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RARE CASE
PRESENTATION
D R . V I K R A M K U M A R A R R A G U D L A
D R . PA R T H A S A R A D H I A N C H A L A
A N C H A L A S K I N I N S T I T U T E
A RARE CASE PRESENTATION
• A 5yr old female pt came to Anchala Skin Institute with
chief complaints of rash all over body since 3yrs.
• HISTORY OF PRESENTING ILLNESS : pt was
asymptomatic 3yrs back then few raised lesions started
appearing on hands and scalp spread all over body
involving Trunk & Extremities
• The lesions come on and off every 1to 3month duration.
• Lesions regressing on the trunk and extremities without
any specific therapy
• No H/O pain, itching
• PAST HISTORY: h/o frequent recurrences every 1-2
weeks since 3yrs
• FAMILY HISTORY: Not significant
• PERSONAL HISTORY: Moderately built other wise
normal looking child
• No h/o childhood DM
HISTORY ..
• DRUG HISTORY: Not significant
• GENERAL EXAMINATION: No pallor ,icterus, cyanosis ,clubbing, Edema, Cervical
lymphadenopathy present
• CUTANEOUS EXAMINATION:
– Multiple discrete erythematous to skin colored papules and pustules of 0.5-1 cm size with
crusting and scaling over face, upper extremities, back & trunk, scalp
– PIH surrounding few lesions over shoulders.
– Hair, Nail & Mucosa are Normal
DD’S PLEASE
DD SENT FOR BIOPSY
• Psoriasis
• Plc
• Drug reaction
INVESTIGATIONS
•CBP
•ESR
•RBS WNL
•LFT
HISTOPATHOLOGY DISCUSSION
PLEASE
OUR DIAGNOSIS
• Pustular generalised perforating variant
– Although some lesions clinically appear as follicular pustules , there is no histopathological
evidence of suppurative folliculitis that might be contributing to perifollicular inflammation .
– More likely clinical pustules are filled with necrobiotic material that is eliminated via the hair
follicle .
TREATMENT GIVEN
• SCALP
– TOPICAL FUSIDIC
– COAL TAR + SA SHAMPOO
• OTHER AREAS
– TOPICAL CALICITRIOL
– WHITE PARAFFIN TOPICAL
– ORAL ANTIHITSTAMINES
DISSCUSSION
• Clinical types :
• LOCALISED
• GENERALISED
• SUBACUTANEOUS
• PERFORATING
• Other Patterns:
– PAPULAR UMBILICATED GA
– FOLLICULAR PUSTULOUS GA
– PUSTULAR GENERALISED PERFORATING GA
– Generalised GA in Folliculocentric distribution
– LINEAR GA
– PATCH TYPE GA
OTHER RARE CASE REPORT
• Generalized papular granuloma annulare
Bansal M, Pandey SS, Manchanda
K. Generalized papular granuloma
annulare. Indian Dermatol Online J
[serial online] 2012 [cited 2016 Oct
26];3:74-6. Available
from: http://www.idoj.in/text.asp?201
2/3/1/74/93492
GEN GA IN FOLLICULOCENTRIC
DISTRIBUTION
• 4 cases reported so far
• Pt reported treatment for folliculitis with doxy at start of clinical course
• Owing to follicular distribution of eruption, it is possible that Gen GA developed after
ANTIGENIC changes as a result of diffuse suppurative folliculitis
DISCUSSION
• Granuloma annulare is a benign, inflammatory dermatosis with no
proven etiology. It involves dermis or subcutis in a process characterized
by necrobiosis surrounded by lymphohistiocytes
• Various morphological forms are described, including localized,
generalized, perforating and subcutaneous.
• But the distinctive papular postules morphology in the absence of the
plaques is a rare phenomenon for the generalized variety and presents a
diagnostic dilemma.
• We report this case for the rarity of Papulo pustular presentation of the
disease.
ETIOLOGY
• insect bites , scabies , a cat bite , waxing-induced pseudofolliculitis ,
tuberculin tests ,BCG vaccination, hepatitis B vaccination , chronic HBV
,antitetanus and diphtheria toxoid vaccination ,occupational pressure on
the fingers in a milkman and saphenectomy
• GA : as an ISOTOPIC response to a previous infection
• Viral: HPV, EBV, varicella/zoster, parvo, HCV, HIV
• Bacterial: borrelia burgdorferi
• Sun exposure , (post PUVA)…GA on tattoos, on EMF minor
• Drug induced : gold , allopurinol
• Familial , HLA-Bw35
• A delayed-type hypersensitivity reaction and cell-mediated immune
response are hypothesized
• association of GGA with autoimmune thyroiditis and lipid profile
abnormalities
• GA may represent a paraneoplastic response to certain malignancy like
lymphoma.
DISCUSSION
• Clinical Types of Granuloma
Annulare:
• 1. LOCALIZED : 75% cases.
– Localized granuloma annulare starts
as a ring of small, firm, flesh-colored
or red papules.
– Isolated/coalesced. lateral or dorsal
surfaces of the hands and feet .
>50% patients will have
spontaneous resolution within 2 yrs.
• 2. GENERALISED: 8-15% cases
– similar to the localized variant but is
more widespread, having 10 or more
lesions. extremities, trunk, and neck.
persist for 3 -4 years or longer.
DISSCUSSION
• 3. SUBCUTANEOUS :
– children(2-5 yrs). asymptomatic, rapidly
growing subcutaneous nodules on the
extremities, hands, scalp, buttocks, and
pretibial and periorbital areas.
solitary/clusters.
– excisional biopsy. resolve spontaneously
or may recur after excision.
• 4. PERFORATING:
– rare , children and young adults. It is also
more common in women. localized and
generalized forms. The localized form is
found on the upper limbs and pelvis, and
the generalized form, which is more
common, is present on the abdominal
area, trunk, and upper and lower limbs.
ASSOCIATIONS OF GA
• Diabetes mellitus with GA
• Granuloma annulare of the skin, intra-abdominal lesions, insulin-dependent
diabetes and polyendocrine disease(Thomas et al BMJ 1986; 293: 977–8.)
• Generalized GA with toxic adenoma of the thyroid(Tursen et al J ECAD2002; 16:
419–20)
• uveitis and granuloma annulare (Eur J Ophthalmol 2003; 13: 93–5.)
• Granuloma annulare and malignant neoplasms (Li et al. J Dermatopathol 2003; 25:
113–6)
• temporal arteritis with generalized GA (Fukai et al Clin Exp Dermatol 1990; 15:
70–2.)
• granuloma annulare and morphoea .(Ben et al Clin Exp Dermatol 1999; 24: 86–9.)
• granuloma annulare and necrobiosis lipoidica in the same patient [29–32],
• granuloma annulare and sarcoidosis (FeldmanArch Dermatol 1968: 98: 677–8.)
MANAGEMENT
• Various treatment modalities that have been tried include
– topical steroids, topical imiquimod, and calcineurin inhibitors.
– Cryosurgery
– phototherapy,
– Dapsone
– retinoids,
– cyclosporine,
– chlorambucil,
– Antimalarials
– niacinamide,
– vitamin E,
– pentoxifylline,
– infliximab,
pustular granuloma annulare.pptx
pustular granuloma annulare.pptx
pustular granuloma annulare.pptx
pustular granuloma annulare.pptx

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pustular granuloma annulare.pptx

  • 1. RARE CASE PRESENTATION D R . V I K R A M K U M A R A R R A G U D L A D R . PA R T H A S A R A D H I A N C H A L A A N C H A L A S K I N I N S T I T U T E
  • 2. A RARE CASE PRESENTATION • A 5yr old female pt came to Anchala Skin Institute with chief complaints of rash all over body since 3yrs. • HISTORY OF PRESENTING ILLNESS : pt was asymptomatic 3yrs back then few raised lesions started appearing on hands and scalp spread all over body involving Trunk & Extremities • The lesions come on and off every 1to 3month duration. • Lesions regressing on the trunk and extremities without any specific therapy • No H/O pain, itching • PAST HISTORY: h/o frequent recurrences every 1-2 weeks since 3yrs • FAMILY HISTORY: Not significant • PERSONAL HISTORY: Moderately built other wise normal looking child • No h/o childhood DM
  • 3. HISTORY .. • DRUG HISTORY: Not significant • GENERAL EXAMINATION: No pallor ,icterus, cyanosis ,clubbing, Edema, Cervical lymphadenopathy present • CUTANEOUS EXAMINATION: – Multiple discrete erythematous to skin colored papules and pustules of 0.5-1 cm size with crusting and scaling over face, upper extremities, back & trunk, scalp – PIH surrounding few lesions over shoulders. – Hair, Nail & Mucosa are Normal
  • 4.
  • 5.
  • 6.
  • 7.
  • 9. DD SENT FOR BIOPSY • Psoriasis • Plc • Drug reaction
  • 12. OUR DIAGNOSIS • Pustular generalised perforating variant – Although some lesions clinically appear as follicular pustules , there is no histopathological evidence of suppurative folliculitis that might be contributing to perifollicular inflammation . – More likely clinical pustules are filled with necrobiotic material that is eliminated via the hair follicle .
  • 13. TREATMENT GIVEN • SCALP – TOPICAL FUSIDIC – COAL TAR + SA SHAMPOO • OTHER AREAS – TOPICAL CALICITRIOL – WHITE PARAFFIN TOPICAL – ORAL ANTIHITSTAMINES
  • 14.
  • 15.
  • 16.
  • 17.
  • 18. DISSCUSSION • Clinical types : • LOCALISED • GENERALISED • SUBACUTANEOUS • PERFORATING • Other Patterns: – PAPULAR UMBILICATED GA – FOLLICULAR PUSTULOUS GA – PUSTULAR GENERALISED PERFORATING GA – Generalised GA in Folliculocentric distribution – LINEAR GA – PATCH TYPE GA
  • 19. OTHER RARE CASE REPORT • Generalized papular granuloma annulare Bansal M, Pandey SS, Manchanda K. Generalized papular granuloma annulare. Indian Dermatol Online J [serial online] 2012 [cited 2016 Oct 26];3:74-6. Available from: http://www.idoj.in/text.asp?201 2/3/1/74/93492
  • 20. GEN GA IN FOLLICULOCENTRIC DISTRIBUTION • 4 cases reported so far • Pt reported treatment for folliculitis with doxy at start of clinical course • Owing to follicular distribution of eruption, it is possible that Gen GA developed after ANTIGENIC changes as a result of diffuse suppurative folliculitis
  • 21.
  • 22. DISCUSSION • Granuloma annulare is a benign, inflammatory dermatosis with no proven etiology. It involves dermis or subcutis in a process characterized by necrobiosis surrounded by lymphohistiocytes • Various morphological forms are described, including localized, generalized, perforating and subcutaneous. • But the distinctive papular postules morphology in the absence of the plaques is a rare phenomenon for the generalized variety and presents a diagnostic dilemma. • We report this case for the rarity of Papulo pustular presentation of the disease.
  • 23. ETIOLOGY • insect bites , scabies , a cat bite , waxing-induced pseudofolliculitis , tuberculin tests ,BCG vaccination, hepatitis B vaccination , chronic HBV ,antitetanus and diphtheria toxoid vaccination ,occupational pressure on the fingers in a milkman and saphenectomy • GA : as an ISOTOPIC response to a previous infection • Viral: HPV, EBV, varicella/zoster, parvo, HCV, HIV • Bacterial: borrelia burgdorferi • Sun exposure , (post PUVA)…GA on tattoos, on EMF minor • Drug induced : gold , allopurinol • Familial , HLA-Bw35 • A delayed-type hypersensitivity reaction and cell-mediated immune response are hypothesized • association of GGA with autoimmune thyroiditis and lipid profile abnormalities • GA may represent a paraneoplastic response to certain malignancy like lymphoma.
  • 24. DISCUSSION • Clinical Types of Granuloma Annulare: • 1. LOCALIZED : 75% cases. – Localized granuloma annulare starts as a ring of small, firm, flesh-colored or red papules. – Isolated/coalesced. lateral or dorsal surfaces of the hands and feet . >50% patients will have spontaneous resolution within 2 yrs. • 2. GENERALISED: 8-15% cases – similar to the localized variant but is more widespread, having 10 or more lesions. extremities, trunk, and neck. persist for 3 -4 years or longer.
  • 25. DISSCUSSION • 3. SUBCUTANEOUS : – children(2-5 yrs). asymptomatic, rapidly growing subcutaneous nodules on the extremities, hands, scalp, buttocks, and pretibial and periorbital areas. solitary/clusters. – excisional biopsy. resolve spontaneously or may recur after excision. • 4. PERFORATING: – rare , children and young adults. It is also more common in women. localized and generalized forms. The localized form is found on the upper limbs and pelvis, and the generalized form, which is more common, is present on the abdominal area, trunk, and upper and lower limbs.
  • 26. ASSOCIATIONS OF GA • Diabetes mellitus with GA • Granuloma annulare of the skin, intra-abdominal lesions, insulin-dependent diabetes and polyendocrine disease(Thomas et al BMJ 1986; 293: 977–8.) • Generalized GA with toxic adenoma of the thyroid(Tursen et al J ECAD2002; 16: 419–20) • uveitis and granuloma annulare (Eur J Ophthalmol 2003; 13: 93–5.) • Granuloma annulare and malignant neoplasms (Li et al. J Dermatopathol 2003; 25: 113–6) • temporal arteritis with generalized GA (Fukai et al Clin Exp Dermatol 1990; 15: 70–2.) • granuloma annulare and morphoea .(Ben et al Clin Exp Dermatol 1999; 24: 86–9.) • granuloma annulare and necrobiosis lipoidica in the same patient [29–32], • granuloma annulare and sarcoidosis (FeldmanArch Dermatol 1968: 98: 677–8.)
  • 27. MANAGEMENT • Various treatment modalities that have been tried include – topical steroids, topical imiquimod, and calcineurin inhibitors. – Cryosurgery – phototherapy, – Dapsone – retinoids, – cyclosporine, – chlorambucil, – Antimalarials – niacinamide, – vitamin E, – pentoxifylline, – infliximab,