Increase in the incidence of dapsone hypersensitivity syndrome--an appraisal

There has been an increase in the reports of dapsone hypersensitivity syndrome (DHS) in the past few years, coinciding with the introduction of multidrug therapy (MDT) for leprosy world-wide. The exact cause of this phenomenon is not clear. We report four cases of DHS observed among 252 leprosy patients on MDT and one case of DHS in a patient taking dapsone for nodulocystic acne in the Dermatology Department of the Osmania General Hospital, Hyderabad, India, between June 1997 and January 1999 with few unusual features. In two of these five patients maculopapular rash was severe and progressed to erythroderma. Introduction of MDT in 1982 has not only decreased the prevalence of leprosy but also brought about a positive change in the attitude of people which increased the voluntary reporting of leprosy patients. This, coupled with improvements in organization of leprosy control and awareness among medical personnel of DHS, are probably the most important reasons for the increased reporting of DHS in recent years.     

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

Background

Erythroderma is characterized by erythema and scaling affecting more than 90% of the body surface area. Inflammatory, neoplastic and, more rarely, infectious diseases may culminate with erythroderma. Diagnosis of the underlying disorder is therefore crucial to institute the appropriate therapy. Leprosy is a chronic infectious disease that is endemic in Brazil. Here we present an unusual case of leprosy and reversal reaction causing erythroderma, and we discuss the underlying immunological mechanisms which could contribute to the generalized skin inflammation.

Case presentation

We report a case of a patient with reversal reaction (RR) in borderline borderline leprosy presenting with erythroderma and neural disabilities. Histopathology of the skin showed regular acanthosis and spongiosis in the epidermis and, in the dermis, compact epithelioid granulomas as well as grouped and isolated bacilli. This duality probably reflects the transition from an anergic/multibacillary state to a state of more effective immunity and bacillary control, typical of RR. Leprosy was successfully treated with WHO’s multidrug therapy, plus prednisone for controlling the RR; the erythroderma resolved in parallel with this treatment. Immunologic studies showed in situ predominance of IFNγ?+?over IL-4+ lymphocytes and of IL-17+ over Foxp3+ lymphocytes, suggesting an exacerbated Th-1/Th-17 immunoreactivity and poor Th-2 and regulatory T-cell responses. Circulating Tregs were also diminished. We hypothesize that the flare-up of anti-mycobacteria immunoreactivity that underlies RR may have triggered the intense inflammatory skin lesions that culminated with erythroderma.

Conclusions

This case report highlights the importance of thorough clinical examination of erythrodermic patients in search for its etiology and suggests that an intense and probably uncontrolled leprosy RR can culminate in the development of erythroderma.

     

Netherton’s syndrome and lepromatous leprosy: a mere coincidence?

Objectives Netherton’s syndrome (NS) is a rare autosomal recessive condition, first described in 1958, which involves a complex immunological dysfunction, ichthyosiform dermatitis, and erythroderma, characteristic defects of the hair shaft and atopy. Recurrent bacterial infection in the skin of patients with NS is frequent. Methods This paper represents the first case report of leprosy and concurrent NS. Discussion This case merits discussion among doctors in endemic and non‐endemic areas to evaluate the chronic use of systemic corticosteroids as a risk factor for leprosy. The present patient came from an endemic area of leprosy and was treated chronically with systemic corticosteroids for erythroderma. This treatment, along with the immunodeficiency related to the syndrome and caused by a genetic mutation in SPINK5, may be a facilitating factor for the infection.     

Expansion of large granular lymphocytes in psoriatic erythroderma. A case report

A psoriasis patient developed erythroderma after the withdrawal of a self-administered chronic topical glucocorticoid therapy. A marked expansion of cells with the morphological and phenotypic features of large granular lymphocytes was noticed in peripheral blood. Functional investigations revealed that these cells responded poorly to polyclonal activators and exhibited antibody-dependent cellular cytotoxicity and natural killer activity. Blood abnormalities completely subsided in about two months in the absence of any cytostatic therapy and coincided with the recovery from the erythroderma and the spreading of classical psoriatic plaque lesions. This excluded an underlying malignant process. This patient represents the first report of a previously undescribed immunological disorder in psoriatic erythroderma.     

Zusammentreffen einer Psoriasis und einer kongenitalen lamellären Ichthyose

An erythrodermic skin disease occurring in a patient with an already existing erythroderma of different cause is a rare phenomenon. A 13 year old girl with congenital lamellar ichthyosis suffered from both erythroderma and generalized scaling. Probably at the age of 11, a clinically not recognized psoriatic erythroderma appeared associated with a pustular palmoplantar psoriasis of the Barber-Königsbeck type as well as a psoriatic osteoarthropathy. The identification off such overlapping disorders is of great importance for proper therapy.     

Generalized mucinosis in a patient with erythroderma

We describe an 81-year-old Japanese patient with erythroderma overlapping with widespread and symmetrical deposits of mucin in the upper dermis. Clinically, the mucinous lesions on the nape and upper trunk were localized papular mucinosis. Histologically, there was a perivascular infiltrate of lymphohistiocytic cells mingled with plasma cells in the upper dermis but no sclerosis. Immunohistochemical staining revealed that more than 90% of these infiltrating plasma cells produced immunoglobulin lambda-chain. Both the erythroderma and generalized mucinosis responded to topical steroid and PUVA therapy. To the best of our knowledge, this is the first case of erythroderma accompanied by generalized mucinosis.     

儿童红皮病16例临床分析

目的分析儿童红皮病的病因,探讨儿童红皮病的治疗。方法对16例儿童红皮病住院病人进行回顾性分析。结果儿童红皮病多数继发于其他皮肤病,包括银屑病6例、湿疹3例、异位性皮炎1例、脱屑性红皮病1例、药物性红皮病2例,特发性(原因不明)3例。结论寻找红皮病的病因有助于指导治疗。     

Coexistent leprosy and lupus erythematosus

We present a patient who, while under therapy with systemic corticosteroids to control lupus erythematosus, noted lesions of leprosy (Hansen's disease) on her elbow and knee. The corticosteroids may have played a role in the emergence of the leprosy. Leprosy should be considered in the differential diagnosis of cutaneous lesions with sensory loss to hasten diagnosis and therapy.     

Leprosy treatment during pregnancy and breastfeeding: A case report and brief review of literature

Leprosy is a chronic disease which primarily affects the skin, mucous membranes and peripheral nerves due to Mycobacterium leprae. It is now infrequent in Europe and is rarely reported during pregnancy. Leprosy can be exacerbated during pregnancy, and without treatment it can permanently damage the skin, nerves, limbs and eyes. Therefore, it is important to treat leprosy during pregnancy. This article describes a patient with multibacillary lepromatous leprosy who was treated with multidrug therapy during pregnancy and breastfeeding. The patient delivered a healthy baby girl without perinatal complications, and the infant's growth and development were normal during the 1‐year follow‐up period. Multidrug therapy consisting of dapsone, rifampicine, and clofazimine is highly effective for people with leprosy and considered safe, both for the mother and the child. Antileprosy drugs are excreted into human milk but there is no report of adverse effects except for skin discoloration of the infant due to clofazimine. Multidrug therapy for leprosy patients should be continued unchanged during pregnancy and breastfeeding.     

Efficacy of hydroxyurea in the treatment of a patient with erythrodermic psoriasis and chronic myelogenous leukaemia

A patient presenting with erythrodermal symptoms was diagnosed as having psoriatic erythroderma and chronic myeloid leukaemia. Both pathological conditions were effectively controlled with hydroxyurea within 9 months after diagnosis, without occurrence of adverse reactions or intolerance to therapy.     

Penile tuberculoid leprosy in a five year old boy

A 5-year-old contact of a lepromatous leprosy patient with a tuberculoid lesion on the anterior aspect of the shaft of the penis is reported. The child was clinically suspected to have borderline tuberculoid leprosy during a survey of contacts of leprosy patients, which on histopathology revealed features of subpolar tuberculoid leprosy. The father of the child was recently detected as a case of lepromatous leprosy and was started on multibacillary regime of WHO multidrug therapy. The reason for the localization of the lesion to the shaft of the penis is also suggested. Skin as a route of transmission of tuberculoid leprosy is also emphasized.     

红皮病性银屑病致病因素及治疗方法探讨

目的分析红皮病性银屑病病因及治疗方法。方法 回顾性研究。治疗方法:①中药(丹参或清开灵注射液)+支持疗法:用于初次发作、临床症状较轻者,治愈率75％,平均用29.67 d;②免疫抑制剂(甲氨喋呤,新山地明,雷公藤多甙)或维甲酸类制剂(体卡松,新体卡松)+中药(丹参或清开灵注射液)+支持疗法:用于临床症状较重、无用此类药物禁忌者,治愈率分别为72.2％、73.9％,平均用49.62 d、49.24 d。结果>40岁者发病率高(82.2％)。内服“中药”、激素减量过快或突然停药、上呼吸道感染是主要诱因。结论免疫抑制剂或维甲酸类制剂+中药+支持疗法治疗红皮病性银屑病安全有效,可作为首选疗法之一。     

Vitamin D Deficiency After Oral Retinoid Therapy for Ichthyosis

Oral retinoids are being increasingly used to treat ichthyotic disorders in children. We report on two children with ichthyotic disorders who developed unusual manifestations after they were started on oral retinoids. The first case is a 10‐year‐old girl with nonbullous ichthyosiform erythroderma and the second is a 2‐year‐old girl with lamellar ichthyosis. The child with ichthyosiform erythroderma developed features of rickets within months of initiation of systemic retinoids. Her baseline examination before initiation of oral retinoids was normal. The second patient with lamellar ichthyosis was found to have low vitamin D levels after 6 months of retinoid therapy, and prompt supplementation reversed the levels in 2 months. These cases are being reported to bring attention to the probable need for initiation of vitamin D supplementation with systemic retinoid therapy in ichthyotic disorders in children.     

Generalized vitiligo after erythroderma

We report a 65-year-old black man whose onset of an erythroderma was followed in 6 weeks by a generalized vitiligo. The etiology of the erythroderma was unclear, although a prostate adenocarcinoma was found on evaluation. This is to our knowledge the first patient reported with a vitiligo following exfoliative dermatitis, and the first report of two uncommon markers of internal malignancy (erythroderma and vitiligo) occurring with prostate adenocarcinoma in the same patient.     

Shall we continue with nerve trunk decompression in leprosy?

There is a tendency to compare the results of surgery with that of oral corticosteroid therapy in leprous neuritis as if the two are competing methods. Surgery helps by removing the external compressive force and improves circulation so that steroids can reach and effectively act at the site of inflammation, minimizing the ischaemic and compression damage to nerve fibres. Often nerve decompression in leprosy is requested rather late so that the desired results are not always achieved. With emphasis on "elimination of leprosy", the disease is being managed in endemic states by field programmes where individual patient is not the priority unlike in the general hospitals and among practitioners where the welfare of the patient is the priority. It is therefore important to create awareness about compression neuropathy in leprosy and the need for combination therapy so as to bring down the morbidity and disability.     

Involvement of genitofemoral nerve with genital lesions in lepromatous leprosy

The case of a male patient diagnosed to have lepromatous leprosy with type 2 reaction on multibacillary multidrug therapy, with unusual, widespread involvement of genitalia in the form of plaque and nodules of leprosy over scrotum and perimeatal region of glans, necrotic lesions of erythema nodosum leprosum over scrotum, neuritis of genital branch of genitofemoral nerve bilaterally, and azoospermia, is reported.     

LOCALIZED LEPROMATOUS LEPROSY AND ITS RESPONSE TO CHEMO-IMMUNOTHERAPY

Background. This is an unusual presentation of lepromatous leprosy (ll ) in a young boy, 12 years of age. The study forms part of a large scale immunotherapeutic trial with Mycobacterium w (M.w) antileprosy vaccine. The trial is being conducted in two major hospitals in New Delhi, India. Materials and Methods. This patient presented with three lesions: one on each forearm and the third on the left leg. He was classified initially as borderline tuberculoid leprosy. Slit-skin smears and histopathology from the lesions proved the diagnosis to be lepromatous leprosy with a bacterial index (bi ) 6+. The initial lepromin test was negative. The patient was treated with chemo-immunotherapy (standard multidrug therapy and immunotherapy with Mycobacterium w vaccine). Results. Investigations after 1 year (15 months) of multidrug therapy and three doses of vaccine, showed a remarkable fall in the bi from 6 to 0 in the lesions, a lepromin positivity of 5 mm, and a histological upgrading from lepromatous leprosy to borderline tuberculoid. Immunologic studies at 15 months revealed a good LTT response and high levels of cytokines, specifically IL-2 and IFN-γ. Conclusions. This report presents an LL patient with disease limited to a few sites. It stresses the importance of slit-smear and biopsy in all patients of leprosy, and it highlights the upgrading observed on administration of chemo-immunotherapy.     

麻风患者166例统一联合化疗后2年随访

目的 探讨6个月麻风统一联合化疗方案对各型麻风患者的疗效。方法 对166例各型麻风患者采取世界卫生组织多菌型方案治疗6个月,观察临床和细菌学方面的疗效。结果 在166例患者中因各种原因退出31例,完成治疗并随访2年的患者135例。135例疗前查菌阴性者为45例（33.3%）,其余90例患者细菌指数从0.1 ~ 6.0不等,细菌指数疗前平均为2.91 ± 1.45,在停止治疗第2年末,45例细菌阴性患者显示总的皮损消退和改善率达到93.3%。神经体征改善率达80.0%。在90例查菌阳性患者中,皮肤损害消退和改善率达95.6%,神经改善率达77.8%。疗前细菌阳性的90例患者中有49例患者细菌阴转,占54.4%,平均细菌指数降低为0.66 ± 0.99。从开始治疗后的2.5年中,平均每年下降 0.9。在完成治疗停药随访满2年的135例患者中有25例发生麻风反应。其中Ⅰ型和Ⅱ型反应分别为13例和12例。在166例患者中,有1例多菌型患者在停止治疗后13个月复发。结论 统一联合化疗的近期疗效与常规MDT方案治疗2年的结果相似,其反应发生率差异以及远期复发率尚待观察。     

Type 2 lepra reaction with HIV1 co-infection: a case report with interesting management implications

A patient co-infected with leprosy and Human Immunodeficiency Virus (HIV)-type 1 who developed type 2 lepra reaction in the absence of antiretroviral therapy is presented. The reaction responded only after initiating anti retroviral therapy (ART) despite normal CD4+ counts. The present report suggests that type 2 reactions in leprosy and HIV co-infected patients may not always be the typical manifestation of immune reconstitution inflammatory syndrome (IRIS) and stresses the importance of considering concomitant HIV infection in refractory lepra reactions. Extensive research is required into the manifestations of HIV in leprosy patients.     

Some unusual type 2 reactions in leprosy

Background Type 2 reactions with lepromatous leprosy (LL) not occurring during multi‐drug therapy (MDT) have been reported. Methods Three patients have been described, each representing a prototype, the first presenting as bullous erythema nodosum leprosum (ENL), second with ENL erupting after treatment for co‐existing pulmonary tuberculosis and resembling immune reconstitution inflammatory syndrome, and a third patient with recurrent Sweets‐syndrome like presentation who had taken incomplete MDT in the past for leprosy. In all, the diagnosis was established by demonstration of acid‐fast bacilli (AFB) on slit‐skin smears (SSS) and histopathology. Results & Conclusion The fact that reactions can occur in patients with clinically inapparent LL, who are more likely to present in general hospitals, has been reemphasized to enhance awareness among physicians. First presentation of leprosy as ENL is probably precipitated by common antibiotics taken for other illnesses. Since reactional episodes can occur before, during and after MDT for leprosy and the clinical picture is not specific to any of them, it is important to ascertain the status of anti‐leprosy therapy during these episodes and treat them accordingly.