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CASE REPORT
Year : 2019  |  Volume : 10  |  Issue : 5  |  Page : 564-566  

Granulomatous variant of giant centrifugal miliaria profunda in a hypothyroid infant: A case report


Department of Dermatology, Seth G.S. Medical College and KEM Hospital, Mumbai, Maharashtra, India

Date of Web Publication28-Aug-2019

Correspondence Address:
Sunanda A Mahajan
B/17, Bhagyanagar Society, Lt. Dilip Gupte Road, Mahim, Mumbai - 400 016, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_422_18

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   Abstract 


The miliarias are a clinically heterogeneous group of diseases which occur when the free flow of eccrine sweat to the skin surface is impeded. Miliaria profunda is a variant with obstruction of the duct at or below the level of dermoepidermal junction. The giant centrifugal variant of miliaria profunda has been described in the past at the sites of occlusive tapes and in febrile patients. Thyroid hormone has a regulatory effect on the skin and its appendages and an association of hypothyroidism with this variant of miliaria profunda has not been described in the past. We report a case of giant centrifugal miliaria profunda in an infant with congenital hypothyroidism.

Keywords: Annular, giant centrifugal miliaria profunda, granulomatous, hypothyroidism


How to cite this article:
Chadha AA, Mahajan SA, Dongre A, Khopkar US. Granulomatous variant of giant centrifugal miliaria profunda in a hypothyroid infant: A case report. Indian Dermatol Online J 2019;10:564-6

How to cite this URL:
Chadha AA, Mahajan SA, Dongre A, Khopkar US. Granulomatous variant of giant centrifugal miliaria profunda in a hypothyroid infant: A case report. Indian Dermatol Online J [serial online] 2019 [cited 2019 Sep 19];10:564-6. Available from: http://www.idoj.in/text.asp?2019/10/5/564/265633




   Introduction Top


Miliarias are a group of eccrine disorders having in common retention of sweat due to occlusion of the sweat duct producing a clinically heterogeneous eruption. Three variants of miliaria have been defined according to the depth at which this sweat duct obstruction occurs. Miliaria profunda refers to the development of flesh-coloured papules resembling gooseflesh, associated with obstruction of the sweat duct near the dermoepidermal junction. It usually follows severe persistent miliaria rubra and is associated with anhidrosis. Giant centrifugal miliaria profunda and its granulomatous variant has been described in the past.[1],[2] We report a case of granulomatous variant of giant centrifugal miliaria profunda in an infant with congenital hypothyroidism.


   Report Top


A 10-month-old infant with congenital pelviureteric junction obstruction and hydronephrosis was referred from the paediatric surgery department, in view of asymptomatic red raised lesions all over body since 1 month in the winter season. Patient gave history of similar lesions on the extremities six months back, which resolved on application of topical steroid containing creams. There was no history of consanguinity in parents. Mother had history of autoimmune hypothyroidism and was on thyroxine supplementation. Infant was diagnosed with non-autoimmune hypothyroidism at two months of age, and was started on levothyroxine 25 microgram. General examination revealed dysmorphic facies with depressed nasal bridge. The patient was afebrile. Thyroid was impalpable. Cutaneous examination showed multiple skin coloured to erythematous papules coalescing to form infiltrated annular plaques mainly over occipital scalp, neck, ear helices, proximal extremities, elbows, knees, buttocks, dorsa of hands and feet and few over trunk. There was no evidence of scaling. Hair were dry and sparse and nails were normal [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d and [Figure 2].
Figure 1: (a-d) Original: multiple pink erythematous to skin coloured papules coalescing to form larger papules and plaques over neck, proximal and distal upper extremity, buttocks, thighs, knees with sparing of palms and soles

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Figure 2: Original: multiple erythematous papules coalescing to form large well defined infiltrated annular plaques with central crusting

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Keeping in mind the dysmorphic facies and the monomorphic nature of the papules, we kept the differentials of generalised granuloma annulare, Giannotti Crosti syndrome, congenital syphilis and infantile systemic hyalinosis. The patient's hepatitis B antigen and serum VDRL were negative. Thyroid profile was deranged with a high TSH of 7.16, normal T3 T4 levels and normal anti-thyroid peroxidase antibodies. Histopathological examination showed orthohyperkeratosis with mild acanthosis and small nodular granulomas made of epithelioid cells, giant cells, lymphocytes and plasma cells centred around hyperplastic and dilated intradermal portion of sweat ducts. The sweat ducts were plugged with orthokeratotic laminated pink keratin and infiltrated with neutrophils in foci. The wall of the eccrine duct is partially deficient in mid dermis and it is in these foci that neutrophils and granuloma predominate. The acrosyringia show mild spongiosis and plugging [Figure 3]a and [Figure 3]b Based on these findings, we diagnosed the patient as granulomatous variant of giant centrifugal miliaria profunda as described in two previous case reports.[1],[2]
Figure 3:(a) Original: The acrosyringia plugged with orthokeratotic laminated pink keratin, ruptured in mid dermis and the extruded keratin plug is surrounded by granulomatous infiltrate (H and E, 25X with inset 40X); (b) Original: Granulomas consisting of langhans giant cells, foreign body giant cells, epithelioid cells, neutrophils and few lymphocytes and plasma cells are surrounding the ruptured sweat duct (H and E stain,100X)

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The patient was managed conservatively only with soothing lotions and emollients, and in a span of one month, erythema and infiltration reduced with reduction in the size of the lesions.


   Discussion Top


Miliaria profunda refers to the development of flesh-coloured deep-seated asymptomatic papules resembling gooseflesh concentrated on the trunk and extremities, associated with obstruction of the sweat duct near the dermoepidermal junction. It usually lasts only 1 hour, follows severe persistent miliaria rubra and is associated with anhidrosis.

Rogers et al. reported a case of a 4-month-old infant with centrifugal expansion of white plaques with erythematous margins, histopathology showing distended pores and acrosyringia of the eccrine duct with ortho and parakeratotic keratin, neutrophils and eosinophils around these ducts as well as in the surrounding interstitium, and labelled it as giant centrifugal miliaria profunda.[1] The lesions occurred at sites where occlusive tape was applied.

Doshi et al. reported two similar infants with asymptomatic papules and annular plaques over trunk and extremities with granulomatous inflammatory reaction around the eccrine apparatus on histopathology and called it a granulomatous variant of giant centrifugal miliaria profunda.[2] However, both the patients had history of fever at the onset of the lesions.

It has been presumed since the 19th century that obstruction of the eccrine duct is involved in the pathogenesis of the miliarias. The first demonstrable histological change is the accumulation of PAS-positive, diastase-resistant material in the distal pore. Holzle and Kligman[3] suggested that this material represented the primary obstruction which cannot always be demonstrated in tissue sections but has been shown experimentally. The nature of this PAS positive material is still unclear. Dobson et al.[4] believed it to be derived from the secretary cells of the sweat coils while Holzle and Kligman[3] were of the view that it originated from the cytoplasm of the sweat duct luminal cells. Various factors may contribute to the initial duct obstruction. These include changes in the horny layer related to excess sweating, the toxins secreted by the increased bacterial colonisation on the skin due to increased sweating, the presence of sodium chloride in more than isotonic concentration and lipoid depletion

After several days, a keratin plug forms as part of the repair process, leading to further obstruction of the duct, often at a deeper level.[4],[5] The intraepidermal part of the sweat duct dilates and ruptures causing extravasation of sweat into the dermis which leads to intense local oedema and the lesions of miliaria profunda and anhidrosis.[5] As the repair progresses, the plug moves upwards and is shed leading to restoration of sweating.

Thyroid hormone is an important regulator of epidermal homeostasis. All three thyroid hormone binding isoforms of thyroid receptor have been recognised in skin tissue. The skin in hypothyroidism is rough and covered with fine scales, notably on the extensor extremities.[6] Xerosis may resemble an acquired ichthyosis. The etiology of xerosis in hypothyroidism is unknown. Theories have included hypohidrosis related to cytological alterations in the eccrine apparatus such as PAS-positive, diastase-resistant granules in the pale cells of the secretory coil,[7] diminished sebaceous gland secretion,[8] and diminished epidermal sterol biosynthesis, especially cholesterol and cholesterol esters.[9] Hypothyroidism has been reported to be a cause of increased sweat electrolytes.[10]In vitro keratinocyte studies have shown that depletion of T3 results in elevated levels of transglutaminase, which is involved in the formation of the cornified envelope. Further, T3 depleted keratinocytes have diminished levels of plasminogen activator, an enzyme implicated in the corneocyte shedding process.[11]

This accumulation of the PAS positive material in the eccrine apparatus, the sweat electrolyte changes and the defective keratinisation seen in hypothyroidism could be believed to be contributory to the occurrence of giant centrifugal miliaria profunda in our patient.


   Conclusion Top


The giant centrifugal variant of miliaria profunda is a rare variant of miliaria profunda described as infiltrated erythematous to lichenoid papules and plaques which may be annular in configuration secondary to obstruction in the deeper portions of the eccrine duct. It has been previously reported in infants with febrile episodes or at sites covered under occlusive tapes. The granulomatous reaction is to the keratin plug from the ruptured distended eccrine duct. Our case is a representation of the granulomatous variant of giant centrifugal miliaria profunda in a child without any external physical contributory factor like sweating or occlusion or history of fever. We propose it to be due to the altered keratinisation due to his underlying hypothyroid state.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Rogers M, Kan A, Stapieton K, Kemp A. Giant centrifugal miliariaprofunda. PediatrDermatol 1990;7:140-6.  Back to cited text no. 1
    
2.
Doshi BR, Mahajan S, Kharkar V, Khopkar US. Granulomatous variant of giant centrifugal miliaria profunda. Pediatr Dermatol 2013;30:48-51.  Back to cited text no. 2
    
3.
Holzle E, Kligman AM. The pathogenesis of miliaria rubra. Role of resident microflora. Br J Dermatol 1978;99:117-37.  Back to cited text no. 3
    
4.
Dobson RI, Lobitz WC. Hanover NH. Some histochemical observations on the human eccrine sweat glands. II. The pathogenesis of miliaria. Arch Dermatol1957;75:653-66.  Back to cited text no. 4
    
5.
O'Brien JP. A study of miliaria rubra, tropical anhidrosis and anhidrotic asthenia. Br J Dermatol Syph 1947;59:125-58.  Back to cited text no. 5
    
6.
Slominski A, Wortsman J, Kohn L, Ain KB, Venkataraman GM, Pisarchik A, et al. Expression of hypothalamic-pituitary-thyroid axis related genes inhuman skin. J Invest Dermatol 2002;119:1449-55.  Back to cited text no. 6
    
7.
Means MA, Dobson RL. Cytological changes in the sweat gland in hypothyroidism. JAMA 1963;186:113-5.  Back to cited text no. 7
    
8.
Heymann WR. Cutaneous manifestations of thyroid disease.J Am Acad Dermatol 1992;26:885-902.  Back to cited text no. 8
    
9.
Rosenberg RM., Isseroff RR, Ziboh VA, Huntley AC. Abnormal lipogenesis in thyroid hormone-deficient epidermis. J Invest Dermatol 1986;86:244-8.  Back to cited text no. 9
    
10.
Squires L, Dolan TF. Abnormal sweat chloride in auto-immune hypothyroidism. ClinPediatr 1989;28:535-6.  Back to cited text no. 10
    
11.
Isseroff RR, Chun KT, Rosenberg RM. Triiodothyronine alters the cornification of cultured human keratinocytes. Br J Dermatol 1989;120:503-10.  Back to cited text no. 11
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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