Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Fresh Look at an Old Foe.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are some of the less common cutaneous adverse drug reactions with significant mortality. Objectives: This study was undertaken with the objective of studying the demographics and clinical profile of SJS/TEN and identifying parameters associated with mortality. Materials and Methods: All patients with SJS/TEN over 10 years (2010-2020) were included in the study. Data obtained from in-patient and out-patient records were analysed. Results: A total of 82 patients with SJS/TEN were admitted to our centre over a period of 10 years. Patients with SJS were significantly younger than those with TEN, with a male: female ratio >1 in SJS and <1 in TEN. The most commonly implicated drugs were antiepileptics (n = 29, 35.4%), antibiotics (n = 20, 24.4%). and Non-steroidal antiinflammatory drugs (NSAIDs) (n = 7, 8.5%). The mortality rate in the TEN group was 16% (n = 8). Certain factors such as cutaneous lesions preceding mucosal lesions at onset, high mean Body surface area (BSA) of denudation and a transfer to intensive care unit (ICU) more than 7 days after admission were significantly associated with higher mortality. There was no difference between survivors and deaths in terms of delay in hospitalisation, total disease duration, implicated drug, delay in initiation of therapy, the onset of re-epithelialisation, Severity-of-illness score for TEN (SCORTEN) and total duration of hospital stay. Conclusion: Factors significantly associated with increased mortality in TEN were cutaneous onset of lesions, mean BSA of involvement and transfer to the intensive care unit (ICU) beyond day 7 of admission.

The master of masquerade: A case series of unusual presentations of cutaneous tuberculosis in the immunocompromised host.

Cutaneous tuberculosis is known for its varied presentations, especially in the setting of immunosuppression. Clinical manifestations can be modified by the site of involvement as well as the type of cutaneous tuberculosis in a particular patient. Although cutaneous tuberculosis only accounts for a small percentage (1-2%) of extrapulmonary cases, it is encountered fairly frequently in dermatology practice in an endemic country like India. These cases are being presented to highlight unusual presentations of well-known forms of cutaneous TB (TB gumma and erythema induratum of Bazin) and rare morphologies (panniculitis) in immunosuppressed individuals, thereby emphasizing the need for the dermatologist in a TB-endemic country to be constantly vigilant.

Intralesional immunotherapy with purified protein derivative (PPD) for cryotherapy-resistant warts.

BACKGROUND: Cryotherapy and immunotherapeutic modalities elicit nonspecific immune response against the human papillomavirus. There is a paucity of literature on the effects of a sequential shift to immunotherapy in cryotherapy-resistant warts. AIM: To study the efficacy of intralesional purified protein derivative (PPD) immunotherapy in cryotherapy-resistant warts. METHODS: Patients with cryotherapy-recalcitrant cutaneous warts were given intralesional injections of PPD into the index warts (oldest or largest) at 2-week intervals until complete clearance or up to a maximum of six injections. The response in the treated index and distant warts was defined as complete, partial, and no response (<25%). Complete responders were followed up for another 3 months to check for recurrence. RESULTS: Twenty-eight patients completed the study protocol. Of the eight patients with single warts, four (50%), one (12.5%), and three (37.5%) patients had complete, partial, and no response, respectively. Of the 20 patients with multiple warts, nine (45%) had complete clearance of all warts, two (10%) each had complete and partial response in the index wart, respectively, with no response of the distant warts, and seven (35%) had no response in all warts. Complete response was seen in an average of 3.1 injections (range 1-5). There was no recurrence at the follow-up visit. CONCLUSION: Immunotherapy with PPD has potential in producing regional and remote wart regression even in cryotherapy-resistant warts. It is a safe and economical modality in children, multiple warts, and difficult-to-treat warts.

Oral Provocation Testing in Cutaneous Adverse Drug Reactions to Antiretroviral and Antitubercular Therapy: A Study at a Tertiary Care Hospital in South India.

AIM: To collect data pertaining to oral drug provocation testing (DPT) in hospitalized patients with antiretroviral (ARV)/antitubercular agent-induced rashes. METHODS: Patients with cutaneous adverse drug reactions (ADRs) to ARV/antitubercular drugs and who underwent oral DPT during a 5-year period were included in this study. RESULTS: Data were collected from the records of 21 patients. Of the 21, 19 had HIV infection. The most commonly implicated drug was nevirapine (NVP), followed by cotrimoxazole and antitubercular agents. Of the 11 ADRs that occurred on rechallenge, the ADR on rechallenge was similar in clinical presentation to the initial ADR in 6 patients, while a different rash was elicited in 5 patients. CONCLUSION: Oral DPT is a safe and effective tool to accurately diagnose ADRs, especially in patients on multiple drugs and in situations such as HIV infection and tuberculosis where second-line agents are expensive and/or not easily available through the national AIDS control/tuberculosis programs.

Lichen planus hypertrophicus of the vulva - a rare entity.

Vulvovaginal lichen planus is a type of chronic inflammatory dermatosis of the vulva, usually seen as a part of widespread lichen planus. The common clinical types of vulval lichen planus include papulosquamous, erosive and pigmented types. Hypertrophic lichen planus of the vulva is a rare entity. We report a case of hypertrophic lichen planus of the vulva which clinically simulated genital warts.

Cutaneous tuberculosis of the pinna: a report of two cases.

BACKGROUND: Cutaneous tuberculosis (TB) is less common than other forms of TB but accounts for 1.5% of all cases of extrapulmonary TB. The source of mycobacterial infection and the immune status of the host determine the presentation of any of a wide spectrum of clinical manifestations. Lupus vulgaris (LV) is a post-primary, paucibacillary form of TB caused by hematogeneous, lymphatic, or contiguous spread from elsewhere in the body. METHODS: We report two recent cases of LV in women presenting with the exclusive involvement of the ear lobe. In Patient 1, clinical presentation appeared as an apparently benign chronic eczematous process. In Patient 2, it appeared as a fulminant ulceronecrotic process. Both women were immunocompetent, and neither had a personal or family history of TB. RESULTS: Both patients were diagnosed with LV and treated with standard antitubercular therapy (ATT). In both patients, mycobacterial culture showed growth of Mycobacterium tuberculosis sensitive to streptomycin, rifampicin, and ethambutol. CONCLUSIONS: Lupus vulgaris is the most common form of cutaneous TB. It is important to diagnose LV because it can result in chronic disfigurement and because 10-20% of LV patients have active pulmonary TB or TB of the bones and joints. In addition, longstanding LV is known to lead to the development of squamous cell carcinoma, which can be avoided by early diagnosis and treatment with ATT.

Phage typing in dermatitis cruris pustulosa et atrophicans: does staphylococcal carrier status have a role?

BACKGROUND: Dermatitis cruris pustulosa et atrophicans (DCPA) is a form of chronic folliculitis of the legs with a multifactorial etiopathogenesis, seen primarily in tropical countries. Staphylococcus aureus has been isolated from the pustules in earlier studies, although the organisms isolated have not been further characterized. MATERIALS AND METHODS: Patients with DCPA, who attended the Dermatology outpatient clinic at JIPMER, Pondicherry, India, during the study period (December 2006-June 2008) were included. Pus from the lesions as well as swabs from carrier sites (nares, axillae, and gluteal fold) were cultured. Staphylococcus aureus isolates were subjected to phage typing at the National Staphylococcal Phage Typing Center, Department of Microbiology, Maulana Azad Medical College, New Delhi, India. RESULTS: Thirty-seven patients were included in the study. Pus from the folliculitic lesions grew S. aureus in 32 (86.49%) patients. Based on the comparison of antibiotic sensitivity patterns, isolates from pus and carrier sites were found to be similar in 15 patients. Phage typing established the organism to be identical in five of these patients. CONCLUSIONS: Characterization of S. aureus in DCPA shows that there is no specific phage type that is uniformly responsible for the lesions in most patients. However, in view of the unclear etiology of this condition, the pathogenicity of a staphylococcal carrier state in individual patients needs to be addressed.

Diet in dermatology: revisited.

Diet has an important role to play in many skin disorders, and dermatologists are frequently faced with the difficulty of separating myth from fact when it comes to dietary advice for their patients. Patients in India are often anxious about what foods to consume, and what to avoid, in the hope that, no matter how impractical or difficult this may be, following this dictum will cure their disease. There are certain disorders where one or more components in food are central to the pathogenesis, e.g. dermatitis herpetiformis, wherein dietary restrictions constitute the cornerstone of treatment. A brief list, although not comprehensive, of other disorders where diet may have a role to play includes atopic dermatitis, acne vulgaris, psoriasis vulgaris, pemphigus, urticaria, pruritus, allergic contact dermatitis, fish odor syndrome, toxic oil syndrome, fixed drug eruption, genetic and metabolic disorders (phenylketonuria, tyrosinemia, homocystinuria, galactosemia, Refsum's disease, G6PD deficiency, xanthomas, gout and porphyria), nutritional deficiency disorders (kwashiorkar, marasmus, phrynoderma, pellagra, scurvy, acrodermatitis enteropathica, carotenemia and lycopenemia) and miscellaneous disorders such as vitiligo, aphthous ulcers, cutaneous vasculitis and telogen effluvium. From a practical point of view, it will be useful for the dermatologist to keep some dietary information handy to deal with the occasional patient who does not seem to respond in spite of the best, scientific and evidence-based therapy.

Dermatitis cruris pustulosa et atrophicans revisited: our experience with 37 patients in south India.

BACKGROUND: Dermatitis cruris pustulosa et atrophicans (DCPA) is a distinctive type of chronic superficial folliculitis, with a number of unique features such as its peculiar symmetric localization to legs, extreme chronicity, resistance to therapy, and inevitable alopecia and atrophy. METHODS: All patients with DCPA, attending the Dermatology Outpatient Department at Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) Hospital, Pondicherry, from December 2006 to June 2008 were included. Parameters recorded were detailed history and examination, hemogram, erythrocyte sedimentation rate, random blood sugar, skin biopsy and cultures from pus and carrier sites (nares, axillae and gluteal fold). RESULTS: 37 patients were studied (35 males and 2 females). Sixteen patients (43.24%) belonged to the 21-30 year-old age group. The disease most commonly began on the legs (81.1%). Majority (78.38%) had a disease duration of less than 5 years. Itching was the most common symptom (89.19%), followed by bleeding and scaling, with no significant systemic symptoms. The lower limbs were involved in all patients. Eleven patients (29.73%) had involvement of other sites--beard, axillae, chest, moustache, abdomen, and eyebrows. Pustules, papules, and scaling were seen in all patients, followed by wiry roughness, atrophy, alopecia, and pigmentation. Aggravating factors included use of full-length synthetic trousers, occupational exposure to potential irritants, and season (summer). Pus culture from the folliculitic lesions grew Staphylococcus aureus in 32 (86.49%) patients. Twenty one patients (56.75%) were carriers of S. aureus in one or more sites. CONCLUSION: DCPA is a chronic folliculitis of the legs, with a multifactorial etiopathogenesis, in which staphylococcal carrier status may be a new potential pathogenetic factor.

Dermatitis cruris pustulosa et atrophicans.

Dermatitis cruris pustulosa et atrophicans (DCPA) is a distinctive type of chronic superficial folliculitis, primarily affecting the lower limbs. It is characterized by symmetrical follicular pustules of both legs, with cutaneous edema, resulting in alopecia, atrophy and scarring. It was first described by Clarke, from West Nigeria, in 1952 and well illustrated in his book "Skin diseases in the African," under the initial label of "Nigerian shin disease." Subsequently, it was described in India as well, in 1964, and continues to be a problem in dermatology clinics across the country. It is predominantly a disease of men and has a high prevalence in some geographical regions; up to 3-4% in Madras, South India. Some unique features that distinguish DCPA from banal pustular folliculitis include its peculiar localization to the legs, extreme chronicity, resistance to therapy and inevitable alopecia and atrophy of the involved skin, with little postinflammatory hyper- or hypopigmentation. Further, even in the presence of extensive lesions, there are no systemic features. Coagulase-positive Staphylococcus aureus is known to have a role in the etiology of DCPA, but the exact etiopathogenesis still needs to be elucidated. Immunological postulates such as hypergammaglobulinemia have been put forward to explain the chronicity of the condition. A number of therapeutic agents have been tried in various studies, including cotrimoxazole, psoralen with ultraviolet A (PUVA) therapy, ciprofloxacin, pentoxifylline, rifampicin, dapsone, minocycline and mupirocin (topical) with variable success rates. Although a well-recognized entity in dermatology clinics in tropical countries, DCPA has received little attention in the dermatological literature and has only a few studies to its credit. Its unique clinical picture, unclear etiopathogenesis and resistance to therapy afford a vast scope for further investigation and study.

Relapse in leprosy.

Leprosy is unique in terms of the nature of the causative organism (Mycobacterium leprae), the chronicity of the disease, its prolonged treatment and the definitions of "cure" and "relapse." The principal mode of assessing the efficacy of therapeutic regimens in leprosy is the "relapse rate." There are wide variations in estimates of relapse rates after the World Health Organization (WHO) multidrug therapy in different regions. The important predisposing factors for relapse include the presence of "persister" bacilli, monotherapy, inadequate/irregular therapy, presence of multiple skin lesions/thickened nerves and lepromin negativity. The conventional methods of confirming activity or relapse in an infectious disease (demonstration and/or culture of the etiologic agent) have limited utility in leprosy because of the difficulty in demonstrating bacilli in paucibacillary (PB) cases and absence of a method of in vitro cultivation of M. leprae. Bacteriological parameters are useful in multibacillary (MB) leprosy, whereas in PB leprosy, the criteria for relapse depend primarily on clinical features. Although there are no widely available serologic tests for leprosy other than in a research setting, various immunological tests may be useful for monitoring patients on chemotherapy as well as for confirming suspected cases of relapse. The main differential diagnoses for relapse are reversal reactions, erythema nodosum leprosum and reactivation/resistance/reinfection. The most reliable criteria for making an accurate diagnosis of relapse include clinical, bacteriological and therapeutic criteria. Additional ones that may be used, depending on the setting, are histopathological and serologic criteria. Relapsed cases of leprosy should be identified and put back on chemotherapy as soon as possible to prevent further disability and transmission of infection. Factors that should be considered in choosing an appropriate regimen are the type of leprosy (PB or MB), previous treatment and drug resistance. Occasionally, clinicians may need to use their judgement to modify the standard WHO treatment regimens according to the scenario in each patient.