A case of generalized pustular psoriasis in a 12 year-old girl is described. She had generalized pustular eruptions on erythematous base, presenting lake of pus, yellowish brown crusts and denudation associated with high fever and chills. With the administration of oral etretinate at a dose of 0.8mg/kg/day for 1 week, rapid and dramatic remission was achieved. About 3 months after her discharge, there developed another wave of new pustules with fever. She was again placed on etretinate therapy. However, there was no expected response as before with developing new pustules continuously. At that time, we tried combination therapy with etretinate and methotrexate. the pustules and erythema began to remit, with eventual clearing of the skin in 6 weeks.
Acitretin* ; Child ; Chills ; Erythema ; Etretinate* ; Female ; Fever ; Humans ; Lakes ; Methotrexate* ; Psoriasis ; Skin ; Suppuration

We report a case of histiocytic cytophagic panniculitis in a 5-year-old male. The patient has complained of multiple erythematous indurated palques with tenderness on the lower abdomen, periocular areas, left forearm and both thighs associated with high fever for 45 days. Hepatosplenomegaly and enlarged lymph nodes were found. Laboratory data revealed pancytopenia, elevation of liver enzymes and prolon gation of PT and PTT. Histopathologic findings of subcutaneous plaque taken from the left forenrm showed lobular panniculitis with fat necrosis and some phagocytic histiocytes containing erythrocytes, leukocytes, lymphocytes and nuclear debris, that had the appearance of bean bag. There found no atypical histiocytic infiltrations or cellular pleomorphism.
Abdomen ; Child, Preschool ; Erythrocytes ; Fat Necrosis ; Fever ; Forearm ; Histiocytes ; Humans ; Leukocytes ; Liver ; Lymph Nodes ; Lymphocytes ; Male ; Pancytopenia ; Panniculitis* ; Thigh

BACKGROUND: Redundant and wrinkled face is a common finding in patients with long-standing leprosy, which is responsible for the appearance of premature aging. Chemical peeling plays a role in dermal regeneration in the treatment of wrinkles. However, the effect of chemical peeling has rarely been studied in patients with leprosy. OBJECTIVE: To investigate the effect of chemical peeling on dermal connective tissue components and clinical improvement of facial wrinkles in patients with leprosy. METHODS: Five patients with clinically and bacteriologically inactive lepromatous leprosy were treated with 70% glycolic acid plus 35% trichloroacetic acid (GA-TCA). Histologic and clinical changes were evaluated at 0 and 90 days postpeel. RESULTS: Histologically, collagen fibers and ground substance increased significantly but elastic fibers did not change at 90 days postepeel. Clinically, fine wrinkles improved considerably, whereas most deep wrinkles remained unchanged at day 90. CONCLUSION: This study demonstrates that chemical peeling with GA-TCA, or medium-depth peeling, causes an increase in collagen and ground substance but not in elastic fibers. These changes result in limited clinical improvement in the facial wrinkles of leprosy patients. Therefore, medium-depth chemical peeling may be insufficient to improve the premature aging appearance in patients with leprosy.
Aging, Premature ; Collagen ; Connective Tissue* ; Elastic Tissue ; Humans ; Leprosy* ; Leprosy, Lepromatous ; Regeneration ; Trichloroacetic Acid

BACKGROUND: Ulnar nerve palsy in patients with Hansen disease causes hand wasting and atrophy. To masquerade these deformities, patients injected paraffin or silicone into the lesions, which results in paraffinomas. Several years later, painful inflammation, ulceration or abscesses may develop in the paraffinomas. OBJECTIVE: To investigate the clinical characteristics of paraffinomas in patients with Hansen disease, and to evaluate and compare the effects of various surgical treatment methods of paraffinomas. METHODS: Fifty-seven patients with paraffinomas were randomized to receive debridement(curettage) with primary closure, skin graft, skin flap, or second intention at the hospital of Korean Hansen Welfare Association from 1998 to 2012. We analyzed retrospectively the final cosmetic results of three methods, primary closure, skin graft, and skin flap, in 33 patients with more than 6 months follow-up. The final results were classified into 4 grades, excellent, good, fair, and poor. RESULTS: Of 57 patients, the ratio of male and female was 42:15 and the average age was 73 and 71 years repectively. The location of the lesions was the hand and wrist in 54 patients and the face in 3. The total number of procedures was 72 and that of 3 methods was 55. In 33 patients, 19 showed above fair grades and the recurrence rate was 42%(14/33). There were no serious postoperative complications. CONCLUSION: Wide and thorough curettage as debridement was very important to reduce recurrence and the primary closure was most efficient in consideration of an old age of patient, simplicity of procedure and cosmeosis.
Abscess ; Atrophy ; Congenital Abnormalities ; Curettage ; Debridement ; Female ; Follow-Up Studies ; Hand ; Humans ; Inflammation ; Intention ; Leprosy* ; Male ; Paraffin ; Postoperative Complications ; Recurrence ; Retrospective Studies ; Silicones ; Skin ; Transplants ; Ulcer ; Ulnar Neuropathies ; Wrist

Lepra bacilli invade the nasal mucosa from early stage. The infiltrated mucous membrane breaks down and ulcerates easily. Irritating crusts and ulcers occur, creating in perforation of the septum. When most of the septum and inner mucosal lining of the nose is destroyed, the nose is deprived of its support and it collapses. The nasal bones are typically intact, and the collapsed nose adheres to the facial skeleton, giving the characteristic 'sunken and short nose' deformities. We have operated on 25 patients with deformities such as short nose, sunken nose, collumela deformity and ala collapse since 1992. In short nose we operated with a paramedian forehead flap and rib cartilage graft in a cantilever pattern. In ala collapse we used a conchal cartilage graft. Lastly, in collumela deformity we used the local flap and a composite graft.
Cartilage ; Congenital Abnormalities* ; Forehead ; Humans ; Leprosy* ; Mucous Membrane ; Nasal Bone ; Nasal Mucosa ; Nose ; Ribs ; Skeleton ; Transplants ; Ulcer

BACKGROUND: Damage of the facial nerve, especially buccal and marginal mandibular branches, causes facial palsy and paralytic deformity of the lips such as lower lip ectropion, lip drooping, deviation of the mouth, inability of closing mouth, and the loss of the nasolabial folds. OBJECTIVE: To find out the best surgical method to correct paralytic deformity of the lips. METHODS: We reviewed retrospectively the various surgical treatment modalities of the paralytic deformity of the lips performed in The Institute for Leprosy Research for 13 years. RESULTS: One hundred and twenty five surgical procedures were performed in 80 patients with Hansen's disease. Of these, the static method, dermal suspension flap, was done in 15 cases, the dynamic methods were done in 64 cases, and others were done in 46 cases. All patients showed postoperatively clinical improvement above the average. Two patients received reoperation or additional procedures due to relapse of the condition. CONCLUSIONS: The dynamic method using tensor fascia lata was superior to other treatment modalities.
Congenital Abnormalities ; Ectropion ; Facial Nerve ; Facial Paralysis ; Fascia Lata ; Humans ; Leprosy ; Lip ; Mouth ; Recurrence ; Reoperation ; Retrospective Studies

Facial nerve paralysis in leprosy adversely affects facial regions from the forehead to the lip. In particular, lagophthalmos in patients with leprosy causes exposure keratitis, corneal and conjunctival dryness which can progress blindness and disfigurement. The function of the eyelids is controlled by the oculomotor nerve and the facial nerve. In leprosy patients the oculomotor nerve continues to function causing the levator muscle to lift the eyelids. However, paralysis of the facial nerve prevents the orbicularis oculi muscle from closing the eyelids, resulting in lagophthalmos. Various methods were developed to correct lagophthalmos, one of which was temporal muscle transfer(TMT) reported in 1934 by Gillies. Since we noted relatively high occurrence of ptosis as a complication in patients treated previously with the TMT, we performed other surgical procedures for laglphthalmos over 15 years, not to make the ptosis. Although most of the patients quite improved post-operatively, partial lid-gap frequently persisted. It may be related to involutional changes and paralyzed orbicularis muscle. Recently, we conducted 4 different TMT methods for last 4 years to reduce ptosis. The methods used included Brown & McDowell, McCord & Codner, modified Gillies & Anderson, and modified Gillies. Seventy-five TMT operations in 60 patients(26 males and 34 females) were done between 2011 and 2014. The age range of the patients was 54~87 years(the mean was 70.1). Fifteen patients had bilateral TMT procedures. As a result, ptosis appeared in 14(18.7%) out of 75 TMT procedures for 4 years. Four technical points should be considered for the initial assessment to prevent or reduce the incidence of ptosis. The first is the increase of the length of temporalis muscle flap to approximately 8cm with a parallel course to the lateral canthus, which will reduce the oblique pull. Second, the width of the fascia sling in the upper eyelid is narrow(3~4mm) to reduce weight on the eyelid. Third, the fascia sling in the upper lid is not tied with that of the lower lid at the medial canthal tendon to reduce tension. Lastly, the fascia sling in the upper eyelid is shallow(subdermal level) in location and as near as possible to the lid margin.
Aged ; Blindness ; Eyelids ; Facial Nerve ; Fascia ; Forehead ; Humans ; Incidence ; Keratitis ; Leprosy* ; Lip ; Male ; Oculomotor Nerve ; Paralysis ; Temporal Muscle* ; Tendons

Paralytic lagophthalmos and ectropion in leprosy are serious complications of facial paralysis, which may lead to expose keratitis, corneal ulceration, further lead to blindness. In 1995, 1997 and 1999, we had reported in this journal on the surgical treatment of paralytic lagophthalmos and ectropion. In the first report of 1995 for lid closing, I(Ahn) performed various method of traditional surgery such as temporal muscle transfer, medial and lateral canthoplasty as well as gold implantation. In the 2nd report of 1997, We(Ahn and Park) presented combination treatment of gold plate with minor change of design and weight in the upper lid and medial canthoplasty with horizontal shortening in the lower lid. In the 3rd report of 1999, We described an additional graft of conchal cartilage in the lower lid. However, the lateral end of the cartilage would warp and create new partial ectropion several months later. Therfore, we shifted from cartilage to Alloderm(R), acellular dermal graft. We applied it to the lower border of the tarsus instead of cartilage and fixed each end to medial and lateral canthal ligaments respectively. We think Alloderm(R) is a good substitute for the cartilage and is an effective volume expander to the atrophic lower lid.
Ankle ; Blindness ; Cartilage ; Corneal Ulcer ; Ectropion* ; Facial Paralysis ; Keratitis ; Leprosy ; Ligaments ; Temporal Muscle ; Transplants

To affect the complete closure of the eyelids in leprosy patients with lagophthalmos, we have used various surgical methods to avoid using the temporalis transfer method. Although widely accepted as the most effective treatment for the chronic lagophthalmos patient, the temporalis transfer method creates a simultaneous involuntary closure of the eyelids while biting or chewing. This side effect creates a social stigmata which former leprosy patients wish to avoid. As a result, we have attempted to identify the most effective treatment which will also eliminate this involuntary closure of the eyelids during biting or chewing. In the upper eyelid, we performed gold plate implantation and levator muscle severing. In the lower eyelid, lateral tarsal strip, permanent medial tarsorrhaphy, horizontal shortening, cartilage grafting and alloderm graft have all been used in the treatment of ectropion. Based on our treatment of multiple patients, we now believe medial tarsorrhaphy supplemented with the various other methods mentioned above are insufficient to achieve the desired result of complete closure of the eyelids. As a result, we are now looking to a modified temporalis transfer procedure to ensure complete closure of the eyelids without the eye closing side effect.
Cartilage ; Christianity ; Ectropion ; Eyelids ; Humans ; Leprosy ; Mastication ; Transplants

Recently, there has been a strong decrease in the number of temporalis muscle transfer(TMT) operations in cases of lagophthalmos. The primary cause of this decline is patient dissatisfaction with drawbacks of eye clinching in concert with mouth closure, ectropion and ptosis. This is mostly true of patients who are now more commonly exposed to urban social environments. In 1997, I discontinued the use of TMT as part of my combination treatment method reported in this journal. Since 1998, I have removed the fascia in 20 TMT cases due to complications in the upper and lower eyelid areas. I then corrected these complications using my combination method. I still use TMT in cases of lower lip drooping. Lagophthalmos patients are treated using my combination method of gold lid loading in the upper eyelid and Alloderm insertion in the lower eyelid through fixation of both ends to the medial and lateral canthal ligaments respectively. Previously, I recommended the use of conchal cartilage but noted the complication of cartilage warping which caused creating new partial ectropion several months later. Alloderm has shown no complications and the results of its use remain most satisfactory.
Cartilage ; Ectropion* ; Eyelids ; Fascia ; Humans ; Leprosy* ; Ligaments ; Lip ; Mouth ; Social Environment