CME       OCTOBER 2001 Case presentations       CASE NO : 1

Age : 11 yr.           Sex :F

P/C- Brownish hyperpigmentation of palms and soles and flexural areas since 3 months of age

HOPI-Patient is a product of nonconsanguinous marriage,  was  an FTNVD delivered at home. Patient was apparently well till 3 months of age when the parents noticed development of hyperpigmention over the knuckles and dorsum of I/P joints of hands and  feet.  By 7-8 yrs,  hyperpigmentation gradually appeared bilaterally   on  the  soles, palms , axillae, neck, periorbital area, groin, genitalia, cubital fossa and popliteal fossae b/l , oral mucosa and nails.  The pigmentation progressively has increased in extent and intensity. H/o failure of hair to grow longer than the present length. H/o lightening of hair since 2 yrs of age .Three years back the patient also started developing crops of hyperpigmented, mildly itchy, pinhead sized papules on the extremities  which  heal  in  2  to   3  weeks,  with    residual     hyperpigmentation h/o hyperhidrosis of palms 3 yrs. Past history /Family history / Personal history - not significant  

EXAMINATION

GPE  and vitals    wnl Cutaneous examination
* Brownish  reticulate,   hyperpigmentation  present on  the   neck,  axillae,  periorbital area,  cubital  fossae, popliteal  fossae.
*    Brownish  black  diffuse to  mottled hyperpigmentation  present b/l on the palms and soles, dorsal aspects of  i/p  jts, mcp jts and elbow jts, buttock, groin and external  genitalia.

*    Multiple  discrete   hyperpigmented  0.3 - 0.5 mm  sized       macules primarily on the extremities.
*    Multiple discrete hyperpigmented , pinhead sized,keratotic       follicular papules with a central horny plug present on  the    extremities.
Palms /Soles - diffuse hyperpigmentation, cool moist palms, dermatoglyphics normal
Hair --    Scalp hair is sparse with lightening of hair.
Mucosae --    Tongue and gingival margins show pigmentation.
Nails --    Longitudinal melanonychia +.
Systemic examination - NAD  

INVESTIGATIONS - NAD HISTOPATHOLOGY -
*    Hyperpigmented macule - epidermis shows increased pigment in   the basal layer, with focal areas of basal cell degeneration with  pigment incontinence and melanophages in the papillary dermis.
*    Follicular  papules  -  epidermis shows hyperkeratosis, keratotic  plugging,  follicular  plugging  and  focal   thinning . There is  increased melanin in basal layer and stratum spinosum .Dermis
      shows pigment incontinence with presence of many melanophages and perivascular lymphocytic infiltration.     Infundibulum   of occasional hair follicles shows increased melanin.

DIAGNOSIS :  Generalised cutaneous melanosis.

      

CASE NO. 2

Age : 22 years                    Sex: Male

PRESENTING COMPLAINTS

1.    Pain and swelling involving multiple joints with fever off and on for    last 6 months.
2.    Multiple, erythematous, scaly lesions of variable size present all over
       the body including scalp and genitalia for last 2.5 months.

HISTORY OF PRESENT ILLNESS

        *  Low back pain followed by pain and swelling in right knee, left knee, right ankle, left ankle and left temporomandibular joint with stiffness  for 6 months, associated with high grade fever.

        *  2.5 months  back  he  developed  erythematous,  scaly   lesions  with pustules on right knee and then on whole body.

        *  H/O burning micturition, redness and photophobia both eyes +

GENERAL PHYSICAL EXAMINATION  - Mild pallor +

        Bilateral axillary and inguinal lymph nodes +

CUTANEOUS EXAMINATION

    1.    Multiple, erythematous, scaly, papules and plaques studded with  pustules of variable size present all over the body including palms, soles and scalp with auspitz sign (-ve).
    2.    Oral mucosa -discrete, superficial erosions over palate.
    3.    Circinate balanitis with scanty urethral discharge +
    4.    Nails -subungual hyperkeratosis, onycholysis and shedding of nails.

MUSCULOSKELETAL SYSTEM EXAMINATION

    B/L knee, ankles, left middle finger, left TM joint - swelling, redness,
    tenderness +

INVESTIGATIONS

    *Hb= 10.5g%
    *ESR=50mm/hr
    *Urethral smear: Chlamydial inclusion bodies +
    *H.I.V.: negative
    *RF : negative
    *Histopathology :Consistent with pustular psoriasis

DIAGNOSIS - Reiter's disease

PURPOSE OF PRESENTATION :  For demonstration.

CASE  FOR DISCUSSION

CASE - 3 A

20 Years                        Female

C/O : Raynaud's phenomenon in fingers - 1 1/2 years.
          Digital pitted scarring-9 months.
          Hyperpigmentation of face, neck, hands - 7 months.
          No systemic complaints.

G.P.E. - Mild pallor
             Rest - WNL

CUTANEOUS EXAMINATION

 Mask -like facies with thickening of skin.  Conjunctival sign, pinched nose, microstomia-present.
 Mat like telangiectasias over cheeks.  Melasma, depigmentation along rim of lips, salt and pepper
 pigmentation on anterior aspect of neck, diffuse  hyperpigmentation of face, neck, arms, trunk and shins.
Two keloids present over chest.
          Hands- Thickened, indurated skin over hands and forearms.
                      Raynaud's phenomenon- prsent.
                      Sclerodactyly- present.
                      Digital pitted scarring.
          Nails - Ragged cuticles

SYSTEMIC EXAMINATION- NAD

INVESTIGATIONS

         Routine and Special investigations - WNL
         Antibody profile- LE, ANA, RF - negative
         H.P.E. - Consistent with morphea.

DIAGNOSIS
        PROGRESSIVE SYSTEMIC SCLEROSIS.

TREATMENT RECEIVED

       Tab.Minocycline 50 mg. B.D.

 

CASE  3 B

27 Years                     male

C/O : Raynaud's phenomenon -4 years

         Digital ulceration and scarrying -3 years

         Reflux esophagitis -3 years

G.P.E. : W.N.L.

CUTANEOUS EXAMINATION :

*    Thickened, indusrated skin over face, distal digits of hands, forearms
      and arms.

*    Raynaud's phenomenon -present.

*    Digital pitted scarring

*    Ragged cuticles , nail - fold telangiectasias.

*    Reticulate  pigmentation over upper, anterior chest.

SYSTEMIC EXAMINATION - NAD

INVESTIGATIONS

       Routine and special investigations -WNL

       Chest X-ray : B/L prominence of bronchovascular markings.

       Antibody profile -LE, RF, ANA-negative

                                   Anti-scl 70-positive.

DIAGNOSIS

        PROGRESSIVE SYSTEMIC SCLEROSISSS

CASE NO - 4

Age - 24 yrs                                Sex -M        Farmer
P/C  -    pain  and  swelling  with  multiple  draining sinuses  from Rt foot
x 5 yrs
HOPC - Pt developed a pea sized skin coloured painless raised  lesion 5 yrs back, which doubled in size with exudation of white purulent discharge over tow months followed by development of pain in the foot both on walking and involved the dorsum of  foot . planter surface and the ankle associated with discharge of
serosanguinous fluid with red granules. no h/o trauma/ fever/wt loss/cough/hemoptysis.  Tt history- drainage in village and various antibiotics like Septran, ATT, antifungals.

GENERAL  EXAMINATION - wnl

CUTANEOUS Ex  -  Rt  foot  shows  diffuse  swelling   with hyperpigmentation with multiple draining sinuses some active, which on manipulation extrude a serosanguinous discharge with red granules.  The interdigital spaces of Rt foot show maceration and hemorrhagic crusted lesions.  The sinus opening shows             hypertrophic granulation tissue. There is atrophy of calf muscles of Rt lower limb.
Inguinal LAP - ve.  

ROUTINE INVESTIGATION - wnl
X- ray Rt foot- metatarsals and tarsals shows irregularity and illdefined lytic areas.  Macroscopic Ex- discharge of red granules Microscopic Ex-

        KOH- no septate hyphae seen.
        Grams- eosinophiilic granules
        with filamentous gram +ve
        bacilli.
        AFB-- ve

Pus culture- .+ve for staph. aureus Culture for  AFB- did not show any growth at 1 wk
Fungal culture- no growth at  wk Histopathological  Ex - In the dermis there is formation of abscess with central colony of radiating filamentous gram+ve bacteria, bordered byeosinophilic club like Splendor and Hoeplli material and surrounded by mixed inflammatory infiltrate comprising mainly of neutrophnils, lymphocytes and few macrophages.
DIAGNOSIS- ACTINOMYCETOMA FOOT

Treatment- Dapsone 100 mg 1 OD
                  Amikacin 15 mg/kg/d in divided
doses
Purpose of presentation- For Demonstration

Case No. - 5

Pateint : 9 yrs old girl.

P/C : asymptomatic, hypopigmented, flat lesions with hyperpigmented spots clustered over them, involving Rt upper chest, Rt axilla, Rt upper back noticed at 2 yrs of age.
HOPI : Pt was apparently well at birth. At 2 yrs of age mother noticed hypopigmented,asymptomatic, flat lesions of size varying from pinhead to 1 Re. coin with overlying hyperpigmented spots involving
the Rt upper chest, Rt axilla and Rt upper back.  The lesions remained stable, although they enlarged proportionately with  the growth of the child, to the present size.

H/o an episode of partial focal seizures, involving the Lt side of body, preceeded by vomiting, controlled by iv diazepam and dislantin 4 yrs back.  Pt was not on any maintainance anticonvulsants and seizures never recurred.  No h/o headache/ mental retardation/lower back pain / stopy / other skin lesions.
No family  H/o similar lesions.

CUTANEOUS Ex : Nevus depigmentosus with co-localised
lentiginosis involving Rt mammary region, Rt axilla and Rt upper
scapular region.
GPE, Systemic Ex, Routine Inv - NAD
X-ray lumbosacral spine - transitional vertebra +
                                    - spina bifida of S-1 vertebra +
                                    - I.V.Disc spaces maintained
USG abdomen, CT scan head, EEG - NAD

SKIN BIOPSY -  Hyperpigmented spot - consistent with lentigenes, hypopigmented macule  -  consistent with nevus
depigmentosus.

DIAGNOSIS  :  NEVUS DEPIGMENTOSUS  with co- loclised LENTIGENES with SPINA BIFIDA.

PURPOSE  :  Demonstration. 

CASE NO.6

10 years                male

P/C:  generalized peeling of skin since  2 years of age.

HOPI:    Generalized peeling of skin was noticed by the parents at 2 years             of age involving the shoulders, trunk, upper extremities, tips of fingers and        lower extremities with sparing of scalp, fce, palms and soles.  There was no         h/o erythema or pruritus. Since then there have been similar episodes occurring periodically with h/o superficial versicles on both extermities in summers which ruptured spontaneously, giving rise to peeling of skin without leaving any moist  skin surface.
No h/o consanguinity.

G.P.E. -   WNL

CUTANEOUS  EXAMINATION

*    Multiple discrete patches of peeling skin without underlying
      erythema present over upper and lower limbs.
*    Mild hyperkeratotic, hyperpigmented skin with rippled
      appearance over the extersor aspect of knees, elbows and ankles.
*    Diffuse keratoderma of palms and soles with focal areas of peeling
      over fingertips.
*    Hair, nails and mucous membranes normal.
 

LAB. INVESTIGATIONS

*    Routine investigations : WNL
*    H.P.E.:moderate hyperkeratosis, cleavage within stratum
      corneum,with broadened rete ridges and normal
      dermis. Suggestive of peeling skin syndrome.

DIAGNOSIS

     PEELING  SKIN SYNDROME

PURPOSE OF PRESENTATION

    For demostration

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