Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review.

Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare childhood disease with characteristic cutaneous and rheumatic manifestations. Cutaneous manifestations include a combination of nodules affecting peri-articular (especially interphalangeal joints) and head and neck areas; and linearly arranged ivory white papules over an erythematous indurated skin. Despite a benign course, an abrupt onset of symptoms with extensive cutaneous involvement often leads to parental anxiety, overenthusiastic evaluation and sometimes aggressive treatment. A peculiar cutaneous distribution in SHJCM including nodular lesions and periorbital edema, arthritis and arthralgia in a few cases, may simulate juvenile dermatomyositis. It is, therefore, important for dermatologists and pediatricians to be aware of this entity. In this report, we describe two cases of SHJCM and briefly review similarly reported cases in children.

Spectrum of mucocutaneous manifestations in an Asian cohort of patients with leukemia.

BACKGROUND: Literature on cutaneous manifestations of leukemia is limited. OBJECTIVE: To determine the pattern of mucocutaneous manifestations in adult Asian patients with leukemia and to establish their relation with the leukemia type. SUBJECTS AND METHODS: After previous consent, 196 consecutively registered patients with leukemia aged ≥18 years were recruited. All patients were prospectively followed for 3 months to evaluate the patterns of mucocutaneous involvement. The mucocutaneous manifestations were categorized into specific lesions with leukemic infiltration and non-specific lesions. RESULTS: Seventy-nine (40.3%) of 196 (males 128 and females 68) recruited patients showed one or more mucocutaneous manifestations. The total number of complaints observed was 87 with mean number of dermatoses per patient being 0.44. Specific manifestations (leukemia cutis) were present in six (3.06%) and nonspecific mucocutaneous manifestations in 73 (37.2%, reactive dermatoses n = 21 and infections n = 52). Cutaneous viral infections were significantly associated with acute lymphoblastic leukemia (P < 0.005). Antiviral prophylaxis with acyclovir significantly reduced the incidence of varicella-zoster infection (P = 0.016). CONCLUSION: Cutaneous manifestations are common in Asian patients with leukemia, and a thorough cutaneous examination will aid in their management.

Intralesional Mycobacterium w Vaccine Versus Cryotherapy in Treatment of Refractory Extragenital Warts: A Randomized, Open-Label, Comparative Study.

BACKGROUND: Initial reports of immunotherapy using intralesional Mycobacterium w (Mw) vaccine have documented its useful role in treatment of genital and extragenital warts. OBJECTIVES: To compare the efficacy and safety of intralesional Mw vaccine versus cryotherapy in the treatment of refractory extragenital warts. METHODS: This was a prospective, randomized, comparative study of 66 patients. The outcome was assessed in terms of complete clearance of warts and change in Dermatology Life Quality Index (DLQI) score. RESULTS: Complete clearance of treated warts was seen in 66.7% (20/30) and 65.5% (19/29) of patients in the Mw and cryotherapy groups, respectively (P = .769). Clearance of distant warts was significantly (P = .004) high in the Mw group. Improvement in DLQI was greater in the Mw group. Both treatment modalities were well tolerated, and no major side effects occurred. CONCLUSIONS: Mw vaccine and cryotherapy are equally efficacious in treatment of refractory extragenital warts. Mw vaccine has an added advantage of clearance of distant warts.

Intralesional Rituximab in the Treatment of Refractory Oral Pemphigus Vulgaris.

IMPORTANCE: Oral lesions of pemphigus vulgaris are usually recalcitrant and respond slowly to treatments. Corticosteroid injection is considered to be the most effective local treatment in oral pemphigus vulgaris. However, intralesional corticosteroids are not effective in all remnant lesions. In 3 such patients with pemphigus vulgaris, we evaluated the utility of 2 injections (on days 1 and 15) of intralesional rituximab, 5 mg/cm², in terms of accelerated healing, limitation of the use of systemic immunosuppressants, and reduction of their adverse effects. OBSERVATIONS: Three patients (1 man and 2 women) received 2 doses of intralesional rituximab in March and April 2013. All 3 patients responded to the treatment. In patients 1 and 2, the objective severity score was reduced to 0 at the final visit from a baseline score of 4 and 5, respectively (range, 0-11). The subject severity score in these patients was reduced to 1.0 and 0 from a baseline score of 22.0 and 22.5, respectively. After clinical remission was achieved, patient 3 developed a relapse of mucosal lesions. At the final visit, all of the patients were satisfied with the treatment, with a mean satisfaction score of 8 (maximum score, 10). We found a marked decline in the CD19 cell count from a pretreatment mean count of 287 cells/µL to 6 cells/µL on day 15 after a single intralesional rituximab injection. Adverse events were limited to local pain in 1 patient. CONCLUSIONS AND RELEVANCE: Intralesional rituximab administration lacks the adverse effects of intravenous administration. This method reduces the amount of drug administered and therefore is less expensive. Encouraging results from our study should prompt further evaluation of this novel route of rituximab administration in patients with refractory oral pemphigus vulgaris.

Use of rituximab in pemphigus patients with chronic viral hepatitis: report of three cases.

Use of rituximab in patients with chronic viral hepatitis can worsen pre-existing hepatitis or reactivate occult infection. There are no reports of use of rituximab in pemphigus patients with co-existing viral hepatitis. Herein, we report three pemphigus patients with co-existing chronic viral hepatitis (hepatitis C (n = 2), hepatitis B (n = 1)), who were treated successfully with rituximab under close supervision and concurrent antiviral drug administration. There was no derangement of the liver function tests or increase in viral load in any of the patients. By incorporating good collaboration with a hepatologist and close follow-up, such patients can be managed successfully with biologic therapies when the conventional treatment modalities have failed.

Vitamin D deficiency and lower TGF-ß/IL-17 ratio in a North Indian cohort of pemphigus vulgaris.

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune bullous disease caused by acantholysis of keratinocytes due to pathogenic desmoglein-3 autoantibodies. Role of vitamin D has been recently implicated in various autoimmune conditions due to its immunomodulatory effects on innate and adaptive immune responses. One of the key mechanisms of the immune regulation by vitamin D is through its anti-inflammatory effects by suppression of Th17 functions. Thus, vitamin D may be involved in pathogenesis of PV. In this study, the serum vitamin D, IL-17 and TGF-ß levels in PV patients as well as healthy controls were estimated in order to understand the underlying immune mechanism involved in disease pathogenesis. RESULTS: This retrospective study included 30 biopsy proven PV patients' sera. Ten age matched volunteers without any cutaneous or autoimmune conditions were recruited as healthy control (HC). Serum Vitamin D levels were measured using chemiluminescence, whereas IL-17 and TGF-ß levels were determined using ELISA. All patients showed deficient vitamin D levels (11.1 ± 5.8 ng/ml). Moreover, all the PV patients had elevated serum IL-17 levels (210.7 ± 105.3), whereas it was not detectable in any (n = 10) of the healthy controls sera (ELISA sensitivity ≥ 8 pg/ml). The mean serum TGF-ß concentration was also lower in patient sera as compared to healthy control, and the TGF-ß/IL-17 ratio was drastically reduced in patients (30.30 ± 28), as compared to healthy controls (1363.34 ± 559.52). CONCLUSIONS: Hypovitaminosis is common in North India, as ascertained by deficient levels in healthy controls, and was also consistently observed in PV patient. These low levels were not related to age or gender. The increased serum IL-17 and dramatic reduction in TGF-ß/IL-17 ratio in diseased patients further indicate that dysregulation of the Treg/Th-17 axis of T effector cells may be of significance in pathogenesis of PV. Thus, the study indicates that vitamin D insufficiency may be a predisposing factor in PV, contributing through its role in any of the various adaptive immune mechanisms that regulate T cell functions in vivo. Thus, there is a need to further evaluate the Treg/Th-17 axis, as it may have an important role in disease progression.

Successful use of rituximab in the treatment of childhood and juvenile pemphigus.

BACKGROUND: There is a lack of data on outcomes of management of pemphigus in children. OBJECTIVE: We sought to evaluate rituximab treatment in childhood and juvenile pemphigus. METHOD: All cases of pemphigus treated with rituximab in patients younger than 18 years were included. Clinical and epidemiologic data and details of rituximab administration were recorded. Response to treatment was assessed as control of disease activity, partial remission, complete remission, and relapse/flare. RESULTS: Ten patients aged 9 to 17 years received rituximab treatment. After therapy, they were followed up for a median period of 16 months (range 8-36 months). Complete remission without concomitant therapy was achieved in 7 patients by a mean of 21 weeks. One patient each achieved complete remission (on immunosuppressant therapy), control of disease activity, and partial remission (on immunosuppressant therapy) by 15, 8, and 14 weeks, respectively. Relapse/flare occurred in 6 patients by a mean period of 13 months. Two patients received a second cycle of rituximab infusions with good clinical response. Infusion reactions were the most common adverse event. There were no long-term complications. LIMITATION: Small sample size and retrospective study design are limitations. CONCLUSION: The current data suggest that rituximab is useful in treating childhood and juvenile pemphigus.

Primary cutaneous mucormycosis presenting as a giant plaque: uncommon presentation of a rare mycosis.

Mucormycosis is an uncommon systemic mycosis affecting the immunocompromised individuals. It is usually caused by organisms of the genera Rhizopus and Mucor, although rarely other organisms have also been implicated. Mycoses due to these angioinvasive fungi have an acute onset, rapidly progressive course with high mortality rate. A rare and less well known is the chronic subtype of primary cutaneous mucormycosis (PCM). Herein, we report a case of PCM clinically presenting as a chronic, giant destructive plaque in a young immunocompetent male and coin the term chronic granulomatous mucormycosis. A clinicopathological classification for cutaneous mucormycosis is also proposed.

Angiolymphoid hyperplasia with eosinophilia of the infra-axillary region: report of a case.

Angiolymphoid hyperplasia with eosinophilia is an uncommon, benign hyperproliferative disorder. Papules and nodules occur predominantly in the head and neck region. Involvement of other sites such as the trunk and mucosae has been rarely reported. We herein report a case of angiolymphoid hyperplasia with eosinophilia involving the right infra-axillary region.

Granulomatous invasive aspergillosis of paranasal sinuses masquerading as actinomycosis and review of published literature.

Cutaneous aspergillosis is a common systemic mycosis affecting immunosuppressed patients. Here, we describe a novel morphological type of cutaneous aspergillosis in a young immunocompetent woman who presented with a chronic history of multiple nodules and discharging sinuses over left side of the face, mimicking cervicofacial actinomycosis. Skin biopsy showed granulomatous inflammation, and of septate fungal hyphae with acute-angled branching, morphologically resembling Aspergillus. This was confirmed on fungal culture as Aspergillus flavus.

IgG/IgA pemphigus reactive with desmoglein 1 with additional undetermined reactivity with epidermal basement membrane zone.

IgG/IgA pemphigus is an extremely rare subset of pemphigus, showing anti-keratinocyte cell surface antibodies of both IgG and IgA classes. Herein, we describe a unique case of IgG/IgA pemphigus with clinical features of edematous erythema and peripheral vesiculopustules. Histopathology showed the presence of subcorneal pustules and acantholytic blisters in the mid-epidermis with neutrophilic infiltration and eosinophilic spongiosis. Direct immunofluorescence of perilesional skin showed both IgG and IgA deposits to keratinocyte cell surfaces and unusual granular deposits of IgG, IgM, and C3 along basement membrane zone. On enzyme linked immunosorbent assay , the auto-antibodies were found to be reactive to desmoglein 1 antigen. Various clinical, histopathological, and immunological findings in our case overlapped with the features of IgA pemphigus, pemphigus herpetiformis, and pemphigus foliaceus. These findings indicate that IgG/IgA pemphigus may be a transitional form between IgA pemphigus and pemphigus herpetiformis, and thus provides insight into the pathogenicity of this rare disorder.