Orf with unusual features.

We describe a case of Orf in an immunocompetent man with no history of direct contact with farm animals. The patient presented with numerous large lesions on hands and feet including a lesion in the subungual area. Later on multiple lesions with more bizarre morphology developed on the trunk. The diagnosis was suspected on clinical appearance of the lesion and confirmed later by histopathology. We consider contact with contaminated soil as the possible source of infection as virus can survive in pastures. Awareness of unusual clinical patterns of known entities is important to avoid unnecessary interventions.

Langerhan cell histiocytosis: a child presenting with nine months history of fever.

We report a case of Langerhan Cell Histiocytosis in a three and a half years old child. The child presented with history of low grade fever, off and on for 9 months. There was 2 months history of progressive pallor and 5 days history of epistaxis. Blood complete picture revealed pancytopenia and the patient was referred to a paediatric oncologist. Initial diagnosis of acute lymphoblastic leukaemia (ALL), Lymphoma and disseminated Tuberculosis (TB) was made on basis of initial investigations. Coetaneous involvement occurred 7 months later along with Diabetes Insipidis, bone changes and pulmonary involvement.

Facial lichen nitidus actinicus.

We report an 18-year-old woman with lichen nitidus with exclusive facial distribution.

Late onset erythropoietic porphyria (Gunther's disease).

Congenital erythropoietic porphyria (CEP) or Gunther's disease is inherited disorder of porphyrin heme synthetic pathway that usually presents early in life. A very rare form of this disease has its onset in later years of life, called late onset erythropoietic porphyria (late onset EP). Fourteen cases of late onset EP have been reported to-date. We report another case of this rare entity in a 40 years old male with associated findings of haemolysis and thrombocytopenia.

Paraneoplastic Addisonian pigmentation and acquired ichthyosis as presenting features of multiple myeloma.

Black brown hyperpigmentation of the mucosae, sunexposed skin, palmar creases and frictional sites (Addisonian pigmentation) is characteristic of Addison disease. However, it can also occur as a paraneoplastic manifestation of tumours like bronchogenic carcinoma. Acquired ichthyosis starts later in life and can also be a paraneoplastic presentation. We report a unique combination of paraneoplastic Addisonian pigmentation and acquired ichthyosis as presenting features in a patient with undiagnosed multiple myeloma. To the best of our knowledge this combination of paraneoplastic dermatosis has not been documented before in multiple myeloma. It is concluded that the presence of more than one suspicious dermatosis may be an indicator of being paraneoplastic requiring necessary work-up.

Constitutional and behavioral risk factors for chilblains: a case-control study from Pakistan.

OBJECTIVES: To study constitutional and behavioral risk factors for chilblains in patients at Abbottabad and Sialkot, Pakistan. METHODS: One hundred patients and matched controls completed a single-page, close-ended questionnaire which included demographic data and questions related to possible constitutional and behavioral risk factors for chilblains. Computer program SPSS-10 was used to manage and analyze the data. Risk factors were identified statistically by determining odds ratios and 95% confidence intervals using multivariate analysis. RESULTS: There were an equal number of male and female respondents in each group. Age of the patients and controls ranged from 2 to 80 years with a mean of 24.51 +/- 16.02. Twenty-six patients and 3 controls had a positive family history for chilblains (odds ratio = 9.33); 42 patients and 14 controls reported a history of constipation (odds ratio = 2.69); 32 patients and 8 controls had a history of either numbness or tingling of fingers or toes (odds ratio = 2.93); 55 patients and 45 controls led sedentary lifestyles (odds ratio = 1.27); 85 patients and 58 controls consumed a low number of cups of tea daily (odds ratio = 3.20); 65 patients and 29 controls frequently washed their hands and/or feet (odds ratio = 4.93); and 56 patients and 33 controls had occasional sun exposure during winter months (odds ratio = 2.08). CONCLUSION: Significant risk factors for the development of chilblains for people at Abbottabad and Sialkot included a history of chilblains in first-degree relatives, numbness and tingling sensations of fingers or toes, frequent hand or feet washing, and lower tea consumption during winter months.

Depression in adult dermatology outpatients.

OBJECTIVE: To determine the frequency of depression in adult male dermatology outpatients. STUDY DESIGN: Cross-sectional study. PLACE AND DURATION OF STUDY: Combined Military Hospital, Bahawalpur, from January to March 2007. METHODOLOGY: A consecutive sample was screened for depression by using Urdu version of General Health Questionnaire-12 (GHQ-12). The final diagnosis of depression was based on criteria of International Classification of Diseases-10. RESULTS: Out of the 114 adult males with dermatological disorders, 39 (34.11%) had depression. The frequency and percentage of depression in dermatological conditions was 6 (100%) in psychocutaneous disorders, 2 (66.6%) in urticaria, 3 (66.6%) in pruritis, 7 (57.5%) in acne vulgaris, 4 (50%) in psoriasis, 4 (44.4%) in vitiligo, 3 (37.5%) in melasma, 1 (33.3%) each in hyperhidrosis and alopecia areata, and 9 (20.4%) in eczema. It was not recorded in leprosy and chronic fungal infections. CONCLUSION: Depression was frequently in adult males with dermatological disorders especially psychocutaneous disorders, urticaria, pruritis, acne vulgaris and psoriasis. Depressive symptoms should be specifically explored even at busy dermatology outdoors for early recognition and timely appropriate psychiatric referral.

Rosai Dorfman disease--a rare entity report of two cases with nodal and extranodal involvement.

Rosai Dorfman Disease (RDD) also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML)is a very rare variety of reactive histiocytoses. It commonly involves cervical lymph nodes; although involvement of other lymph node regions, skin and other organ involvement can occur. It has a good prognosis so there is a need to differentiate it from other lympho proliferative disorders of poorer prognosis. We present two cases of this rare disorder illustrating its clinical spectrum; a 22 years old woman with involvement of submandibular lymph nodes, nasal septum and sub-glottic region and a 45 years old male with prominent skin involvement.

Localized lesions in secondary syphilis.

The clinical manifestations of secondary syphilis are variable and can mimic many skin diseases, mostly being generalized and symmetrical in distribution. Localized lesions of secondary syphilis are rarely seen in dermatology clinics. We report an unusual presentation wherein a patient had localized lesions over face and soles only. There is a need for increased awareness on the part of physicians to recognize new patterns of syphilitic infection, together with a willingness to consider the diagnosis of syphilis in patients with unusual clinical features.

Papulonecrotic tuberculids associated with uveitis.

Papulonecrotic tuberculids represent an immunological expression of an internal focus of tuberculosis in an individual with a moderate or high degree of immunity. It responds to anti-tuberculosis treatment and is characterized by an eruption of necrotizing papules occurring in symmetrical crops, particularly affecting the elbows, knees, buttocks and face. A case of papulonecrotic tuberculids associated with immune mediated unilateral uveitis is reported in which all investigations were negative for tuberculosis except a strongly positive tuberculin test. There was an excellent response to anti-tuberculosis treatment. The association of immune mediated uveitis with papulonecrotic tuberculids has not been described before.

Klippel-Feil syndrome with situs inversus--a rare association.

Klippel-Feil Syndrome (KFS) is a congenital anomaly characterized by a defect in the formation or segmentation of the cervical vertebrae. The clinical triad consists of short neck, low posterior hairline and limited neck movement. Multiple congenital anomalies have been associated with this disease. This is a case of KFS in a young girl along with situs inversus, which is an extremely rare association. Various systemic associations occurring in this multi-system disorder are also discussed.

Clinical study of papular urticaria.

OBJECTIVE: To determine the clinical features and demographic profile of the patients having papular urticaria. STUDY DESIGN: A case-series. PLACE AND DURATION OF STUDY: Dermatology Department of Combined Military Hospital, Abbottabad from January to December 2006. PATIENTS AND METHODS: Individuals of all age groups and either gender either suspected of or having definite history of insect bite were included in the study. A specially-designed proforma was filled for each patient separately. The proforma included demographic features, information regarding clothing and sleeping habits, personal or family history of atopy and clinical patterns of the lesions. Computer programme SPSS 10 was used to manage and analyze the data. RESULTS: Out of 280 patients, 201 (71.8%) were children upto 12 years of age, 178 (63.6%) were males, 91 (32.5%) had atopy, 194 (69.3%) were non-locals, 212 (75.7%) came from urban/peri-urban areas and 173 (61.8%) presented during May-August. Lesions were present over exposed parts of the body in 36 (12.9%), arranged in groups in 152 (54.3%) and were papular urticaria in 185 (66.1%) patients. CONCLUSION: Children, adult males, non-locals and those belonging to urban/peri-urban areas are more vulnerable to papular urticaria in a particular region. Papular and urticarial lesions arranged in groups over both exposed as well as covered body parts of a single patient is the most common clinical pattern.

Secondary syphilis lesions resembling pityriasis lichenoides.

This case report describes a male patient who presented with generalized, centrally-ulcerated papules with crusts and hypopigmented macules. Initially, differential diagnostic considerations included pityriasis lichenoides but the serology for syphilis was positive and there was a rapid response to penicillin with clearing of the lesions at the end of three weeks treatment.

Juvenile onset classical pityriasis rubra pilaris: every patient may not require systemic therapy.

Juvenile onset classical Pityriasis Rubra Pilaris (PRP), although rare, is the most common presentation of PRP in children. It has good prognosis and resolution over a few months. Use of potentially hazardous systemic drugs in a disease, that is likely to resolve spontaneously, needs to be assessed carefully. We report a 09 years old boy suffering from this rare disorder, whose disease is well under control with emollients and a combination of diluted topical steroids and keratolytics, for over 06 months.

Granulomatous cheilitis: sustained response to combination of intralesional steroids, metronidazole and minocycline.

Various conservative methods for treatment of labial swelling in patients with granulomatous cheilitis have been attempted, often with only moderate success. We report a case of granulomatous cheilitis who showed excellent sustained response to combination of intralesional steroids, metronidazole and minocycline for initial one month followed by prolonged maintenance treatment with minocycline alone.

Hair coil strangulation--an uncommon cause of penile edema.

Penile strangulation by hair coil is an uncommon, under-recognized condition with a spectrum of potential complications including urethral transection and partial or complete penile amputation. Early recognition and treatment can greatly reduce the incidence of these devastating complications. We report a 5-year-old, circumcised boy with penile edema and erosions secondary to a hair wrapped around the coronal sulcus. Prompt diagnosis and removal of the hair led to rapid resolution without surgical repair. We also analyze the possible underlying causes which can lead to strangulation of the penis with a hair.

Anaphylaxis during anesthesia - role of skin prick testing in diagnosing multiple sensitivities.

The true incidence of anaphylactic reactions and their associated morbidity and mortality remain poorly defined. This is due to uncertainties in reporting accuracy and exhaustivity. A 23 years old male developed severe anaphylaxis under general anesthesia with cardiovascular collapse, bronchospasm and angio-edema. He was promptly managed with intravenous Adrenaline, Hydrocortisone, colloid solutions and Promethazine. The temporal relation suggested Atracurium to be the cause. However, skin prick test, performed 8 weeks later, showed a very strong (+++) positive test for Atracurium and mild sensitivity (+) with Suxamethonium. No sensitivity to Nalbuphine, Thiopentone or Pancuronium was noted. The relevant information was endorsed in patient's medical record sheet for his future exposure.

Exuberant ulcerated lesions of secondary syphilis on the palms--an unusual presentation.

Ulcerated lesions in secondary syphilis are rare. Exuberant tissue is seen in condylomas of syphilis, which occur in flexural and occluded areas of the body and not in open areas such as palms. We report a case of secondary syphilis that presented with ulcerated exuberant lesions on palms, which on initial examination gave an impression of lesions of Orf and Milker's Nodule.

Simultaneous onset of herpes zoster in a father and son.

The Varicella Zoster virus persists in sensory nerve ganglion cells after chicken pox and gets reactivated to cause herpes zoster after variable periods of time as a result of waning of specific cellular immunity. Susceptible contacts of herpes zoster can develop chicken pox and very rarely herpes zoster. We report an interesting case of a father and his son who developed herpes zoster simultaneously without any obvious common predisposition and discuss the possible underlying mechanism.