Anubhav Garg

Randomized comparison of topical betamethasone valerate foam, intralesional triamcinolone acetonide and tacrolimus ointment in management of localized alopecia areata.

Background: Alopecia areata (AA) is a common, non-scarring, patchy loss of hair at scalp and elsewhere. Its pathogenesis is uncertain; however, auto-immunity has been exemplified in various studies. Familial incidence of AA is 10-42%, but in monozygotic twins is 50%. Local steroids (topical / intra-lesional) are very effective in treatment of localized AA. Aim: To compare hair regrowth and side effects of topical betamethasone valerate foam, intralesional triamcinolone acetonide and tacrolimus ointment in management of localized AA. Materials and Methods: 105 patients of localized AA were initially registered but 27 were drop out. So, 78 patients allocated at random in group A (28), B (25) and C (25) were prescribed topical betamethasone valerate foam (0.1%) twice daily, intralesional triamcinolone acetonide (10mg/ml) every 3 weeks and tacrolimus ointment (0.1%) twice daily, respectively, for 12 weeks. They were followed for next12 weeks. Hair re-growth was calculated using “HRG Scale”; scale I- (0-25%), S II-(26-50%), S III - (51-75%) and S IV- (75-100%). Results: Hair re-growth started by 3 weeks in group B (Scale I: P<0.03), turned satisfactory at 6 weeks in group A and B (Scale I: P<0.005, Scale IV: P<0.001)), good at 9 weeks (Scale I: P<0.0005, Scale IV: P<0.00015), and better by 12 weeks of treatment (Scale I: P<0.000021, Scale IV: P<0.000009) in both A and B groups. At the end of 12 weeks follow-up hair re-growth (>75%, HRG IV) was the best in group B (15 of 25, 60%), followed by A (15 of 28, 53.6%) and lastly group-C (Nil of 25, 0%) patients. Few patients reported mild pain and atrophy at injection sites, pruritus and burning with betamethasone valerate foam and tacrolimus. Conclusion: Intralesional triamcinolone acetonide is the best, betamethasone valerate foam is better than tacrolimus in management of localized AA.

Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy

Sir, Scleromyxedema is an uncommon disorder of mucin deposition associated with paraproteinemia, especially IgG gammopathy. Its pathogenesis is still uncertain, but there are various hypotheses, one of which suggests that some circulating factor, not the paraprotein, might be stimulating fibroblast activity. Scleromyxedema shows little tendency for spontaneous remission. Its treatment is frequently disappointing and the evidence of therapeutic efficacy is largely anecdotal. We would like to share our experience of using dexamethasonecyclophosphamide pulse (DCP) therapy in a patient of scleromyxedema. We chose DCP therapy because of its successful use in auto-immune disorders like pemphigus, scleroderma and SLE.

Multisystem langerhans cell histiocytosis in adult

Langerhans cell histiocytosis (LCH), is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Occurrence of LCH in adults is rare. Here, we report the case of an adult with acute onset of polymorphic eruptions all over the body, which on biopsy showed features of multisystem LCH, and was confirmed by immunohistochemistry. Although multisystem LCH has a poor prognosis, our patient responded well to chemotherapy.

Kaposi's sarcoma as a presenting manifestation of HIV

Kaposis sarcoma is a multi-focal vascular tumor involving skin and the other organs. HIV associated Kaposis sarcoma is one of the AIDS defining condition. It is rarely reported from India. We report a 40-year-old heterosexual married male with widespread cutaneous lesions of Kaposis sarcoma without any oral lesions or systemic association as a presenting manifestation of HIV.

Guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis: An Indian perspective.

Background: Stevens–Johnson syndrome and toxic epidermal necrolysis are severe, life-threatening mucocutaneous adverse drug reactions with a high morbidity and mortality that require immediate medical care. The various immunomodulatory treatments include systemic corticosteroids, cyclosporine, intravenous immunoglobulin, cyclophosphamide, plasmapheresis and tumor necrosis factor-α inhibitors. Aim: The ideal therapy of Stevens–Johnson syndrome/toxic epidermal necrolysis still remains a matter of debate as there are only a limited number of studies of good quality comparing the usefulness of different specific treatments. The aim of this article is to comprehensively review the published medical literature and frame management guidelines suitable in the Indian perspective. Methods: The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL) assigned the task of preparing these guidelines to its special interest group on cutaneous adverse drug reactions. The group performed a comprehensive English language literature search for management options in Stevens–Johnson syndrome/toxic epidermal necrolysis across multiple databases (PubMed, EMBASE, MEDLINE and Cochrane) for keywords (alone and in combination) and MeSH items such as “guidelines,” “Stevens–Johnson syndrome,” “toxic epidermal necrolysis,” “corticosteroids,” “intravenous immunoglobulin,” “cyclosporine” and “management.” The available evidence was evaluated using the strength of recommendation taxonomy and graded using a three-point scale. A draft of clinical recommendations was developed on the best available evidence which was also scrutinized and critically evaluated by the IADVL Academy of Dermatology. Based on the inputs received, this final consensus statement was prepared. Results: A total of 104 articles (meta-analyses, prospective and retrospective studies, reviews [including chapters in books], previous guidelines [including Indian guidelines of 2006] and case series) were critically evaluated and the evidence thus gathered was used in the preparation of these guidelines. Recommendations: This expert group recommends prompt withdrawal of the culprit drug, meticulous supportive care, and judicious and early (preferably within 72 h) initiation of moderate to high doses of oral or parenteral corticosteroids (prednisolone 1-2 mg/kg/day or equivalent), tapered rapidly within 7-10 days. Cyclosporine (3-5 mg/kg/day) for 10-14 days may also be used either alone, or in combination with corticosteroids. Owing to the systemic nature of the disease, a multidisciplinary approach in the management of these patients is helpful.

Pulmonary and cutaneous sarcoidosis in a treated case of renal tuberculosis

Sir, Tuberculosis and sarcoidosis are chronic diseases which can occur in the same patient rarely. It has been suggested that infective agents including Mycobacteria, Propionibacteria, fungi such as Candida, and parasites such as Schistosoma, are likely triggers in a genetically predisposed individual and that this initial event leads to the sarcoidal granulomatous response.[1] The histological similarity between sarcoidosis and tuberculosis featuring epitheloid cell granuloma as a typical common finding, has stimulated the search for an association between sarcoidosis and mycobacteria. We, herein, report a case who developed sarcoidosis after successful treatment of renal tuberculosis.

Terminal 4q deletion syndrome

Terminal deletion of the long arm of chromosome 4, (4q) is a rare event. It is characterized by spectral phenotypic manifestations, depending upon the site and quantity of chromatin lost. The chromosomal loss which span 4 (q31-q35) segment often manifests as craniofacial anomalies, mental retardation with ocular, cardiac, genitourinary defects and pelvic/limb dysmorphism. These abnormalities are usually unilateral. We report a female child (46, XX), aged 11 months, born to nonconsanguineous parents, bearing chromosomal deletion of 4 (q31.2-35.2) segment, which has manifested as craniofacial hypoplasia of left side of face, ipsilateral ptosis, erythroderma and bilateral thumb anomalies.

A case of zosteriform Darier's disease with seasonal recurrence

Dariers disease is an uncommon genodermatosis characterized by keratotic papules in seborrheic distribution. The disease can rarely present in unilateral zosteriform pattern, as a mosaic form following the Blaschkos line. We present a 35-year-old woman with zosteriform pattern of Dariers disease over right infra mammary region. The lesions occurred strictly during summers. Histologically, suprabasal acantholysis with abundant dyskeratotic cells were seen.

Congenital hypertrichosis lanuginosa

A 3-month-old male infant of non-consanguineous parents, born full term by normal vaginal delivery, was seen with excessive hair growth over entire body since birth. None of the family members had abnormal hair growth. Mother denied intake of alcohol or any other medication during pregnancy. There was no history of seizures in infant. The developmental milestones were normal for his age. He weighed 5 kg. The head circumference and crown-heel length were 41 and 57 cm, respectively. Dermatological examination revealed dense growth of gray to light brown, silky hair over most of the body [Figure 1]. Hair was relatively profuse and long over face [Figure 2], axillae, extremities and genitalia [Figure 3]. The hair over scalp was dark and coarse [Figure 2]. The palms, soles [Figure 3] and lips were spared. Nails and mucosae were normal. Systemic examination did not reveal any abnormality. The diagnosis of congenital hypertrichosis lanuginosa (CHL) was made on the basis of above findings.

Phacomatosis cesioflammea with Klippel Trenaunay syndrome: A rare association

A 30-year-old Indian male presented with bilateral Nevus of Ota, extensive nevus flammeus over the trunk and left lower limb with soft tissue hypertrophy and varicosities affecting the left lower limb. He was otherwise in good general health. A diagnosis of Phacomatosis cesioflammea or Phacomatosis pigmentovasularis Type II with Klippel Trenaunay syndrome was made. The case is being reported on account of its rarity.