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46548
An eight year old boy with rash

Session: Premeeting Session: Pediatric Fellows' Day Workshop $
Wednesday, October 8, 2014: 8:40 AM
Room: The Pennsylvania Convention Center: 111-AB
Final Diagnosis:

Hansen’s Disease

Brief history of the Present Illness:

UP is an 8- year old boy with an erythematous, raised rash that has waxed and waned over 5 years. The rash is not pruritic and is not painful. He has had no drainage or oozing from the rash. In 2009, the rash first appeared and he was treated with topical steroids which led to improvement but not to resolution of the rash. He continued to seek treatment for the rash whenever a new lesion would appear. Despite therapy with topical and oral steroids as well as topical and oral anti-fungal medications, the rash never resolved.

No history of fever, joint pain, GI or respiratory complaints.

 

Past Medical History including allergies:

No chronic medical conditions, No hospitalizations, No surgeries.

Key Medications:

He was recently started on hydrocortisone ointment and oral prednisone. He has been treated with clobetasol ointment, oral fluconazole, topical oxiconazole and clotrimazole in the past.

Epidemiological history:

He was born in India. He moved to US in 2009. He has had no sick contacts and no family members with similar symptoms/rash.

Physical Examination:

The patient appeared well. The blood pressure was 129/78 mm Hg, pulse 97 beats per minute, temperature 37.1 C and respirations 22 breaths per minute.

Skin examination revealed raised, erythematous, plaque like lesions with mild central clearing over his upper & lower lip, his chest, right knee and sole of right foot (figure 1 & 4). Multiple, raised, erythematous, scaly, plaque like lesions in a serpiginous pattern were seen over his right upper extremity (figure 2 & 3). The rest of his examination was unremarkable.

 

Studies:

A complete blood count showed a white blood cell count of 6.57 (45%  neutrophils, 45% lymphocytes, 7% monocytes), hemoglobin of 15.8 g/dL, hematocrit of 44%, and platelet count of 256,000. Basic metabolic profile and liver function tests were within normal limits.

 

Clinical Course Prior to Diagnosis:

He was initially treated with topical steroid therapy which made his rash better (less erythematous) but it never completely faded away. He was next treated with topical anti-fungal medications that did not improve his rash. He was later seen by a dermatologist and was treated with topical steroids and oral steroids which made the rash better (decreased erythema and less raised). However, the rash still did not resolve. Therefore, his dermatologist performed a punch biopsy.

 

 

Differential Diagnosis:

 

  1. Tinea corporis
  2. Sporotrichosis
  3. Hansen’s disease
  4. Sarcoidosis
  5. Psoriasis
  6. Granuloma annulare/multiforme

 

 

Diagnostic Procedure(s) and Result(s):

A skin punch biopsy was done. Gram stain, AFB stain and KOH prep were negative. The pathology specimen revealed multiple, non-caseating granulomas in the dermis with some in a linear configuration around peripheral nerves. Several peripheral nerves were entrapped by the granulomas (figure 5 & 6). PAS and AFB stains were negative. M. leprae PCR was weakly positive.

Treatment/Follow-up:

Treatment was initiated with rifampin and dapsone for borderline active tuberculoid disease. He developed a leprosy reaction soon after initiation of treatment and was treated with prednisolone for 2 months. His rash has shown significant improvement after treatment (figure 7 & 8).

Major Teaching points of case:

 

This child has Hansen’s disease (HD) which is caused by Mycobacterium leprae, an obligate, intracellular, acid-fast bacillus. The disease involves the skin, peripheral nerves, eyes, mucosa of the upper respiratory tract, and testes. The Ridley-Jopling classification of HD includes tuberculoid, borderline tuberculoid, mid-borderline, borderline lepromatous, lepromatous and indeterminate. Classification is based on clinical features, histologic features, lepromin reaction response, and bacillary density. The WHO classification scheme for HD includes: single lesion paucibacillary, paucibacillary (2-5 lesions) and multibacillary (6 or more lesions) disease.

The incubation period ranges from one to many years but is usually 3-5 years. 95 percent of the human population is not susceptible to infection with M. leprae.  There is an increased risk for disease development in siblings, parents and children who are close contacts of untreated patients. The major source of infectious material is probably nasal secretions from patients with untreated infection. Minimal shedding of M.leprae occurs from involved intact skin.

Clinical manifestations include: one to multiple skin lesions (flat or raised, light or pigmented) with or without loss of sensation; nodules or plaques; thickened dermis; and /or involvement of the nasal mucosa resulting in nasal congestion and epistaxis.

Histopathologic examination of a skin biopsy is the best method of establishing the diagnosis and is the basis for the classification of leprosy. Fite staining and PCR for M. leprae can be done on biopsy specimens. The primary goal of therapy is the prevention of permanent nerve damage, which can be accomplished by early diagnosis and treatment. Treatment is with dapsone and rifampin x 12 months for pauci-bacillary disease and rifampin, dapsone, and clofazimine x 24 months for multi-bacillary disease.

A leprosy reaction or erythema nodosum leprosum (ENL) can occur after treatment. Clinically, leprosy reactions (a type 1 reaction) are usually evidenced by edema and erythema of pre-existing lesions. Neuritis and occasionally new lesions or fever may also occur. Leprosy reactions are usually treated with steroids. ENL is a type 2 reaction and usually manifests with fever and painful erythematous nodules. However, peripheral neuritis, orchitis, lymphadenitis, iridocyclitis, nephritis, periostitis and arthralgias may also occur. ENL is treated with thalidomide, clofazimine or steroids. ENL occurs almost exclusively in borderline lepromatous and lepromatous patients, and leprosy reactions occur in borderline tuberculoid, borderline and borderline lepromatous patients.

The National Hansen’s Disease Program (NHDP) located in Baton Rouge, Louisiana provides free consultations for physicians, pathologic review of skin biopsies and consultation concerning molecular techniques for identification of M. leprae. The NHDP also provides free antibiotics for leprosy treatment.

Final Diagnosis:

Hansen’s Disease - borderline tuberculoid, paucibacillary

References:

1. American Academy of Pediatrics Red Book

2. Feigin and Cherry Textbook of Pediatric Infectious Diseases; 7thedition

3. Centers for Disease Control & Prevention (Hansen’s Disease)

4. National Hansen’s Disease Program (NHDP); www.hrsa.gov/hansensdisease

IMAGES:

 

Figure #, location of image, type of image, legend

 

Figure 1. Picture of the rash over the chest

Figure 2. Picture of the rash over the right arm

Figure 3. Picture of the rash over the right arm

Figure 4. Picture of the rash over upper & lower lip

Figure 5. Histo-pathological slide stained with hematoxylin & eosin revealing the multiple granulomas

Figure 6. Histo-pathological slide stained with hematoxylin & eosin revealing the nerve involvement

Figure 7. Picture of the rash over upper & lower lip after treatment

Figure 8. Picture of the rash over the right arm after treatment

Ciji Arthur, MD1, Nada Harik, MD2, Jin-Young Han, MD, Ph.D1 and Jerad Gardner, MD3, (1)Pediatric Infectious Diseases, University of Arkansas for Medical Sciences, Little Rock, AR, (2)University of Arkansas for Medical Sciences, Little Rock, AR, (3)Dermatopathology, Bone & Soft Tissue Pathology, University of Arkansas for Medical Sciences, Little Rock, AR


Disclosures:

C. Arthur, None

N. Harik, None

J. Y. Han, None

J. Gardner, None

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