1-Dermatological Case Presentation

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Dermatological Case Presentationfrom the Department of Dermatology

at Preah Kossamak Hospitalin Phnom Penh

Sithach MeyDepartment of DermatologyPreah Kossamak-Hospital

Dermatological Case Presentation from the Department of Dermatology AtPreahKossamak Hospital in Phnom PenhSithachMey, MDPreahKossamak Hospital, Phnom PenhParticularly in third world countries, dermatology tends to be not high on the list of priorities, because it is not considered to be a serious medical issue. In the Dermatology-consultation in PreahKossamak Hospital we met very often severs, life-threatening Problems. Sometimes patients can die from complications of dermatological diseases. Here, we report about some difficult and rare cases we met so far:1- A young man presented with an ulcerated, painless plaque on the left wing of the nose which progressed rapidly for about 4 months. His operation in the ENT-Hospital did not render a clear diagnosis. A second diagnostic biopsy was done in our dermatology department which was sent to Germany. Using immunohistopathology, a NK-cell lymphoma was diagnosed. Prevalence of the disease is higher in people of Asian descent than in whites. When compared with other subtypes of lymphoma found in the head and neck region, NK-/T-cell lymphoma carries a much higher mortality rate. Overall, median survival time is reported as 12.5 months. Our patient died three months after the diagnosis from respiratory obstruction. 2- A 56 year- old man presented with multiple infiltrated tumors on trunk and limbs. He had been treated for cutaneous tuberculosis for 6 months already in Cambodia, subsequently he received therapy for deep mycosis for 4 months in Viet Nam. A biopsy was diagnosed as melanoma metastasis in Cambodia. As the diagnosis did not match the clinical picture, German pathologists were asked for additional staining. Thus the diagnosis of malignant Histiocytosis was confirmed. The patient was lost from observation thereafter. 3- The third and the fourth reports we would like to show simple, but rather rare of different types of T-cell Lymphoma (plaque stage and Sezarysyndrom).

Abstract

A young man presented with eczematous plaque on the buttock which was treated for nearly two years by colleagues out side our department as Eczema and Tinea. A biopsy confirmed diagnosis of Mycosis Funggoid. The fourth case report about a 45 years old woman presented with multiple disseminated tumors which are rapidly growing. Her general status was altered (35 kg BW). Histology confirmed about Sézary Syndrome. 4- 7 months old girl referred by Pediatric hospital kunthabopha with a large, infiltrated congenital naevus which is partly ulcerated and presented some nodules in the middle. The girl has breathing difficulty. Malignant melanoma in a giant congenital naevus was diagnosed. The tumor dept was from 2,6 mm to 4, 2 mm. Surgeon in KuthaBopha decided not send her to Oncologist as the tumor was too big for an operative treatment.

Can dermatological problems pose a danger to the patient’s life?

Some dermatoses may threaten the patient’s life indeed, e.g.:

● Erysipelas ● Lupus erythematosus● TEN● Systemic scleroderma● Dermatomysitis● blistering diseases like Pemphigus● some neoplasms of the skin

We report some difficult and/or rare cases we met in the outpatient department of the Department of Dermatology in Preah Kossamak-Hospital (DDPKH) in 2010.

Case 1: History

A young man presented with:- ulcerated, painless plaque on the leftwing of the nose

- which progressed rapidly for about 4months.

Case 1: History

An operation in the Department of ENT of Ang Duong Hospital did not render a clear diagnosis

A second diagnostic biopsy which was sent to a specialized laboratory in Germany was performed in DDPKH

Case 1: Clinical Presentation

Case 1: Histopathological Findings

Necrosis of epidermis

Dense infiltrate of lymphocytes involving dermis and subcutis

Case 1: Histopathological Findings

Small and medium-seized atypical lymphoid cells

Case 1: Histopathological Findings

Immuno-histopathology with - CD3- CD4- CD8 - CD56- cytotoxic marker-> negative

Case 1: Diagnosis

Extranodal NK/T-cell lymphoma

nasal type

Case 1: Comment

the disease affects people of Asian descent more frequently than whites

the mortality rate is higher compared with other subtypes of lymphoma found in the head and neck region

Overall median survival time is 12.5 months

Our patient died three months after diagnosis from respiratory obstruction

Case 2: History A 60 year old man presented with multiple

purple and skin-colored, infiltrated, ulcerated tumors on head, trunk and limbs

He had been treated for cutaneous tuberculosis for 6 months in Cambodia

He received therapy for deep mycosis for 4 months in Viet Nam

Case 2: Clinical Findings

Case 2: Clinical Findings

Case 2: Findings A biopsy was diagnosed as melanoma

metastasis in Cambodia

Additional staining by German pathologists was done as the diagnosis did not match the clinical picture

Diagnosis of malignant histiocytosis was confirmed

Case 2: Comment

Malignant histiocytosis is a malignant condition in which there is uncontrolled proliferation of histiocytes

Atypical histiocytes spread throughout the body; usually affecting liver, spleen, lymph nodes and bone marrow

Diagnosis can be made by blood tests, bone marrow aspirate and tissue biopsies

Case 2: TherapyMalignant histiocytosis responds in most

patients to chemo- or radiotherapy Malignant histiocytosis progresses very

quickly and treatment must be started as early as possible

Some people will not respond to treatment and some people will die before the condition can be diagnosed and treated.

Case 3: History

A young man presented with eczematous plaques on the left hip and on buttocks

He was treated to no avail for nearly two years for eczema and tinea

Case 3: Clinical Findings

Case 3: Clinical Findings

Case 3: Diagnosis and Therapy

Histopathology performed in Hamburg (Germany) confirmed mycosis fungoides

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma

Treatment consisted of a topical betamethasone combined with exposure to ultraviolet light

Case 4: History A 45 year old woman presented with

multiple disseminated, skin-colored, ulcerated nodules and tumors which were growing rapidly

TB and HIV could not be confirmed

Her general status extremely reduced:35 kg BW, weakness, loss of appetite, general malaise

Case 4: Clinical Findings

Case 4: Clinical Findings

Case 4: Clinical Findings

Case 4: Clinical Findings

Case 4: Diagnosis and Comment

Immunohistology:Sézary syndrome

Sézary syndrome is a variant of mycosis fungoides, which occurs in about 5% of all cases of mycosis fungoides

Case 5: History and Findings 7 month old girl referred by Kantha-Bopha

Pediatric Hospital with a large, infiltrated hyperpigmented plaque on lower and middle part of trunk

Plaque was partly ulcerated and presented some central nodules

Patient had respiratory distress due to serious infiltration of the plaque

Case 5: Clinical Findings

Case 5: Diagnosis

Two diagnostic biopsies were taken from a nodule and from a plaque

Immunohistology Münster (Germany): malignant melanoma on giant congenital naevus

Tumor depth:- 2,6 mm (plaque)- 4,2 mm (nodular lesion)

Case 5: Therapy

Prednisolone 1mg/kg BW to improve infiltration

After one month, softening of plaques allowing the patient to breath normally

Surgeon in Kantha-Bopha Pediatric Hospital decided to refer her to oncology as the tumor was too extended for an excision

Case 6: History 33 year old woman, pregnancy month III

Weakness, accompanied by joint and muscle-pain for one month

For about 3 weeks development of multiple purpuric macules and ecchymotic patches on entire body

Gingivorrhagia, erosion and oedema of gums

Case 6: Clinical Findings

Case 6: Clinical Findings

Case 7: History 20 year old woman

Weakness, joint- and muscle-pain,severe head-ache

multiple purpuric macules and ecchymotic patches on body and oral mucosa

Gingivorrhagia, oedema of gums

Case 7: Clinical Findings

Case 7: Clinical Findings

Case 6 and 7:Diagnosis, Comment and TherapyScurvy

Scurvy arises from ascorbic acid (vitamin C) deficiency

Vitamin C plays a crucial role in the formation of collagen, a major component of connective tissue

Treatment: Vitamin C 500mg BIDNutritional education:Intake of fruit and vegetables containing Vitamin C (citrus fruit, kiwi, cabbage and liver)