and duration of recovery were significantly lower in the cyclosporine group (P < 0.001, P= 0.007, P= 0.01, respectively). The standardized mortality ratio was 0.32 in cyclosporine group which is nearly 3.3 times lower than the only supportive treatment. LIMITATIONS: As it was a record-based study, certain confounding factors (serum blood urea nitrogen) could not be adjusted. CONCLUSION: Cyclosporine (5 mg/kg/day) for 10 days from onset of Stevens-Johnson syndrome/toxic epidermal necrolysis may decrease the risk of dying, may provide faster healing of lesions and might lead to early discharge from hospital.

Loeffler's Syndrome Following Cutaneous Larva Migrans: An Uncommon Sequel.

Cutaneous larva migrans (CLM) is characterized by the formation of distinctive, tortuous, and serpentine skin lesions occurring as a result of epidermal burrowing by certain helminthic larvae. Although this condition is usually uneventful, rarely it may result in patchy pulmonary infiltration with peripheral eosinophilia, also called Loeffler's syndrome. This association is fairly uncommon and is thus being reported.

Unilateral, Linear Blue Rubber Bleb Nevus Syndrome (Bean's syndrome): An Unfamiliar Presentation: First Case from India.

Blue rubber bleb nevus syndrome (BRBNS) also called Bean's syndrome is a rare disorder characterized by multiple cutaneous venous malformations in association with visceral lesions, most commonly affecting the gastrointestinal tract. We report here, a 21-year-old woman patient, who presented with unilateral, blaschkoid distribution of cutaneous venous malformations along with blue rubber bleb nevus and recurrent episodes of hematochezia due to vascular lesions in the sigmoid colon; likely to be a case of BRBNS. The unusual unilateral, blaschkoid distribution of BRBNS prompted this present report.

Malignant Melanoma Clinically Masquerading Vascular Tumor: A Diagnostic Dilemma.

Malignant melanoma is an invasive neoplasm of the skin, whose incidence is reported to be rising among Indians. We hereby present a unique case of unilateral, multiple, asymptomatic, pigmented, nodular lesions over the lower limb; resembling vascular tumor, revealing itself as malignant melanoma only on histopathology. To the best of our knowledge, such a unique presentation of malignant melanoma has not yet been reported from the Indian subcontinent.

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.

Angiokeratoma circumscriptum neviforme: An entity, few and far between.

Angiokeratomas are a group of vascular ectasias that involve the papillary dermis and may produce papillomatosis, acanthosis and hyperkeratosis of the epidermis. Angiokeratoma circumscriptum is the least common variant among many types. Angiokeratoma circumscriptum neviforme is a still rarer variety of angiokeratoma, which is classically seen at birth. We report here a case of congenital, linear, unilateral, verrucous plaques on the leg of a young girl, diagnosed as angiokeratoma circumscriptum neviforme (ACN).

Linear milia en plaque on the forearm.

A 64-year-old man presented with asymptomatic eruption on his right forearm and the dorsum of the hand present for 2 weeks. There was no history of trauma, prolonged sun exposure, or application of or contact with any substance prior to the development of lesions. He was a known hypertensive and diabetic and was taking treatment for these conditions. The rest of his history was noncontributory. On examination, multiple grouped tiny white papules were found on both normal skin and on the erythematous plaque. These papules were of almost uniform size (2-4 mm) and were notable for absence of umbilication. The erythematous plaque was roughly 15 cm in length and was extending along the ulnar border of forearm and dorsum of hand in a linear pattern (Figure 1). The surface temperature of the plaque appeared similar to the surrounding area, and the surface was studded with multiple tiny white papules. There were no lesions suggestive of chronic actinic damage in the surrounding area. The papules revealed solid whitish material on expression with a needle. The rest of the mucocutaneous examination was noncontributory. Based on clinical presentation, a diagnosis of linear milia en plaque was made.

Single lesion of sarcoidosis presenting as cicatricial alopecia: a rare report from India.

Sarcoidosis affects different systems of the body including the skin where it can affect various cutaneous sites. Among these sites, the scalp is a very unusual location for lesions of sarcoidosis. Sarcoidosis of the scalp can very rarely be accompanied by cicatricial alopecia. We report here a rare case of sarcoidosis of scalp with cicatricial alopecia. To the best of our knowledge, this is the first such report from India.