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.

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.

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.

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.

Use of granulocyte colony-stimulating factor in the treatment of toxic epidermal necrolysis--experience with 3 patients.

The use of recombinant granulocyte colony-stimulating factor has recently been advocated in the treatment of toxic epidermal necrolysis as it may help in faster regeneration of detached skin. Three patients who presented with toxic epidermal necrolysis were managed as per the protocol followed at our department. In addition, they received recombinant granulocyte colony-stimulating factor in a dose of 300 microg/d (5 microg/ kg/d) for 5 days. Severity of Illness Score for Toxic Epidermal Necrolysis was calculated on day 1 and the lesions were observed for re-epithelialization. The 3 patients were aged 16 years, 20 years, and 65 years, with the latter showing leucopenia at presentation. All 3 patients showed a significant rise in total leukocyte count (reaching up to 45,000/mm3) after administering recombinant granulocyte colony-stimulating factor, with the rise being comparatively less in the third patient (maximum of 12,000/mm3). A similar pattern was seen in re-epithelialization of skin, with rapid re-epithelialization occurring in the first 2 patients and much slower re-epithelialization in the third. The first two survived but the third died from refractory sepsis. Recombinant granulocyte colony-stimulating factor improves epithelialization of skin and should be used for treating toxic epidermal necrolysis irrespective of the leucopenic status of the patient.

Atrophic sarcoidosis: an unusual presentation of cutaneous sarcoidosis.

A 66-year-old woman presented with asymptomatic skin-colored to hypopigmented scaly plaques over the extremities, reportedly of 12 years' duration. The lesions started as well-defined erythematous scaly papules over the forearms, gradually followed by the appearance of similar lesions over both legs and dorsum of feet. During this period, the lesions increased in size, with peripheral extension and central clearing, leading to the present morphology. She is a known patient with coronary artery disease currently on treatment. There is history of exertional dyspnea, which has been related to her cardiologic ailment. She is not a known diabetic and has no other significant medical history.

Acroangiodermatitis of Mali in a patient with congenital myopathy.

Pseudo-Kaposi sarcoma is a disease entity that encompasses acroangiodermatitis as well as Steward-Bluefarb syndrome. It has varied etiologies and clinical presentations. Most important distinction is from Kaposi sarcoma and this is mainly histopathological. Here we report a case of acroangiodermatitis in a patient with congenital myopathy and have also discussed its pathogenesis.

Bowen Disease over photoprotected site in an Indian male.

Bowen Disease is squamous cell carcinoma in situ in which the basement membrane is intact on histopathology. Lesions are usually solitary but may be multiple in 10-20 percent of cases. About three-quarters of these lesions are situated on the lower limb. It typically presents as an erythematous enlarging plaque having irregular borders with scaling and crusting. Our patient presented with a lesion on the chest that was not sun exposed thus leading to a diagnostic dilemma.

Bullous eruption in a patient infected with the human immunodeficiency virus.

A 30-year-old man diagnosed with human immunodeficiency virus (HIV) infection 10 years earlier, presented with large tense blisters associated with minimal itching of 10 days' duration. He had no history of oral or genital erosions or ulcerations and showed no symptoms of HIV-related illnesses. Highly active antiretroviral therapy (HAART) had been started 6 weeks earlier when his CD4 count was 116/mL. He initially received nevirapine 200 mg once daily; after 2 weeks with no skin eruptions or other adverse reactions, the dose was increased to 200 mg twice daily. Other components of his HAART included lamivudine and stavudine. The patient was not taking any other prescription or alternative medicines. During the past year, he experienced 4 episodes of intensely itchy urticarial lesions that subsided with antihistamines. The present episode of bullous lesions was also preceded by urticarial lesions. On examination, he had multiple, large, tense bullae over relatively normal-looking skin involving all parts of the body (Figure 1). There were a few well-defined erosions. Nikolsky and bullae spread signs were negative, and no oral or genital erosions or ulcerations were noted. Results of a complete blood count, renal and liver function tests, and chest x-ray were within normal limits. Skin biopsy from one of the blisters showed a subepidermal bulla filled with eosinophils and polymorphonuclear leukocytes (Figure 2). The underlying dermis showed perivascular inflammatory infiltrate composed of polymorphonuclear and lymphomononuclear cells. The overall features were suggestive of bullous pemphigoid. A direct immunofluorescence test could not be done because of possible risk of cross-infection to the operator of the cryostat. Workup for herpes simplex virus and cytomegalovirus infection also could not be performed. HAART was discontinued temporarily with the suspicion that it was the causative factor. The patient was started on oral prednisolone 40 mg/d and topical clobetasol propionate (0.05%). Within 1 week of treatment, he had significant improvement with almost complete disappearance of the lesions. A few small, tense vesicles continued to appear between. Once the lesions completely disappeared, the prednisolone was gradually tapered off and all the components of HAART were resumed. The patient did well without any recurrence of lesions, thus virtually excluding HAART as the cause of the bullous pemphigoid-like eruptions. Subsequently, he did not return for follow-up.

Genital elephantiasis and sexually transmitted infections - revisited.

Genital elephantiasis is an important medical problem in the tropics. It usually affects young and productive age group, and is associated with physical disability and extreme mental anguish. The majority of cases are due to filariasis; however, a small but significant proportion of patients develop genital elephantiasis due to bacterial sexually transmitted infections (STIs), mainly lymphogranuloma venereum (LGV) and donovanosis. STI-related genital elephantiasis should be differentiated from elephantiasis due to other causes, including filariasis, tuberculosis, haematological malignancies, iatrogenic, or dermatological diseases. Laboratory investigations like microscopy of tissue smear and nucleic acid amplification test for donovanosis, and serology and polymerase chain reaction for LGV may help in the diagnosis, but in endemic areas, in the absence of laboratory facilities, diagnosis largely depends on clinical characteristics. The causative agent of LGV, Chlamydia trachomatis serovar L1-L3, is a lymphotropic organism which leads to the development of thrombolymphangitis and perilymphangitis, and lymphadenitis. Long-standing oedema, fibrosis and lymphogranulomatous infiltration result in the final picture of elephantiasis. Elephantiasis in donovanosis is mainly due to constriction of the lymphatics which are trapped in the chronic granulomatous inflammatory response generated by the causative agent, Calymmatobacterium (Klebsiella) granulomatis. The LGV-associated genital elephantiasis should be treated with a prolonged course of doxycycline given orally, while donovanosis should be treated with azithromycin or trimethoprim-sulphamethoxazole combination given for a minimum of three weeks. Genital elephantiasis is not completely reversible with medical therapy alone and often needs to be reduced surgically.

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.

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 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.

Genital elephantiasis.

Genital elephantiasis (esthiomene), which is the dramatic end-result of lymphatic obstruction, is rather rare. Although mainly associated with filariasis and sexually transmitted diseases, such as lymphogranuloma venereum and donovanosis, it could also be an uncommon complication of tubercular lymphadenitis, a common infection in tropical countries. We report a rare case of a 32-year-old Indian female in whom genital elephantiasis occurred as a complication of tubercular lymphadenitis.

Co-existence of variants of porokeratosis: a case report and a review of the literature.

Rarely, different variants of porokeratosis may coexist in an individual patient or their family members. A patient with the linear form of porokeratosis present since birth subsequently developed the disseminated superficial actinic form at a later age. A review of the literature pertaining to the coexistence of variants of porokeratosis suggests a significant association between the linear and disseminated superficial actinic forms. Genetic linkage between different variants and the basis for their association is discussed.

Severe angioedema induced by angiotensin converting enzyme inhibitors: role of precipitating factors.

Angiotensin converting enzyme inhibitors like captopril, enalapril, lisinopril, trandopril and ramipril may rarely induce a life threatening angioedema. We present two cases of severe angioedema induced by enalapril and ramipril along with possible precipitating factors observed in these patients. The importance of prompt recognition and early management of such cases is emphasized.